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<div class="pre-content"><div><div class="bk_prnt"><p class="small">NCBI Bookshelf. A service of the National Library of Medicine, National Institutes of Health.</p><p>PDQ Cancer Information Summaries [Internet]. Bethesda (MD): National Cancer Institute (US); 2002-. </p></div><div class="iconblock clearfix whole_rhythm no_top_margin bk_noprnt"><a class="img_link icnblk_img" title="Table of Contents Page" href="/books/n/pdqcis/"><img class="source-thumb" src="/corehtml/pmc/pmcgifs/bookshelf/thumbs/th-pdqcis-lrg.png" alt="Cover of PDQ Cancer Information Summaries" height="100px" width="80px" /></a><div class="icnblk_cntnt eight_col"><h2>PDQ Cancer Information Summaries [Internet].</h2><a data-jig="ncbitoggler" href="#__NBK65869_dtls__">Show details</a><div style="display:none" class="ui-widget" id="__NBK65869_dtls__"><div>Bethesda (MD): <a href="http://www.cancer.gov/" ref="pagearea=page-banner&targetsite=external&targetcat=link&targettype=publisher">National Cancer Institute (US)</a>; 2002-.</div></div><div class="half_rhythm"></div><div class="bk_noprnt"><form method="get" action="/books/n/pdqcis/" id="bk_srch"><div class="bk_search"><label for="bk_term" class="offscreen_noflow">Search term</label><input type="text" title="Search this book" id="bk_term" name="term" value="" data-jig="ncbiclearbutton" /> <input type="submit" class="jig-ncbibutton" value="Search this book" submit="false" style="padding: 0.1em 0.4em;" /></div></form></div></div></div></div></div>
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<div class="main-content lit-style" itemscope="itemscope" itemtype="http://schema.org/CreativeWork"><div class="meta-content fm-sec"><h1 id="_NBK65869_"><span class="title" itemprop="name">Bile Duct Cancer (Cholangiocarcinoma) Treatment (PDQ®)</span></h1><div class="subtitle whole_rhythm">Health Professional Version</div><p class="contrib-group"><span itemprop="author">PDQ Adult Treatment Editorial Board</span>.</p><p class="small">Published online: March 27, 2020.</p></div><div class="jig-ncbiinpagenav body-content whole_rhythm" data-jigconfig="allHeadingLevels: ['h2'],smoothScroll: false" itemprop="text"><div id="_abs_rndgid_" itemprop="description"><p id="CDR0000062905__227">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of bile duct cancers. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.</p><p id="CDR0000062905__228">This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).</p></div><div id="CDR0000062905__1"><h2 id="_CDR0000062905__1_">General Information About Bile Duct Cancer</h2><p id="CDR0000062905__83">Cancer of the bile duct (also called cholangiocarcinoma) is extremely rare. The true incidence of bile duct cancer is unknown, however, because establishing an accurate diagnosis is difficult.</p><p id="CDR0000062905__226">Traditionally, bile duct tumors located within the liver had been classified with hepatocellular carcinoma as primary liver tumors.[<a class="bk_pop" href="#CDR0000062905_rl_1_1">1</a>] In contrast, bile duct tumors located outside of the liver had been classified with gallbladder cancer as extrahepatic biliary tract tumors.[<a class="bk_pop" href="#CDR0000062905_rl_1_1">1</a>] The classification of bile duct tumors has changed to include intrahepatic tumors of the bile ducts and extrahepatic tumors (perihilar and distal) of the bile ducts.</p><p id="CDR0000062905__341">Approximately 50% of cholangiocarcinomas arise in the bile ducts of the perihilar region; 40% arise in the distal region; and 10% arise in the intrahepatic region.</p><p id="CDR0000062905__3">Many bile duct cancers are multifocal. In most patients, the tumor cannot be completely removed by surgery and is incurable. Palliative measures such as resection, radiation therapy (e.g., brachytherapy or external-beam radiation therapy), or stenting procedures may maintain adequate biliary drainage and allow for improved quality of life. </p><div id="CDR0000062905__4"><h3>Anatomy</h3><p id="CDR0000062905__84">The biliary system consists of a network of ducts that carry bile from the liver to the small bowel and is classified by its anatomic location (Figure <a href="#CDR0000062905__228">1</a>). Bile is produced by the liver and is important for fat digestion.</p><div id="CDR0000062905__349"><h4>Intrahepatic bile duct</h4><p id="CDR0000062905__309">The bile ducts located within the liver are called intrahepatic bile ducts. Tumors of the intrahepatic bile ducts originate in small intrahepatic ductules or large intrahepatic ducts that are proximal to the bifurcation of the right and left hepatic ducts. These tumors are also known as intrahepatic cholangiocarcinomas.</p><div id="CDR0000062905__253" class="figure bk_fig"><div class="graphic"><a href="/core/lw/2.0/html/tileshop_pmc/tileshop_pmc_inline.html?title=Figure%201&p=BOOKS&id=555363_CDR0000765897.jpg" target="tileshopwindow" class="inline_block pmc_inline_block ts_canvas img_link" title="Click on image to zoom"><div class="ts_bar small" title="Click on image to zoom"></div><img src="/books/NBK65869.10/bin/CDR0000765897.jpg" alt="Anatomy of the intrahepatic bile duct; drawing shows the liver, intrahepatic bile ducts, right and left hepatic ducts, gallbladder, pancreas, and small intestine. An inset shows a cross section of a liver lobule with a network of bile ductules leading into a bile duct." class="tileshop" title="Click on image to zoom" /></a></div><div class="caption"><p>Figure 1. Anatomy of the intrahepatic bile duct.</p></div></div></div><div id="CDR0000062905__159"><h4>Extrahepatic bile duct</h4><p id="CDR0000062905__229">The bile ducts located outside of the liver are called extrahepatic bile ducts. They include part of the right and left hepatic ducts that are outside the liver, the common hepatic duct, and the common bile duct. The extrahepatic bile ducts can be further divided into the perihilar (hilum) region and distal region.</p><div id="CDR0000062905__254" class="figure bk_fig"><div class="graphic"><a href="/core/lw/2.0/html/tileshop_pmc/tileshop_pmc_inline.html?title=Figure%202&p=BOOKS&id=555363_CDR0000659742.jpg" target="tileshopwindow" class="inline_block pmc_inline_block ts_canvas img_link" title="Click on image to zoom"><div class="ts_bar small" title="Click on image to zoom"></div><img src="/books/NBK65869.10/bin/CDR0000659742.jpg" alt="Anatomy of the extrahepatic bile ducts; drawing shows the liver, right and left hepatic ducts, gallbladder, cystic duct, common hepatic duct (hilum region), common bile duct (distal region), extrahepatic bile duct, pancreas, and small intestine. An inset shows the liver, bile ducts, and gallbladder." class="tileshop" title="Click on image to zoom" /></a></div><div class="caption"><p>Figure 2. Anatomy of the extrahepatic bile duct.</p></div></div><ul id="CDR0000062905__230"><li class="half_rhythm"><div><b>Perihilar (hilum) region.</b> The hilum is the region where the right and left hepatic ducts exit the liver and join to form the common hepatic duct that is proximal to the origin of the cystic duct. Tumors of this region are also known as perihilar cholangiocarcinomas or Klatskin tumors.</div></li><li class="half_rhythm"><div><b>Distal region.</b> This region includes the common bile duct and inserts into the small intestine. Tumors of this region are also known as extrahepatic cholangiocarcinomas (Figure <a class="figpopup" href="/books/NBK65869.10/figure/CDR0000062905__254/?report=objectonly" target="object" rid-figpopup="figCDR0000062905254" rid-ob="figobCDR0000062905254">2</a>).</div></li></ul></div></div><div id="CDR0000062905__91"><h3>Risk Factors</h3><p id="CDR0000062905__92">Bile duct cancer may occur more frequently in patients with
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a history of primary sclerosing cholangitis, chronic ulcerative colitis,
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choledochal cysts, or infections with the liver fluke <i>Clonorchis sinensis</i>.[<a class="bk_pop" href="#CDR0000062905_rl_1_2">2</a>] </p></div><div id="CDR0000062905__231"><h3>Clinical Features</h3><p id="CDR0000062905__232">Distal and perihilar bile duct cancers frequently cause biliary tract obstruction, leading to the following symptoms: </p><ul id="CDR0000062905__233"><li class="half_rhythm"><div> Jaundice.</div></li><li class="half_rhythm"><div>Weight loss.</div></li><li class="half_rhythm"><div>Abdominal pain.</div></li><li class="half_rhythm"><div>Fever.</div></li><li class="half_rhythm"><div>Pruritus.</div></li></ul><p id="CDR0000062905__234">Intrahepatic bile duct cancer may be relatively indolent and difficult to clinically differentiate from metastatic adenocarcinoma deposits in the liver.</p></div><div id="CDR0000062905__93"><h3>Diagnostic and Staging Evaluation</h3><p id="CDR0000062905__94">Clinical evaluation is dependent on laboratory and radiographic imaging tests that include the following: </p><ul id="CDR0000062905__235"><li class="half_rhythm"><div> Liver function tests and other laboratory studies.</div></li><li class="half_rhythm"><div> Abdominal ultrasound.</div></li><li class="half_rhythm"><div>Computed tomography.</div></li><li class="half_rhythm"><div>Magnetic resonance imaging.</div></li><li class="half_rhythm"><div>Magnetic resonance cholangiopancreatography.</div></li></ul><p id="CDR0000062905__257">These tests demonstrate the extent of the primary tumor and help determine the presence or absence of distant metastases.</p><p id="CDR0000062905__236">If a patient is medically fit for surgery and the tumor is amenable to surgical resection, surgical exploration is performed. Pathologic examination of the resected specimen is done to establish definitive pathologic staging. </p></div><div id="CDR0000062905__7"><h3>Prognosis</h3><p id="CDR0000062905__8">Prognosis depends in part on the tumor’s anatomic location, which affects its resectability. Because of its proximity to major blood vessels and diffuse extension within the liver, a bile duct tumor can be difficult to resect. Total resection is possible in 25% to 30% of lesions that originate in the distal bile duct; the resectability rate is lower for lesions that occur in more proximal sites.[<a class="bk_pop" href="#CDR0000062905_rl_1_3">3</a>] </p><p id="CDR0000062905__151">Complete resection with negative surgical margins offers the only chance of cure for bile duct cancer. For localized, resectable extrahepatic and intrahepatic tumors, the presence of involved lymph nodes and perineural invasion are significant adverse prognostic factors.[<a class="bk_pop" href="#CDR0000062905_rl_1_4">4</a>-<a class="bk_pop" href="#CDR0000062905_rl_1_6">6</a>]</p><p id="CDR0000062905__152">Additionally, the following have been associated with worse outcomes among patients with intrahepatic cholangiocarcinomas:[<a class="bk_pop" href="#CDR0000062905_rl_1_7">7</a>-<a class="bk_pop" href="#CDR0000062905_rl_1_9">9</a>]</p><ul id="CDR0000062905__237"><li class="half_rhythm"><div>A personal history of primary sclerosing cholangitis.</div></li><li class="half_rhythm"><div>Elevated cancer antigen 19-9 level.</div></li><li class="half_rhythm"><div>Periductal infiltrating tumor growth pattern.</div></li><li class="half_rhythm"><div>Presence of hepatic venous invasion.</div></li></ul></div><div id="CDR0000062905__258"><h3>Related Summaries</h3><p id="CDR0000062905__259">Other PDQ summaries containing information related to bile duct cancer include the following:</p><ul id="CDR0000062905__260"><li class="half_rhythm"><div><a href="/books/n/pdqcis/CDR0000062906/">Adult Primary Liver Cancer Treatment</a></div></li></ul></div><div id="CDR0000062905_rl_1"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000062905_rl_1_1">Siegel R, Ma J, Zou Z, et al.: Cancer statistics, 2014. CA Cancer J Clin 64 (1): 9-29, 2014 Jan-Feb. [<a href="https://pubmed.ncbi.nlm.nih.gov/24399786" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24399786</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_1_2">de Groen PC, Gores GJ, LaRusso NF, et al.: Biliary tract cancers. N Engl J Med 341 (18): 1368-78, 1999. [<a href="https://pubmed.ncbi.nlm.nih.gov/10536130" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 10536130</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_1_3">Stain SC, Baer HU, Dennison AR, et al.: Current management of hilar cholangiocarcinoma. Surg Gynecol Obstet 175 (6): 579-88, 1992. [<a href="https://pubmed.ncbi.nlm.nih.gov/1280374" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 1280374</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_1_4">Wakai T, Shirai Y, Moroda T, et al.: Impact of ductal resection margin status on long-term survival in patients undergoing resection for extrahepatic cholangiocarcinoma. Cancer 103 (6): 1210-6, 2005. [<a href="https://pubmed.ncbi.nlm.nih.gov/15685618" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15685618</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_1_5">Klempnauer J, Ridder GJ, von Wasielewski R, et al.: Resectional surgery of hilar cholangiocarcinoma: a multivariate analysis of prognostic factors. J Clin Oncol 15 (3): 947-54, 1997. [<a href="https://pubmed.ncbi.nlm.nih.gov/9060532" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 9060532</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_1_6">Bhuiya MR, Nimura Y, Kamiya J, et al.: Clinicopathologic studies on perineural invasion of bile duct carcinoma. Ann Surg 215 (4): 344-9, 1992. [<a href="/pmc/articles/PMC1242450/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC1242450</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/1558415" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 1558415</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_1_7">Rosen CB, Nagorney DM, Wiesner RH, et al.: Cholangiocarcinoma complicating primary sclerosing cholangitis. Ann Surg 213 (1): 21-5, 1991. [<a href="/pmc/articles/PMC1358305/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC1358305</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/1845927" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 1845927</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_1_8">Shirabe K, Mano Y, Taketomi A, et al.: Clinicopathological prognostic factors after hepatectomy for patients with mass-forming type intrahepatic cholangiocarcinoma: relevance of the lymphatic invasion index. Ann Surg Oncol 17 (7): 1816-22, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/20135355" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20135355</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_1_9">Isa T, Kusano T, Shimoji H, et al.: Predictive factors for long-term survival in patients with intrahepatic cholangiocarcinoma. Am J Surg 181 (6): 507-11, 2001. [<a href="https://pubmed.ncbi.nlm.nih.gov/11513774" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 11513774</span></a>]</div></li></ol></div></div><div id="CDR0000062905__202"><h2 id="_CDR0000062905__202_">Cellular Classification of Bile Duct Cancer</h2><div id="CDR0000062905__211"><h3>Intrahepatic Bile Duct Cancer </h3><p id="CDR0000062905__212">The most common histopathologic types of intrahepatic bile duct tumor include the following:[<a class="bk_pop" href="#CDR0000062905_rl_202_1">1</a>]</p><ul id="CDR0000062905__213"><li class="half_rhythm"><div>Intrahepatic cholangiocarcinoma.</div></li><li class="half_rhythm"><div>Biliary intraepithelial neoplasia, grade 3 (high-grade dysplasia).</div></li><li class="half_rhythm"><div>Combined hepatocellular-cholangiocarcinoma.</div></li><li class="half_rhythm"><div>Carcinosarcoma.</div></li><li class="half_rhythm"><div>Intraductal papillary neoplasm with an associated invasive carcinoma.</div></li><li class="half_rhythm"><div>Mucinous cystic neoplasm with an associated invasive carcinoma.</div></li><li class="half_rhythm"><div>Neuroendocrine carcinoma.</div></li><li class="half_rhythm"><div>Large cell neuroendocrine carcinoma.</div></li><li class="half_rhythm"><div>Small cell neuroendocrine carcinoma.</div></li><li class="half_rhythm"><div>Intraductal papillary neoplasm with high-grade dysplasia.</div></li></ul></div><div id="CDR0000062905__207"><h3>Perihilar Bile Duct Cancer</h3><p id="CDR0000062905__208">Adenocarcinomas are the most common type of perihilar bile duct tumor. The histologic types of perihilar bile duct cancer include the following:[<a class="bk_pop" href="#CDR0000062905_rl_202_2">2</a>]</p><ul id="CDR0000062905__209"><li class="half_rhythm"><div>Carcinoma <i>in situ</i>.</div></li><li class="half_rhythm"><div>Biliary intraepithelial neoplasia, high grade.</div></li><li class="half_rhythm"><div>Intraductal papillary neoplasm with high-grade dysplasia.</div></li><li class="half_rhythm"><div>Mucinous cystic neoplasm with high-grade intraepithelial neoplasia.</div></li><li class="half_rhythm"><div>Adenocarcinoma.</div></li><li class="half_rhythm"><div>Adenocarcinoma, biliary type.</div></li><li class="half_rhythm"><div>Adenocarcinoma, gastric foveolar type</div></li><li class="half_rhythm"><div>Adenocarcinoma, intestinal type.
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</div></li><li class="half_rhythm"><div>Clear cell adenocarcinoma.</div></li><li class="half_rhythm"><div>Mucinous carcinoma.
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</div></li><li class="half_rhythm"><div>Signet-ring cell carcinoma.
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</div></li><li class="half_rhythm"><div>Squamous cell carcinoma.</div></li><li class="half_rhythm"><div>Adenosquamous carcinoma.
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</div></li><li class="half_rhythm"><div>Undifferentiated carcinoma.</div></li><li class="half_rhythm"><div>High-grade neuroendocrine carcinoma.</div></li><li class="half_rhythm"><div>Small cell neuroendocrine carcinoma.</div></li><li class="half_rhythm"><div>Intraductal papillary neoplasm with an associated invasive carcinoma.</div></li><li class="half_rhythm"><div>Mucinous cystic neoplasm with an associated invasive carcinoma.</div></li></ul></div><div id="CDR0000062905__203"><h3>Distal Bile Duct Cancer</h3><p id="CDR0000062905__204">Adenocarcinomas are the most common type of distal bile duct tumors. The histologic types of distal bile duct cancer include the following:[<a class="bk_pop" href="#CDR0000062905_rl_202_3">3</a>]</p><ul id="CDR0000062905__205"><li class="half_rhythm"><div>Carcinoma <i>in situ</i>.</div></li><li class="half_rhythm"><div>Biliary intraepithelial neoplasia, high grade.</div></li><li class="half_rhythm"><div>Intraductal papillary neoplasm with high-grade intraepithelial neoplasia.</div></li><li class="half_rhythm"><div>Mucinous cystic neoplasm with high-grade intraepithelial neoplasia.</div></li><li class="half_rhythm"><div>Adenocarcinoma.</div></li><li class="half_rhythm"><div>Adenocarcinoma, biliary type.</div></li><li class="half_rhythm"><div>Adenocarcinoma, intestinal type.
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</div></li><li class="half_rhythm"><div>Adenocarcinoma, gastric foveolar type.</div></li><li class="half_rhythm"><div>Mucinous adenocarcinoma.</div></li><li class="half_rhythm"><div>Clear cell adenocarcinoma.
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</div></li><li class="half_rhythm"><div>Signet-ring cell carcinoma.
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</div></li><li class="half_rhythm"><div>Squamous cell carcinoma.</div></li><li class="half_rhythm"><div>Adenosquamous carcinoma.
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</div></li><li class="half_rhythm"><div>Undifferentiated carcinoma.</div></li><li class="half_rhythm"><div>High-grade neuroendocrine carcinoma.</div></li><li class="half_rhythm"><div>Small cell neuroendocrine carcinoma.</div></li><li class="half_rhythm"><div>Mixed adenoneuroendocrine carcinoma.</div></li><li class="half_rhythm"><div>Intraductal papillary neoplasm with an associated invasive carcinoma.</div></li><li class="half_rhythm"><div>Mucinous cystic neoplasm with an associated invasive carcinoma.</div></li></ul></div><div id="CDR0000062905_rl_202"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000062905_rl_202_1">Intrahepatic Bile Ducts. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 295–302.</div></li><li><div class="bk_ref" id="CDR0000062905_rl_202_2">Perihilar bile ducts. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 311–16.</div></li><li><div class="bk_ref" id="CDR0000062905_rl_202_3">Distal bile duct. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 317–25.</div></li></ol></div></div><div id="CDR0000062905__25"><h2 id="_CDR0000062905__25_">Stage Information for Bile Duct Cancer</h2><div id="CDR0000062905__81"><h3>Staging for Bile Duct Cancer</h3><p id="CDR0000062905__82">Bile duct cancer is classified as resectable (localized) or unresectable, with obvious prognostic importance. The TNM (tumor, node, metastasis) staging system is used for staging bile duct cancer, commonly after surgery and pathologic examination of the resected specimen. Evaluation of the extent of disease at laparotomy is an important component of staging.</p></div><div id="CDR0000062905__26"><h3>AJCC Staging System for Bile Duct Cancer </h3><div id="CDR0000062905__47"><h4>AJCC staging system for intrahepatic bile duct cancer</h4><p id="CDR0000062905__50">The AJCC has designated staging by TNM classification to define intrahepatic bile duct cancer.[<a class="bk_pop" href="#CDR0000062905_rl_25_1">1</a>]</p><p id="CDR0000062905__55">Tables <a class="figpopup" href="/books/NBK65869.10/table/CDR0000062905__353/?report=objectonly" target="object" rid-figpopup="figCDR0000062905353" rid-ob="figobCDR0000062905353">1</a>, <a class="figpopup" href="/books/NBK65869.10/table/CDR0000062905__381/?report=objectonly" target="object" rid-figpopup="figCDR0000062905381" rid-ob="figobCDR0000062905381">2</a>, <a class="figpopup" href="/books/NBK65869.10/table/CDR0000062905__356/?report=objectonly" target="object" rid-figpopup="figCDR0000062905356" rid-ob="figobCDR0000062905356">3</a>, <a class="figpopup" href="/books/NBK65869.10/table/CDR0000062905__379/?report=objectonly" target="object" rid-figpopup="figCDR0000062905379" rid-ob="figobCDR0000062905379">4</a>, and <a class="figpopup" href="/books/NBK65869.10/table/CDR0000062905__359/?report=objectonly" target="object" rid-figpopup="figCDR0000062905359" rid-ob="figobCDR0000062905359">5</a> pertain to the intrahepatic bile duct cancer stages.</p><div id="CDR0000062905__353" class="table"><h3><span class="title">Table 1. Definitions of TNM Stage 0 Intrahepatic Bile Duct Cancer<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.10/table/CDR0000062905__353/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__353_lrgtbl__"><table class="no_margin"><thead><tr><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">Stage</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">TNM</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">Description</th></tr></thead><tbody><tr><td colspan="1" rowspan="3" style="vertical-align:top;">0</td><td colspan="1" rowspan="3" style="vertical-align:top;">Tis, N0, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">Tis = Carcinoma <i>in situ</i> (intraductal tumor).</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0 = No regional lymph node metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin">T = primary tumor; N = regional lymph node; M = distant metastasis.</p></div></dd><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Intrahepatic Bile Duct. In: Amin MB, Edge SB, Greene FL, et al., eds.: <i>AJCC Cancer Staging Manual</i>. 8th ed. New York, NY: Springer, 2017, pp. 295–302.</p></div></dd></dl></div></div></div><div id="CDR0000062905__381" class="table"><h3><span class="title">Table 2. Definitions of TNM Stage I Intrahepatic Bile Duct Cancer<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.10/table/CDR0000062905__381/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__381_lrgtbl__"><table class="no_margin"><thead><tr><th colspan="2" rowspan="1" style="vertical-align:top;">Stage</th><th colspan="1" rowspan="1" style="vertical-align:top;">TNM</th><th colspan="1" rowspan="1" style="vertical-align:top;">Description</th></tr></thead><tbody><tr><td colspan="1" rowspan="6" style="vertical-align:top;">I</td><td colspan="1" rowspan="3" style="vertical-align:top;">IA</td><td colspan="1" rowspan="3" style="vertical-align:top;">T1a, N0, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">T1a = Solitary tumor ≤5 cm without vascular invasion.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0 = No regional lymph node metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr><tr><td colspan="1" rowspan="3" style="vertical-align:top;">IB</td><td colspan="1" rowspan="3" style="vertical-align:top;">T1b, N0, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">T1b = Solitary tumor >5 cm without vascular invasion.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0 = No regional lymph node metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin">T = primary tumor; N = regional lymph node; M = distant metastasis.</p></div></dd><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Intrahepatic Bile Duct. In: Amin MB, Edge SB, Greene FL, et al., eds.: <i>AJCC Cancer Staging Manual</i>. 8th ed. New York, NY: Springer, 2017, pp. 295–302.</p></div></dd></dl></div></div></div><div id="CDR0000062905__356" class="table"><h3><span class="title">Table 3. Definitions of TNM Stage II Intrahepatic Bile Duct Cancer<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.10/table/CDR0000062905__356/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__356_lrgtbl__"><table class="no_margin"><thead><tr><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">Stage</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">TNM</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">Description</th></tr></thead><tbody><tr><td colspan="1" rowspan="3" style="vertical-align:top;">II</td><td colspan="1" rowspan="3" style="vertical-align:top;">T2, N0, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">T2 = Solitary tumor with intrahepatic vascular invasion or multiple tumors, with or without vascular invasion.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0 = No regional lymph node metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin">T = primary tumor; N = regional lymph node; M = distant metastasis.</p></div></dd><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Intrahepatic Bile Duct. In: Amin MB, Edge SB, Greene FL, et al., eds.: <i>AJCC Cancer Staging Manual</i>. 8th ed. New York, NY: Springer, 2017, pp. 295–302.</p></div></dd></dl></div></div></div><div id="CDR0000062905__379" class="table"><h3><span class="title">Table 4. Definitions of TNM Stage III Intrahepatic Bile Duct Cancer<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.10/table/CDR0000062905__379/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__379_lrgtbl__"><table class="no_margin"><thead><tr><th colspan="2" rowspan="1" style="vertical-align:top;">Stage</th><th colspan="1" rowspan="1" style="vertical-align:top;">TNM</th><th colspan="1" rowspan="1" style="vertical-align:top;">Description</th></tr></thead><tbody><tr><td colspan="1" rowspan="17" style="vertical-align:top;">III</td><td colspan="1" rowspan="3" style="vertical-align:top;">IIIA</td><td colspan="1" rowspan="3" style="vertical-align:top;">T3, N0, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">T3 =Tumor perforating the visceral peritoneum.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0 = No regional lymph node metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr><tr><td colspan="1" rowspan="14" style="vertical-align:top;">IIIB</td><td colspan="1" rowspan="3" style="vertical-align:top;">T4, N0, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">T4 = Tumor involving local extrahepatic structures by direct invasion.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0 = No regional lymph node metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr><tr><td colspan="1" rowspan="11" style="vertical-align:top;">Any T, N1, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">TX = Primary tumor cannot be assessed.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T0 = No evidence of primary tumor.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">Tis = Carcinoma<i> in situ</i> (intraductal tumor).</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T1 = Solitary tumor without vascular invasion, ≤5 cm or >5 cm.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">–T1a = Solitary tumor ≤5 cm without vascular invasion.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">–T1b = Solitary tumor >5 cm without vascular invasion.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T2 = Solitary tumor with intrahepatic vascular invasion or multiple tumors, with or without vascular invasion.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T3 = Tumor perforating the visceral peritoneum.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T4 = Tumor involving local extrahepatic structures by direct invasion.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N1 = Regional lymph node metastasis present.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin">T = primary tumor; N = regional lymph node; M = distant metastasis.</p></div></dd><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Intrahepatic Bile Duct. In: Amin MB, Edge SB, Greene FL, et al., eds.: <i>AJCC Cancer Staging Manual</i>. 8th ed. New York, NY: Springer, 2017, pp. 295–302.</p></div></dd></dl></div></div></div><div id="CDR0000062905__359" class="table"><h3><span class="title">Table 5. Definitions of TNM Stage IV Intrahepatic Bile Duct Cancer<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.10/table/CDR0000062905__359/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__359_lrgtbl__"><table class="no_margin"><thead><tr><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">Stage</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">TNM</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">Description</th></tr></thead><tbody><tr><td colspan="1" rowspan="13" style="vertical-align:top;">IV</td><td colspan="1" rowspan="13" style="vertical-align:top;">Any T, Any N, M1</td><td colspan="1" rowspan="1" style="vertical-align:top;">TX = Primary tumor cannot be assessed.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T0 = No evidence of primary tumor.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">Tis = Carcinoma<i> in situ</i> (intraductal tumor).</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T1 = Solitary tumor without vascular invasion, ≤5 cm or >5 cm.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">–T1a = Solitary tumor ≤5 cm without vascular invasion.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">–T1b = Solitary tumor >5 cm without vascular invasion.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T2 = Solitary tumor with intrahepatic vascular invasion or multiple tumors, with or without vascular invasion.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T3 = Tumor perforating the visceral peritoneum.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T4 = Tumor involving local extrahepatic structures by direct invasion.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">NX = Regional lymph nodes cannot be assessed.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0 = No regional lymph node metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N1 = Regional lymph node metastasis present.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M1 = Distant metastasis present.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin">T = primary tumor; N = regional lymph node; M = distant metastasis.</p></div></dd><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Intrahepatic Bile Duct. In: Amin MB, Edge SB, Greene FL, et al., eds.: <i>AJCC Cancer Staging Manual</i>. 8th ed. New York, NY: Springer, 2017, pp. 295–302.</p></div></dd></dl></div></div></div></div><div id="CDR0000062905__133"><h4>AJCC staging system for perihilar bile duct cancer</h4><p id="CDR0000062905__134">The AJCC has designated staging by TNM classification to define perihilar bile duct cancer.[<a class="bk_pop" href="#CDR0000062905_rl_25_2">2</a>]</p><p id="CDR0000062905__154">Tables <a class="figpopup" href="/books/NBK65869.10/table/CDR0000062905__360/?report=objectonly" target="object" rid-figpopup="figCDR0000062905360" rid-ob="figobCDR0000062905360">6</a>, <a class="figpopup" href="/books/NBK65869.10/table/CDR0000062905__281/?report=objectonly" target="object" rid-figpopup="figCDR0000062905281" rid-ob="figobCDR0000062905281">7</a>, <a class="figpopup" href="/books/NBK65869.10/table/CDR0000062905__361/?report=objectonly" target="object" rid-figpopup="figCDR0000062905361" rid-ob="figobCDR0000062905361">8</a>, <a class="figpopup" href="/books/NBK65869.10/table/CDR0000062905__380/?report=objectonly" target="object" rid-figpopup="figCDR0000062905380" rid-ob="figobCDR0000062905380">9</a>, and <a class="figpopup" href="/books/NBK65869.10/table/CDR0000062905__364/?report=objectonly" target="object" rid-figpopup="figCDR0000062905364" rid-ob="figobCDR0000062905364">10</a> pertain to the perihilar bile duct cancer stages.</p><div id="CDR0000062905__360" class="table"><h3><span class="title">Table 6. Definitions of TNM Stage 0 Perihilar Bile Duct Cancer<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.10/table/CDR0000062905__360/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__360_lrgtbl__"><table class="no_margin"><thead><tr><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">Stage</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">TNM</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">Description</th></tr></thead><tbody><tr><td colspan="1" rowspan="3" style="vertical-align:top;">0</td><td colspan="1" rowspan="3" style="vertical-align:top;">Tis, N0, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">Tis = Carcinoma <i>in situ</i>/high-grade dysplasia.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0 = No regional lymph node metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin">T = primary tumor; N = regional lymph node; M = distant metastasis.</p></div></dd><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Perihilar Bile Ducts. In: Amin MB, Edge SB, Greene FL, et al., eds.: <i>AJCC Cancer Staging Manual</i>. 8th ed. New York, NY: Springer, 2017, pp. 311–6.</p></div></dd></dl></div></div></div><div id="CDR0000062905__281" class="table"><h3><span class="title">Table 7. Definitions of TNM Stage I Perihilar Bile Duct Cancer<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.10/table/CDR0000062905__281/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__281_lrgtbl__"><table class="no_margin"><thead><tr><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">Stage</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">TNM</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">Description</th></tr></thead><tbody><tr><td colspan="1" rowspan="3" style="vertical-align:top;">I</td><td colspan="1" rowspan="3" style="vertical-align:top;">T1, N0, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">T1 = Tumor confined to the bile duct, with extension up to the muscle layer or fibrous tissue. </td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0 = No regional lymph node metastasis. </td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin">T = primary tumor; N = regional lymph node; M = distant metastasis.</p></div></dd><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Perihilar Bile Ducts. In: Amin MB, Edge SB, Greene FL, et al., eds.: <i>AJCC Cancer Staging Manual</i>. 8th ed. New York, NY: Springer, 2017, pp. 311–6.</p></div></dd></dl></div></div></div><div id="CDR0000062905__361" class="table"><h3><span class="title">Table 8. Definitions of TNM Stage II Perihilar Bile Duct Cancer<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.10/table/CDR0000062905__361/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__361_lrgtbl__"><table class="no_margin"><thead><tr><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">Stage </th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">TNM</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">Description</th></tr></thead><tbody><tr><td colspan="1" rowspan="5" style="vertical-align:top;">II</td><td colspan="1" rowspan="5" style="vertical-align:top;">T2a–b, N0, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">T2 = Tumor invades beyond the wall of the bile duct to surrounding adipose tissue, or tumor invades adjacent hepatic parenchyma.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">–T2a = Tumor invades beyond the wall of the bile duct to surrounding adipose tissue.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">–T2b = Tumor invades adjacent hepatic parenchyma.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0 = No regional lymph node metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin">T = primary tumor; N = regional lymph node; M = distant metastasis.</p></div></dd><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Perihilar Bile Ducts. In: Amin MB, Edge SB, Greene FL, et al., eds.: <i>AJCC Cancer Staging Manual</i>. 8th ed. New York, NY: Springer, 2017, pp. 311–6.</p></div></dd></dl></div></div></div><div id="CDR0000062905__380" class="table"><h3><span class="title">Table 9. Definitions of TNM Stage III Perihilar Bile Duct Cancer<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.10/table/CDR0000062905__380/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__380_lrgtbl__"><table class="no_margin"><thead><tr><th colspan="2" rowspan="1" style="vertical-align:top;">Stage</th><th colspan="1" rowspan="1" style="vertical-align:top;">TNM</th><th colspan="1" rowspan="1" style="vertical-align:top;">Description</th></tr></thead><tbody><tr><td colspan="1" rowspan="17" style="vertical-align:top;">III</td><td colspan="1" rowspan="3" style="vertical-align:top;">IIIA</td><td colspan="1" rowspan="3" style="vertical-align:top;">T3, N0, M0 </td><td colspan="1" rowspan="1" style="vertical-align:top;">T3 = Tumor invades unilateral branches of the portal vein or hepatic artery. </td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0 = No regional lymph node metastasis. </td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr><tr><td colspan="1" rowspan="3" style="vertical-align:top;">IIIB</td><td colspan="1" rowspan="3" style="vertical-align:top;">T4, N0, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">T4 = Tumor invades the main portal vein or its branches bilaterally, or the common hepatic artery; or unilateral second-order biliary radicals with contralateral portal vein or hepatic artery involvement.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0 = No regional lymph node metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr><tr><td colspan="1" rowspan="11" style="vertical-align:top;">IIIC</td><td colspan="1" rowspan="11" style="vertical-align:top;">Any T, N1, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">TX = Primary tumor cannot be assessed.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T0 = No evidence of primary tumor.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">Tis = Carcinoma <i>in situ</i>/high-grade dysplasia.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T1 = Tumor confined to the bile duct, with extension up to the muscle layer or fibrous tissue.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T2 = Tumor invades beyond the wall of the bile duct to surrounding adipose tissue, or tumor invades adjacent hepatic parenchyma.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">–T2a = Tumor invades beyond the wall of the bile duct to surrounding adipose tissue.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">–T2b = Tumor invades adjacent hepatic parenchyma.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T3 = Tumor invades unilateral branches of the portal vein or hepatic artery.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T4 = Tumor invades the main portal vein or its branches bilaterally, or the common hepatic artery; or unilateral second-order biliary radicals with contralateral portal vein or hepatic artery involvement.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N1 = One to three positive lymph nodes typically involving the hilar, cystic duct, common bile duct, hepatic artery, posterior pancreatoduodenal, and portal vein lymph nodes.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin">T = primary tumor; N = regional lymph node; M = distant metastasis.</p></div></dd><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Perihilar Bile Ducts. In: Amin MB, Edge SB, Greene FL, et al., eds.: <i>AJCC Cancer Staging Manual</i>. 8th ed. New York, NY: Springer, 2017, pp. 311–6.</p></div></dd></dl></div></div></div><div id="CDR0000062905__364" class="table"><h3><span class="title">Table 10. Definitions of TNM Stage IV Perihilar Bile Duct Cancer<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.10/table/CDR0000062905__364/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__364_lrgtbl__"><table class="no_margin"><thead><tr><th colspan="2" rowspan="1" style="text-align:center;vertical-align:top;">Stage</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">TNM</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">Description</th></tr></thead><tbody><tr><td colspan="1" rowspan="25" style="vertical-align:top;">IV</td><td colspan="1" rowspan="11" style="vertical-align:top;">IVA</td><td colspan="1" rowspan="11" style="vertical-align:top;">Any T, N2, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">TX = Primary tumor cannot be assessed.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T0 = No evidence of primary tumor.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">Tis = Carcinoma <i>in situ</i>/high-grade dysplasia.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T1 = Tumor confined to the bile duct, with extension up to the muscle layer or fibrous tissue.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T2 = Tumor invades beyond the wall of the bile duct to surrounding adipose tissue, or tumor invades adjacent hepatic parenchyma.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">–T2a = Tumor invades beyond the wall of the bile duct to surround adipose tissue.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">–T2b = Tumor invades adjacent hepatic parenchyma.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T3 = Tumor invades unilateral branches of the portal vein or hepatic artery.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T4 = Tumor invades the main portal vein or its branches bilaterally, or the common hepatic artery; or unilateral second-order biliary radicals with contralateral portal vein or hepatic artery involvement.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N2 = Four or more positive lymph nodes from the sites described for N1.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr><tr><td colspan="1" rowspan="14" style="vertical-align:top;">IVB</td><td colspan="1" rowspan="14" style="vertical-align:top;">Any T, Any N, M1</td><td colspan="1" rowspan="1" style="vertical-align:top;">TX = Primary tumor cannot be assessed.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T0 = No evidence of primary tumor.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">Tis = Carcinoma <i>in situ</i>/high-grade dysplasia.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T1 = Tumor confined to the bile duct, with extension up to the muscle layer or fibrous tissue.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T2 = Tumor invades beyond the wall of the bile duct to surrounding adipose tissue, or tumor invades adjacent hepatic parenchyma.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">–T2a = Tumor invades beyond the wall of the bile duct to surround adipose tissue.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">–T2b = Tumor invades adjacent hepatic parenchyma.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T3 = Tumor invades unilateral branches of the portal vein or hepatic artery.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T4 = Tumor invades the main portal vein or its branches bilaterally, or the common hepatic artery; or unilateral second-order biliary radicals with contralateral portal vein or hepatic artery involvement.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">NX = Regional lymph nodes cannot be assessed.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0 = No regional lymph node metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N1 = One to three positive lymph nodes typically involving the hilar, cystic duct, common bile duct, hepatic artery, posterior pancreatoduodenal, and portal vein lymph nodes.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N2 = Four or more positive lymph nodes from the sites described for N1.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M1 = Distant metastasis.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin">T = primary tumor; N = regional lymph node; M = distant metastasis.</p></div></dd><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Perihilar Bile Ducts. In: Amin MB, Edge SB, Greene FL, et al., eds.: <i>AJCC Cancer Staging Manual</i>. 8th ed. New York, NY: Springer, 2017, pp. 311–6.</p></div></dd></dl></div></div></div></div><div id="CDR0000062905__34"><h4>AJCC staging system for distal bile duct cancer</h4><p id="CDR0000062905__35">The AJCC has designated staging by TNM classification to define distal bile duct cancer.[<a class="bk_pop" href="#CDR0000062905_rl_25_3">3</a>] Stages defined by TNM classification apply to all primary carcinomas arising in the distal bile duct or in the cystic duct; these stages do not apply to perihilar or intrahepatic cholangiocarcinomas, sarcomas, or carcinoid tumors.</p><p id="CDR0000062905__42">Tables <a class="figpopup" href="/books/NBK65869.10/table/CDR0000062905__366/?report=objectonly" target="object" rid-figpopup="figCDR0000062905366" rid-ob="figobCDR0000062905366">11</a>, <a class="figpopup" href="/books/NBK65869.10/table/CDR0000062905__367/?report=objectonly" target="object" rid-figpopup="figCDR0000062905367" rid-ob="figobCDR0000062905367">12</a>, <a class="figpopup" href="/books/NBK65869.10/table/CDR0000062905__368/?report=objectonly" target="object" rid-figpopup="figCDR0000062905368" rid-ob="figobCDR0000062905368">13</a>, <a class="figpopup" href="/books/NBK65869.10/table/CDR0000062905__370/?report=objectonly" target="object" rid-figpopup="figCDR0000062905370" rid-ob="figobCDR0000062905370">14</a>, <a class="figpopup" href="/books/NBK65869.10/table/CDR0000062905__372/?report=objectonly" target="object" rid-figpopup="figCDR0000062905372" rid-ob="figobCDR0000062905372">15</a> pertain to the distal bile duct cancer stages.</p><div id="CDR0000062905__366" class="table"><h3><span class="title">Table 11. Definitions of TNM Stage 0 Distal Bile Duct Cancer<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.10/table/CDR0000062905__366/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__366_lrgtbl__"><table class="no_margin"><thead><tr><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">Stage </th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">TNM</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">Definition</th></tr></thead><tbody><tr><td colspan="1" rowspan="3" style="vertical-align:top;">0</td><td colspan="1" rowspan="3" style="vertical-align:top;">Tis, N0, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">Tis = Carcinoma <i>in situ</i>/high-grade dysplasia.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0 = No regional lymph node metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin">T = primary tumor; N = regional lymph node; M = distant metastasis.</p></div></dd><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Distal Bile Duct. In: Amin MB, Edge SB, Greene FL, et al., eds.: <i>AJCC Cancer Staging Manual</i>. 8th ed. New York, NY: Springer, 2017, pp. 317–325.</p></div></dd></dl></div></div></div><div id="CDR0000062905__367" class="table"><h3><span class="title">Table 12. Definitions of TNM Stage I Distal Bile Duct Cancer<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.10/table/CDR0000062905__367/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__367_lrgtbl__"><table class="no_margin"><thead><tr><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">Stage </th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">TNM</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">Definition</th></tr></thead><tbody><tr><td colspan="1" rowspan="3" style="vertical-align:top;">I</td><td colspan="1" rowspan="3" style="vertical-align:top;">T1, N0, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">T1 = Tumor invades the bile duct wall with a depth <5 mm.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0 = No regional lymph node metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin">T = primary tumor; N = regional lymph node; M = distant metastasis.</p></div></dd><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Distal Bile Duct. In: Amin MB, Edge SB, Greene FL, et al., eds.: <i>AJCC Cancer Staging Manual</i>. 8th ed. New York, NY: Springer, 2017, pp. 317–325.</p></div></dd></dl></div></div></div><div id="CDR0000062905__368" class="table"><h3><span class="title">Table 13. Definitions of TNM IIA Distal Bile Duct Cancer<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.10/table/CDR0000062905__368/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__368_lrgtbl__"><table class="no_margin"><thead><tr><th colspan="2" rowspan="1" style="text-align:center;vertical-align:top;">Stage</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">TNM</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">Definition</th></tr></thead><tbody><tr><td colspan="1" rowspan="15" style="vertical-align:top;">II</td><td colspan="1" rowspan="6" style="vertical-align:top;">IIA</td><td colspan="1" rowspan="3" style="vertical-align:top;">T1, N1, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">T1 = Tumor invades the bile duct wall with a depth <5 mm.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N1 = Metastasis in one to three regional lymph nodes.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr><tr><td colspan="1" rowspan="3" style="vertical-align:top;">T2, N0, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">Tumor invades the bile duct wall with a depth of 5–12 mm.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0 = No regional lymph node metastasis. </td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr><tr><td colspan="1" rowspan="9" style="vertical-align:top;">IIB</td><td colspan="1" rowspan="3" style="vertical-align:top;">T2, N1, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">T2 = Tumor invades the bile duct wall with a depth of 5–12 mm.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N1 = Metastasis in one to three regional lymph nodes.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr><tr><td colspan="1" rowspan="3" style="vertical-align:top;">T3, N0, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">T3 = Tumor invades the bile duct wall with a depth >12 mm.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0 = No regional lymph node metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr><tr><td colspan="1" rowspan="3" style="vertical-align:top;">T3, N1, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">T3 = Tumor invades the bile duct wall with a depth >12 mm.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N1 = Metastasis in one to three regional lymph nodes.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin">T = primary tumor; N = regional lymph node; M = distant metastasis.</p></div></dd><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Distal Bile Duct. In: Amin MB, Edge SB, Greene FL, et al., eds.: <i>AJCC Cancer Staging Manual</i>. 8th ed. New York, NY: Springer, 2017, pp. 317–325.</p></div></dd></dl></div></div></div><div id="CDR0000062905__370" class="table"><h3><span class="title">Table 14. Definitions of TNM Stage IIIA Distal Bile Duct Cancer</span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.10/table/CDR0000062905__370/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__370_lrgtbl__"><table class="no_margin"><thead><tr><th colspan="2" rowspan="1" style="text-align:center;vertical-align:top;">Stage</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">TNM</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">Definition</th></tr></thead><tbody><tr><td colspan="1" rowspan="18" style="vertical-align:top;">III</td><td colspan="1" rowspan="9" style="vertical-align:top;">IIIA</td><td colspan="1" rowspan="3" style="vertical-align:top;">T1, N2, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">T1 = Tumor invades the bile duct wall with a depth <5 mm.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N2 = Metastasis in four or more regional lymph nodes.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr><tr><td colspan="1" rowspan="3" style="vertical-align:top;">T2, N2, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">T2 = Tumor invades the bile duct wall with a depth of 5–12 mm.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N2 = Metastasis in four or more regional lymph nodes.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr><tr><td colspan="1" rowspan="3" style="vertical-align:top;">T3, N2, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">T3 = Tumor invades the bile duct wall with a depth >12 mm.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N2 = Metastasis in four or more regional lymph nodes.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr><tr><td colspan="1" rowspan="9" style="vertical-align:top;">IIIB</td><td colspan="1" rowspan="3" style="vertical-align:top;">T4, N0, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">T4 = Tumor involves the celiac axis, superior mesenteric artery, and/or common hepatic artery.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0 = No regional lymph node metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr><tr><td colspan="1" rowspan="3" style="vertical-align:top;">T4, N1, M0 </td><td colspan="1" rowspan="1" style="vertical-align:top;">T4 = Tumor involves the celiac axis, superior mesenteric artery, and/or common hepatic artery.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N1 = Metastasis in one to three regional lymph nodes.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr><tr><td colspan="1" rowspan="3" style="vertical-align:top;">T4, N2, M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">T4 = Tumor involves the celiac axis, superior mesenteric artery, and/or common hepatic artery.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N2 = Metastasis in four or more regional lymph nodes.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0 = No distant metastasis.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin">T = primary tumor; N = regional lymph node; M = distant metastasis.</p></div></dd><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Distal Bile Duct. In: Amin MB, Edge SB, Greene FL, et al., eds.: <i>AJCC Cancer Staging Manual</i>. 8th ed. New York, NY: Springer, 2017, pp. 317–325.</p></div></dd></dl></div></div></div><div id="CDR0000062905__372" class="table"><h3><span class="title">Table 15. Definitions of TNM Stage IV Distal Bile Duct Cancer<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.10/table/CDR0000062905__372/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__372_lrgtbl__"><table class="no_margin"><thead><tr><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">Stage</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">TNM</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">Definition</th></tr></thead><tbody><tr><td colspan="1" rowspan="11" style="vertical-align:top;">IV</td><td colspan="1" rowspan="11" style="vertical-align:top;">Any T, Any N, M1</td><td colspan="1" rowspan="1" style="vertical-align:top;">TX = Primary tumor cannot be assessed.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">TIS = Carcinoma <i>in situ</i>/high-grade dysplasia.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T1 = Tumor invades the bile duct wall with a depth <5 mm.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T2 = Tumor invades the bile duct wall with a depth of 5–12 mm.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T3 = Tumor invades the bile duct wall with a depth >12 mm.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T4 = Tumor involves the celiac axis, superior mesenteric artery, and/or common hepatic artery.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">NX = Regional lymph nodes cannot be assessed.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0 = No regional lymph node metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N1 = Metastasis in one to three regional lymph nodes.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N2 = Metastasis in four or more regional lymph nodes.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M1 = Distant metastasis.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin">T = primary tumor; N = regional lymph node; M = distant metastasis.</p></div></dd><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Distal Bile Duct. In: Amin MB, Edge SB, Greene FL, et al., eds.: <i>AJCC Cancer Staging Manual</i>. 8th ed. New York, NY: Springer, 2017, pp. 317–325.</p></div></dd></dl></div></div></div></div></div><div id="CDR0000062905_rl_25"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000062905_rl_25_1">Intrahepatic Bile Ducts. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 295–302.</div></li><li><div class="bk_ref" id="CDR0000062905_rl_25_2">Perihilar Bile Ducts. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 311–6.</div></li><li><div class="bk_ref" id="CDR0000062905_rl_25_3">Distal bile duct. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 317–25.</div></li></ol></div></div><div id="CDR0000062905__261"><h2 id="_CDR0000062905__261_">Treatment Option Overview for Bile Duct Cancer</h2><p id="CDR0000062905__271">The treatment of bile duct cancer depends primarily on whether the cancer can be completely removed by surgery.</p><div id="CDR0000062905__28"><h3>Resectable (Localized) Bile Duct Cancer</h3><p id="CDR0000062905__29">Localized intrahepatic and extrahepatic bile duct cancer may be completely removed by surgery. These tumors represent a very small number of cases that are usually in the distal common bile duct. Among patients treated with surgical resection, long-term prognosis varies depending on primary tumor extent, margin status, lymph node involvement, and additional pathologic features.[<a class="bk_pop" href="#CDR0000062905_rl_261_1">1</a>,<a class="bk_pop" href="#CDR0000062905_rl_261_2">2</a>]</p><p id="CDR0000062905__238">Extended resections of hepatic duct bifurcation tumors (Klatskin tumors, also known as hilar tumors) to include adjacent liver, either by lobectomy or removal of portions of segments 4 and 5 of the liver, may be performed. If major hepatic resection is necessary to achieve a complete resection, postoperative hepatic reserve should be evaluated. For patients with underlying cirrhosis, the Child-Pugh class and the Model for End-Stage Liver Disease score are determined. </p></div><div id="CDR0000062905__30"><h3>Unresectable (Including Metastatic and Recurrent) Bile Duct Cancer</h3><p id="CDR0000062905__265">Most cases of intrahepatic, distal, and perihilar bile duct cancer are unresectable and
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cannot be completely removed by the surgeon. Often the cancer invades directly into the portal vein, the adjacent liver, along the common bile duct, and to adjacent lymph nodes. Portal hypertension may result from invasion of the portal vein. Spread to distant parts of the body is
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uncommon, but intra-abdominal metastases, particularly peritoneal metastases, do occur. Transperitoneal and hematogenous hepatic metastases also occur
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with bile duct cancer of all sites. Moreover, most patients who undergo resection will develop recurrent disease within the hepatobiliary system or, less frequently, at distant sites.</p><p id="CDR0000062905__432">In locally advanced disease, phase II trials have evaluated chemoradiotherapy with the goal of improved local control and potential downstaging for surgical resection.[<a class="bk_pop" href="#CDR0000062905_rl_261_3">3</a>,<a class="bk_pop" href="#CDR0000062905_rl_261_4">4</a>] These approaches have not been compared with standard therapy, and the curative potential is unknown.</p><p id="CDR0000062905__239">For patients with unresectable bile duct cancer, management is directed at palliation.</p><p id="CDR0000062905__262">Treatment options for bile duct cancer are described in Table <a class="figpopup" href="/books/NBK65869.10/table/CDR0000062905__499/?report=objectonly" target="object" rid-figpopup="figCDR0000062905499" rid-ob="figobCDR0000062905499">16</a>. </p><div id="CDR0000062905__499" class="table"><h3><span class="title">Table 16. Treatment Options for Bile Duct Cancer</span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.10/table/CDR0000062905__499/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__499_lrgtbl__"><table class="no_top_margin"><thead><tr><th colspan="1" rowspan="1" style="vertical-align:top;">Staging Criteria</th><th colspan="1" rowspan="1" style="vertical-align:top;">Treatment Options</th></tr></thead><tbody><tr><td colspan="1" rowspan="2" style="vertical-align:top;">Resectable (Localized) Bile Duct Cancer</td><td colspan="1" rowspan="1" style="vertical-align:top;"><a href="#CDR0000062905__440">Surgery</a></td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">
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<a href="#CDR0000062905__476">Adjuvant therapy</a></td></tr><tr><td colspan="1" rowspan="4" style="vertical-align:top;">Unresectable (Including Metastatic and Recurrent) Bile Duct Cancer </td><td colspan="1" rowspan="1" style="vertical-align:top;"><a href="#CDR0000062905__447">Palliative therapy</a></td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;"><a href="#CDR0000062905__450">Chemotherapy</a></td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;"><a href="#CDR0000062905__458">Immunotherapy</a></td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;"><a href="#CDR0000062905__460">Targeted therapy</a></td></tr></tbody></table></div></div></div><div id="CDR0000062905_rl_261"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000062905_rl_261_1">Nagorney DM, Donohue JH, Farnell MB, et al.: Outcomes after curative resections of cholangiocarcinoma. Arch Surg 128 (8): 871-7; discussion 877-9, 1993. [<a href="https://pubmed.ncbi.nlm.nih.gov/8393652" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 8393652</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_261_2">Washburn WK, Lewis WD, Jenkins RL: Aggressive surgical resection for cholangiocarcinoma. Arch Surg 130 (3): 270-6, 1995. [<a href="https://pubmed.ncbi.nlm.nih.gov/7534059" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 7534059</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_261_3">Edeline J, Touchefeu Y, Guiu B, et al.: Radioembolization Plus Chemotherapy for First-line Treatment of Locally Advanced Intrahepatic Cholangiocarcinoma: A Phase 2 Clinical Trial. JAMA Oncol : , 2019. [<a href="/pmc/articles/PMC6824230/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC6824230</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/31670746" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 31670746</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_261_4">Cercek A, Boerner T, Tan BR, et al.: Assessment of Hepatic Arterial Infusion of Floxuridine in Combination With Systemic Gemcitabine and Oxaliplatin in Patients With Unresectable Intrahepatic Cholangiocarcinoma: A Phase 2 Clinical Trial. JAMA Oncol : , 2019. [<a href="/pmc/articles/PMC6824231/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC6824231</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/31670750" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 31670750</span></a>]</div></li></ol></div></div><div id="CDR0000062905__500"><h2 id="_CDR0000062905__500_">Resectable (Localized) Bile Duct Cancer Treatment</h2><p id="CDR0000062905__439">Standard treatment options for resectable (localized) bile duct cancer include the following:</p><ol id="CDR0000062905__465"><li class="half_rhythm"><div><a href="#CDR0000062905__440"><div class="milestone-start" id="CDR0000062905__438"></div>Surgery</a>.</div></li><li class="half_rhythm"><div><a href="#CDR0000062905__476">Adjuvant therapy</a>.</div></li></ol><div id="CDR0000062905__440"><h3>Surgery</h3><div id="CDR0000062905__441"><h4>Intrahepatic bile duct cancer</h4><p id="CDR0000062905__442">For intrahepatic bile duct cancers, hepatic resection to achieve negative margins is potentially curative. If a major liver resection is necessary to achieve negative surgical margins, preoperative portal vein embolization may be considered to optimize the volume of the remnant liver.</p><p id="CDR0000062905__443">Partial liver resection or partial hepatectomy to achieve negative margins is a procedure with curative intent for patients with intrahepatic cholangiocarcinoma.[<a class="bk_pop" href="#CDR0000062905_rl_500_1">1</a>] The extent of liver resection necessary is dependent on the extent of hepatic parenchymal involvement and the proximity of the tumor to major blood vessels in this region.</p><p id="CDR0000062905__466">The role of routine portal lymphadenectomy has not been well established because of the risk of common bile duct devascularization. </p></div><div id="CDR0000062905__467"><h4>Perihilar bile duct cancer</h4><p id="CDR0000062905__468">For perihilar cholangiocarcinomas (Klatskin tumors), bile duct resection alone leads to high local recurrence rates resulting from the early confluence of the hepatic ducts and the caudate lobe. The addition of partial hepatectomy that includes the caudate lobe has improved long-term outcomes, but may be associated with increased postoperative complications.[<a class="bk_pop" href="#CDR0000062905_rl_500_2">2</a>] With this aggressive surgical approach, 5-year survival rates of 20% to 50% have been reported.[<a class="bk_pop" href="#CDR0000062905_rl_500_3">3</a>] An understanding of both the normal and varied vascular and ductal anatomy of the porta hepatis has increased the number of hepatic duct bifurcation tumors that can be resected.</p><p id="CDR0000062905__469">The primary site of relapse after surgical resection is local; however, distant recurrence is also frequently reported.[<a class="bk_pop" href="#CDR0000062905_rl_500_4">4</a>]</p><p id="CDR0000062905__470">The optimal surgical procedure for carcinoma of the perihilar bile duct varies according to the location of the tumor along the biliary tree, the extent of hepatic parenchymal involvement, and the proximity of the tumor to major blood vessels in this region. The state of the regional lymph nodes is assessed at the time of surgery because of their prognostic significance. Operations for bile duct cancer are usually extensive; a historical cohort reported an operative mortality rate of approximately 10%, along with roughly 40% risk of disease recurrence.[<a class="bk_pop" href="#CDR0000062905_rl_500_5">5</a>]</p><p id="CDR0000062905__471">In jaundiced patients, the role of percutaneous transhepatic catheter drainage or endoscopic placement of a stent for relief of biliary obstruction is controversial because of inconsistent findings of significant clinical benefit and concerns of increased risk of postoperative complications.[<a class="bk_pop" href="#CDR0000062905_rl_500_6">6</a>] However, percutaneous transhepatic catheter drainage or endoscopic placement of a stent for relief of biliary obstruction may be considered before surgery, particularly if jaundice is severe or an element of azotemia is present.[<a class="bk_pop" href="#CDR0000062905_rl_500_7">7</a>,<a class="bk_pop" href="#CDR0000062905_rl_500_8">8</a>]</p></div><div id="CDR0000062905__472"><h4>Distal bile duct cancer</h4><p id="CDR0000062905__473">Complete surgical resection with negative surgical margins offers the only chance of cure for distal bile duct cancers. Bile duct tumors can be difficult to resect because of their proximity to major blood vessels and diffuse infiltration of adjacent bile ducts. Total resection is possible in 25% to 30% of lesions that originate in the distal bile duct; the resectability rate is lower for lesions that occur in more proximal sites.[<a class="bk_pop" href="#CDR0000062905_rl_500_9">9</a>]</p><p id="CDR0000062905__474">The optimum surgical procedure for carcinoma of the distal bile duct will vary according to the location of the tumor along the biliary tree, the
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extent of hepatic parenchymal involvement, and the proximity of the tumor to
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major blood vessels in this region. The regional lymph nodes are assessed at the time of surgery because they have prognostic significance. Patients with cancer of the
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lower end of the duct and regional lymph node involvement may warrant an
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extensive resection (Whipple procedure). The 5-year outcomes range between 20% and 50%.[<a class="bk_pop" href="#CDR0000062905_rl_500_10">10</a>,<a class="bk_pop" href="#CDR0000062905_rl_500_11">11</a>] Bypass operations or endoluminal
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stents are alternatives if intraoperatively the tumor is found to be unresectable.[<a class="bk_pop" href="#CDR0000062905_rl_500_10">10</a>,<a class="bk_pop" href="#CDR0000062905_rl_500_11">11</a>]</p><p id="CDR0000062905__475">In jaundiced patients, the role of percutaneous transhepatic catheter drainage or endoscopic placement of a stent for relief of biliary obstruction is controversial, but may be considered before surgery, particularly if jaundice is severe or an element of azotemia is present.[<a class="bk_pop" href="#CDR0000062905_rl_500_7">7</a>,<a class="bk_pop" href="#CDR0000062905_rl_500_8">8</a>]</p></div></div><div id="CDR0000062905__476"><h3>Adjuvant therapy</h3><div id="CDR0000062905__477"><h4>Chemotherapy</h4><p id="CDR0000062905__478">Numerous retrospective series have suggested that adjuvant chemotherapy after complete surgical resection may be beneficial.[<a class="bk_pop" href="#CDR0000062905_rl_500_12">12</a>,<a class="bk_pop" href="#CDR0000062905_rl_500_13">13</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335158/" class="def">Level of evidence: 3iiiDiii</a>] However, prospective randomized trials have failed to show a significant benefit in overall survival (OS). </p><p id="CDR0000062905__492">Evidence (chemotherapy):</p><ol id="CDR0000062905__479"><li class="half_rhythm"><div>A multicenter phase III study in the United Kingdom (BILCAP) randomly assigned 447 patients with cholangiocarcinoma or muscle-invasive gallbladder cancer who underwent a macroscopically complete resection with curative intent to eight cycles of capecitabine (1,250 mg/m<sup>2</sup> twice a day on days 1−14 of a 21-day cycle) or observation.[<a class="bk_pop" href="#CDR0000062905_rl_500_14">14</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000632558/" class="def">Level of evidence: 1iiD</a>] At a median follow-up of 60 months, the following were observed:<ul id="CDR0000062905__480"><li class="half_rhythm"><div> There was no statistically significant difference in OS in the intention-to-treat analysis (median OS, 51.1 months in the capecitabine group vs. 36.4 months in the observation group; adjusted hazard ratio [HR], 0.81; 95% confidence interval [CI], 0.63−1.04; <i>P</i> = .094).</div></li><li class="half_rhythm"><div> A prespecified per-protocol analysis did meet statistical significance for OS (median OS, 53 months in the capecitabine group vs. 36 months in the observation group; adjusted HR, 0.75; 95% CI, 0.58–0.97; <i>P</i> = .028).</div></li><li class="half_rhythm"><div> In the intention-to-treat analysis, median recurrence-free survival (RFS) was 24.4 months (95% CI, 18.6–35.9) in the capecitabine group and 17.5 months (95% CI, 12.0–23.8) in the observation group. An adjusted Cox proportional hazards model suggested potential improvement in RFS in the first 24 months from randomization (HR, 0.75; 95% CI, 0.58–0.98; <i>P</i> = .033), but with no significant difference in the period from 24 to 60 months (HR, 1.48; 95% CI, 0.80–2.77; <i>P</i> = .21)</div></li></ul></div></li><li class="half_rhythm"><div>A French multicenter phase III study (PRODIGE 12-ACCORD 18-UNICANCER GI) randomly assigned 196 patients with R0 or R1 resection of localized biliary tract cancer to 12 cycles of adjuvant gemcitabine plus oxaliplatin (GEMOX) or surveillance.[<a class="bk_pop" href="#CDR0000062905_rl_500_15">15</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000632558/" class="def">Level of evidence: 1iiD</a>] After a median follow-up of 46.5 months:<ul id="CDR0000062905__481"><li class="half_rhythm"><div> There was no statistically significant difference in the primary outcome of RFS (median, 30.4 months with GEMOX vs. 18.5 months with observation; HR, 0.88; 95% CI, 0.62–1.25, <i>P</i> = .48).</div></li><li class="half_rhythm"><div> There was also no statistically significant difference in the secondary outcome of OS (75.8 months with GEMOX vs. 50.8 months with observation; HR, 1.08; 95% CI, 0.7–1.66; <i>P</i> = .74).</div></li></ul></div></li><li class="half_rhythm"><div>The Bile Duct Cancer Adjuvant Trial (BCAT), a Japanese multicenter phase III study, randomized 225 patients with resected bile duct cancer to six cycles of adjuvant gemcitabine or observation.[<a class="bk_pop" href="#CDR0000062905_rl_500_16">16</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000632558/" class="def">Level of evidence: 1iiD</a>]<ul id="CDR0000062905__482"><li class="half_rhythm"><div> There was no significant difference in the primary outcome of OS (median, 62.3 months with gemcitabine vs. 63.8 months with observation; HR, 1.01; 95% CI, 0.7–1.45; <i>P</i> = .964).</div></li><li class="half_rhythm"><div> No OS differences were observed, even in subgroups stratified by lymph node status and surgical margin status.</div></li><li class="half_rhythm"><div> There was also no significant difference in the secondary outcome of RFS (median, 36 months with gemcitabine vs. 39.9 months with observation; HR, 0.93; <i>P</i> = .693).</div></li></ul></div></li><li class="half_rhythm"><div>The European Study Group for Pancreatic Cancer (<a href="https://www.cancer.gov/clinicaltrials/NCT00058201" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">ESPAC-3</a> [<a href="https://clinicaltrials.gov/show/NCT00058201" title="Study NCT00058201" ref="pagearea=body&targetsite=external&targetcat=link&targettype=clinical-trial">NCT00058201</a>]) trial randomly assigned 428 patients with periampullary cancer, which included 96 patients with bile duct cancers, to observation, 6 months of fluorouracil (5-FU)/leucovorin, or 6 months of gemcitabine.[<a class="bk_pop" href="#CDR0000062905_rl_500_17">17</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000632558/" class="def">Level of evidence: 1iiD</a>] <ul id="CDR0000062905__483"><li class="half_rhythm"><div>Among all patients, adjuvant chemotherapy, compared with observation, was not associated with significant OS benefit. However, after adjusting for prognostic variables by multivariable analysis, a statistically significant OS benefit was associated with adjuvant chemotherapy (HR, 0.75; 95% CI, 0.57–0.98; <i>P</i> = .03).</div></li><li class="half_rhythm"><div>In a preplanned subgroup analysis of the 96 patients with bile duct cancer, no benefit was seen among patients treated with chemotherapy. Limitations of this subgroup analysis include limited statistical power and difficulty in differentiating ampullary versus distal common bile duct tumors as pathologic site of origin. </div></li><li class="half_rhythm"><div>The median survivals were 27 months for the observation-alone group, 18 months for the 5-FU/leucovorin group, and 20 months for the gemcitabine-alone group.[<a class="bk_pop" href="#CDR0000062905_rl_500_17">17</a>]</div></li></ul></div></li><li class="half_rhythm"><div>A multi-institutional Japanese study compared surgery alone with mitomycin-C and infusional 5-FU followed by 5-FU until disease progression.[<a class="bk_pop" href="#CDR0000062905_rl_500_18">18</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000632558/" class="def">Level of evidence: 1iiD</a>]<ul id="CDR0000062905__484"><li class="half_rhythm"><div>Among the subset of patients with bile duct cancer (n = 139), no survival benefit was seen.</div></li></ul></div></li></ol><p id="CDR0000062905__485">On the basis of these trials, there is no consistent trend in favor of adjuvant therapy in either RFS or OS.</p><p id="CDR0000062905__486">(Refer to the <a href="#CDR0000062905__501">Unresectable [Including Metastatic and Recurrent] Bile Duct Cancer Treatment</a> section of this summary for a list of regimens with potential activity.)</p></div><div id="CDR0000062905__487"><h4>External beam radiation therapy (EBRT)</h4><p id="CDR0000062905__488">Numerous retrospective studies have suggested that adding EBRT after complete surgical resection may be beneficial.[<a class="bk_pop" href="#CDR0000062905_rl_500_19">19</a>,<a class="bk_pop" href="#CDR0000062905_rl_500_20">20</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335125/" class="def">Level of evidence: 1iiA</a>] However, no prospective randomized trials have demonstrated an OS benefit.</p><p id="CDR0000062905__493">Evidence (EBRT):</p><ol id="CDR0000062905__489"><li class="half_rhythm"><div>One small randomized trial of 207 patients with pancreatic and periampullary cancers demonstrated no survival benefit of adding chemoradiation therapy after surgery. This study is limited, however, because only a few patients had a diagnosis of bile duct cancer, and 20% of the patients randomly assigned to receive chemoradiation therapy did not receive treatment.[<a class="bk_pop" href="#CDR0000062905_rl_500_21">21</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000587991/" class="def">Level of evidence: 3iiiDiv</a>]</div></li><li class="half_rhythm"><div>A phase II cooperative group trial, SWOG S0809 (<a href="https://www.cancer.gov/clinicaltrials/NCT00789958" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">NCT00789958</a>), evaluated adjuvant capecitabine and gemcitabine followed by chemoradiation therapy for resected extrahepatic cholangiocarcinoma and gallbladder cancer. In total, 79 eligible patients with pT2 to pT4 disease, node-positive disease, or positive-margin resection were enrolled (extrahepatic bile duct cancer, n = 54; gallbladder cancer, n = 25).[<a class="bk_pop" href="#CDR0000062905_rl_500_22">22</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000593395/" class="def">Level of evidence: 3iiiD</a>] <ul id="CDR0000062905__490"><li class="half_rhythm"><div>The 2-year survival rate of 65% was significantly higher than expected, based on historical controls.[<a class="bk_pop" href="#CDR0000062905_rl_500_22">22</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000593395/" class="def">Level of evidence: 3iiiD</a>]</div></li><li class="half_rhythm"><div>Grade 3 toxicity was observed in 52% of patients, and grade 4 toxicity was observed in 11% of patients.</div></li><li class="half_rhythm"><div>On the basis of these results, this regimen was observed to be well tolerated, but needs to be tested in a randomized controlled trial.</div></li></ul></div></li></ol><p id="CDR0000062905__491">All patients are encouraged to enroll in clinical trials for adjuvant therapies. Information about ongoing clinical trials is available from the <a href="https://www.cancer.gov/about-cancer/treatment/clinical-trials" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">NCI website</a>.<div class="milestone-end"></div></p></div></div><div id="CDR0000062905__TrialSearch_500_sid_5"><h3>Current Clinical Trials</h3><p id="CDR0000062905__TrialSearch_500_22">Use our <a href="https://www.cancer.gov/about-cancer/treatment/clinical-trials/advanced-search" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">advanced clinical trial search</a> to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. <a href="https://www.cancer.gov/about-cancer/treatment/clinical-trials/" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">General information</a> about clinical trials is also available.</p></div><div id="CDR0000062905_rl_500"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000062905_rl_500_1">Dodson RM, Weiss MJ, Cosgrove D, et al.: Intrahepatic cholangiocarcinoma: management options and emerging therapies. J Am Coll Surg 217 (4): 736-750.e4, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/23890842" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 23890842</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_500_2">Burke EC, Jarnagin WR, Hochwald SN, et al.: Hilar Cholangiocarcinoma: patterns of spread, the importance of hepatic resection for curative operation, and a presurgical clinical staging system. Ann Surg 228 (3): 385-94, 1998. [<a href="/pmc/articles/PMC1191497/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC1191497</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/9742921" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 9742921</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_500_3">Nakeeb A, Tran KQ, Black MJ, et al.: Improved survival in resected biliary malignancies. Surgery 132 (4): 555-63; discussion 563-4, 2002. [<a href="https://pubmed.ncbi.nlm.nih.gov/12407338" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 12407338</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_500_4">Hasegawa S, Ikai I, Fujii H, et al.: Surgical resection of hilar cholangiocarcinoma: analysis of survival and postoperative complications. World J Surg 31 (6): 1256-63, 2007. [<a href="https://pubmed.ncbi.nlm.nih.gov/17453285" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 17453285</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_500_5">Loehrer AP, House MG, Nakeeb A, et al.: Cholangiocarcinoma: are North American surgical outcomes optimal? J Am Coll Surg 216 (2): 192-200, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/23266423" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 23266423</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_500_6">Liu F, Li Y, Wei Y, et al.: Preoperative biliary drainage before resection for hilar cholangiocarcinoma: whether or not? A systematic review. Dig Dis Sci 56 (3): 663-72, 2011. [<a href="https://pubmed.ncbi.nlm.nih.gov/20635143" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20635143</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_500_7">Nimura Y: Preoperative biliary drainage before resection for cholangiocarcinoma (Pro). HPB (Oxford) 10 (2): 130-3, 2008. [<a href="/pmc/articles/PMC2504393/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC2504393</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/18773090" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18773090</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_500_8">Laurent A, Tayar C, Cherqui D: Cholangiocarcinoma: preoperative biliary drainage (Con). HPB (Oxford) 10 (2): 126-9, 2008. [<a href="/pmc/articles/PMC2504392/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC2504392</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/18773089" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18773089</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_500_9">Stain SC, Baer HU, Dennison AR, et al.: Current management of hilar cholangiocarcinoma. Surg Gynecol Obstet 175 (6): 579-88, 1992. [<a href="https://pubmed.ncbi.nlm.nih.gov/1280374" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 1280374</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_500_10">Fong Y, Blumgart LH, Lin E, et al.: Outcome of treatment for distal bile duct cancer. Br J Surg 83 (12): 1712-5, 1996. [<a href="https://pubmed.ncbi.nlm.nih.gov/9038548" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 9038548</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_500_11">Bortolasi L, Burgart LJ, Tsiotos GG, et al.: Adenocarcinoma of the distal bile duct. A clinicopathologic outcome analysis after curative resection. Dig Surg 17 (1): 36-41, 2000. [<a href="https://pubmed.ncbi.nlm.nih.gov/10720830" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 10720830</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_500_12">Todoroki T: Chemotherapy for bile duct carcinoma in the light of adjuvant chemotherapy to surgery. Hepatogastroenterology 47 (33): 644-9, 2000 May-Jun. [<a href="https://pubmed.ncbi.nlm.nih.gov/10919004" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 10919004</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_500_13">Murakami Y, Uemura K, Sudo T, et al.: Adjuvant gemcitabine plus S-1 chemotherapy improves survival after aggressive surgical resection for advanced biliary carcinoma. Ann Surg 250 (6): 950-6, 2009. [<a href="https://pubmed.ncbi.nlm.nih.gov/19953713" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 19953713</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_500_14">Primrose JN, Fox RP, Palmer DH, et al.: Capecitabine compared with observation in resected biliary tract cancer (BILCAP): a randomised, controlled, multicentre, phase 3 study. Lancet Oncol 20 (5): 663-673, 2019. [<a href="https://pubmed.ncbi.nlm.nih.gov/30922733" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 30922733</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_500_15">Edeline J, Benabdelghani M, Bertaut A, et al.: Gemcitabine and Oxaliplatin Chemotherapy or Surveillance in Resected Biliary Tract Cancer (PRODIGE 12-ACCORD 18-UNICANCER GI): A Randomized Phase III Study. J Clin Oncol 37 (8): 658-667, 2019. [<a href="https://pubmed.ncbi.nlm.nih.gov/30707660" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 30707660</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_500_16">Ebata T, Hirano S, Konishi M, et al.: Randomized clinical trial of adjuvant gemcitabine chemotherapy versus observation in resected bile duct cancer. Br J Surg 105 (3): 192-202, 2018. [<a href="https://pubmed.ncbi.nlm.nih.gov/29405274" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 29405274</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_500_17">Neoptolemos JP, Moore MJ, Cox TF, et al.: Effect of adjuvant chemotherapy with fluorouracil plus folinic acid or gemcitabine vs observation on survival in patients with resected periampullary adenocarcinoma: the ESPAC-3 periampullary cancer randomized trial. JAMA 308 (2): 147-56, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/22782416" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22782416</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_500_18">Takada T, Amano H, Yasuda H, et al.: Is postoperative adjuvant chemotherapy useful for gallbladder carcinoma? A phase III multicenter prospective randomized controlled trial in patients with resected pancreaticobiliary carcinoma. Cancer 95 (8): 1685-95, 2002. [<a href="https://pubmed.ncbi.nlm.nih.gov/12365016" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 12365016</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_500_19">Kim TH, Han SS, Park SJ, et al.: Role of adjuvant chemoradiotherapy for resected extrahepatic biliary tract cancer. Int J Radiat Oncol Biol Phys 81 (5): e853-9, 2011. [<a href="https://pubmed.ncbi.nlm.nih.gov/21497455" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21497455</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_500_20">Hughes MA, Frassica DA, Yeo CJ, et al.: Adjuvant concurrent chemoradiation for adenocarcinoma of the distal common bile duct. Int J Radiat Oncol Biol Phys 68 (1): 178-82, 2007. [<a href="https://pubmed.ncbi.nlm.nih.gov/17276614" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 17276614</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_500_21">Klinkenbijl JH, Jeekel J, Sahmoud T, et al.: Adjuvant radiotherapy and 5-fluorouracil after curative resection of cancer of the pancreas and periampullary region: phase III trial of the EORTC gastrointestinal tract cancer cooperative group. Ann Surg 230 (6): 776-82; discussion 782-4, 1999. [<a href="/pmc/articles/PMC1420941/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC1420941</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/10615932" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 10615932</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_500_22">Ben-Josef E, Guthrie KA, El-Khoueiry AB, et al.: SWOG S0809: A Phase II Intergroup Trial of Adjuvant Capecitabine and Gemcitabine Followed by Radiotherapy and Concurrent Capecitabine in Extrahepatic Cholangiocarcinoma and Gallbladder Carcinoma. J Clin Oncol 33 (24): 2617-22, 2015. [<a href="/pmc/articles/PMC4534524/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC4534524</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/25964250" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 25964250</span></a>]</div></li></ol></div></div><div id="CDR0000062905__501"><h2 id="_CDR0000062905__501_">Unresectable (Including Metastatic and Recurrent) Bile Duct Cancer Treatment</h2><p id="CDR0000062905__445">Standard treatment options for unresectable (including metastatic and recurrent) bile duct cancer include the following:</p><ol id="CDR0000062905__446"><li class="half_rhythm"><div><a href="#CDR0000062905__447">Palliative therapy</a>.</div></li><li class="half_rhythm"><div><a href="#CDR0000062905__450">Chemotherapy</a>.</div></li><li class="half_rhythm"><div><a href="#CDR0000062905__458">Immunotherapy</a>.</div></li><li class="half_rhythm"><div><a href="#CDR0000062905__460">Targeted therapy</a>.</div></li></ol><div id="CDR0000062905__447"><h3>Palliative therapy</h3><p id="CDR0000062905__448">Relief of biliary obstruction is warranted when symptoms such as pruritus and hepatic dysfunction outweigh other symptoms of the cancer. When possible, such palliation can be achieved with the placement of bile duct stents by operative, endoscopic, or percutaneous techniques.[<a class="bk_pop" href="#CDR0000062905_rl_501_1">1</a>,<a class="bk_pop" href="#CDR0000062905_rl_501_2">2</a>]</p><p id="CDR0000062905__449">Palliative radiation therapy may be beneficial, and patients may be candidates for stereotactic body radiation therapy [<a class="bk_pop" href="#CDR0000062905_rl_501_3">3</a>] and intra-arterial embolization.[<a class="bk_pop" href="#CDR0000062905_rl_501_4">4</a>]</p></div><div id="CDR0000062905__450"><h3>Chemotherapy</h3><p id="CDR0000062905__451">Systemic chemotherapy is appropriate for selected patients with adequate performance status and intact organ function. The following agents have been reported to produce transient partial remissions in a minority of patients:</p><ul id="CDR0000062905__452"><li class="half_rhythm"><div>Fluoropyrimidines (gemcitabine).</div></li><li class="half_rhythm"><div>Platinum agents.</div></li><li class="half_rhythm"><div>Docetaxel.</div></li></ul><p id="CDR0000062905__494">Evidence (chemotherapy):</p><ol id="CDR0000062905__495"><li class="half_rhythm"><div>A phase III study (<a href="https://www.cancer.gov/clinicaltrials/NCT00262769" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">NCT00262769</a>) randomly assigned 410 patients with unresectable, recurrent, or metastatic biliary tract carcinoma to receive cisplatin followed by gemcitabine or to receive gemcitabine alone for up to 6 months and observed the following:[<a class="bk_pop" href="#CDR0000062905_rl_501_5">5</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335125/" class="def">Level of evidence: 1iiA</a>]<ul id="CDR0000062905__496"><li class="half_rhythm"><div>Median overall survival (OS) improved among patients treated with cisplatin and gemcitabine therapy (11.7 months) versus patients treated with gemcitabine alone (8.1 months) (hazard ratio [HR], 0.64; 95% confidence interval [CI], 0.52−0.80; <i>P</i> < .001).[<a class="bk_pop" href="#CDR0000062905_rl_501_5">5</a>]</div></li><li class="half_rhythm"><div>A similar median OS benefit was demonstrated in all subgroups, including 73 patients with extrahepatic bile duct cancer and 57 patients with hilar tumors.</div></li><li class="half_rhythm"><div>Grades 3 and 4 toxicities occurred with similar frequency in both study arms, with the exception of increased hematologic toxicity in patients randomly assigned to the gemcitabine/cisplatin arm and increased hepatic toxicity in patients randomly assigned to the single-agent gemcitabine arm.</div></li></ul></div></li><li class="half_rhythm"><div>A phase III noninferiority study (<a href="https://www.cancer.gov/clinicaltrials/NCT01470443" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">NCT01470443</a>) enrolled 114 patients with metastatic biliary tract cancers, including 30 (26%) with primary gallbladder cancer. Patients were randomly assigned to receive capecitabine plus oxaliplatin (XELOX) or gemcitabine plus oxaliplatin (GEMOX).[<a class="bk_pop" href="#CDR0000062905_rl_501_6">6</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000632558/" class="def">Level of evidence: 1iiD</a>]<ul id="CDR0000062905__497"><li class="half_rhythm"><div> OS was not significantly different, with 10.4 months (95% CI, 8.0−12.6 months) in the GEMOX group compared with 10.6 months (95% CI, 7.3−15.5 months) in the XELOX group (<i>P</i> = .131).</div></li><li class="half_rhythm"><div> The primary endpoint of 6-month progression-free-survival (PFS) was 44.6% for the GEMOX group and 46.7% for the XELOX group (95% CI of difference in 6-month PFS rate, -12% to 16%, meeting criteria for noninferiority). </div></li><li class="half_rhythm"><div>A predefined subgroup analysis based on primary site of disease did not reveal a difference in objective response rate between the two arms in patients with gallbladder cancer (<i>P</i> = .598). </div></li></ul></div></li></ol><p id="CDR0000062905__457">Pending further clinical trials, cisplatin plus gemcitabine is considered the reference standard for patients with unresectable, metastatic, or recurrent bile duct cancer. Potential alternatives include gemcitabine plus capecitabine, GEMOX, and XELOX. Clinical trials should be considered for all patients.</p></div><div id="CDR0000062905__458"><h3>Immunotherapy</h3><p id="CDR0000062905__459">All patients with unresectable, metastatic, or recurrent disease should have molecular testing for deficient mismatch repair (dMMR) or microsatellite instability-high (MSI-H). Extrapolating from a subgroup of patients with gastrointestinal and hepatopancreatobiliary tumors in the <a href="https://www.cancer.gov/clinicaltrials/NCT02534675" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">I-PREDICT</a> (<a href="https://clinicaltrials.gov/show/NCT02534675" title="Study NCT02534675" ref="pagearea=body&targetsite=external&targetcat=link&targettype=clinical-trial">NCT02534675</a>) and <a href="https://www.cancer.gov/clinicaltrials/NCT02628067" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">KEYNOTE-158</a> (<a href="https://clinicaltrials.gov/show/NCT02628067" title="Study NCT02628067" ref="pagearea=body&targetsite=external&targetcat=link&targettype=clinical-trial">NCT02628067</a>) studies, patients with either dMMR or MSI-H tumors can be considered for treatment with pembrolizumab.[<a class="bk_pop" href="#CDR0000062905_rl_501_7">7</a>,<a class="bk_pop" href="#CDR0000062905_rl_501_8">8</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000587991/" class="def">Level of evidence: 3iiiDiv</a>]</p></div><div id="CDR0000062905__460"><h3>Targeted therapy</h3><p id="CDR0000062905__461">Clinical trials of investigational therapies should be considered for patients with targetable mutations.</p><p id="CDR0000062905__498">Evidence (targeted therapy):</p><ol id="CDR0000062905__462"><li class="half_rhythm"><div>IDH1 inhibitor: Up to 15% of bile duct cancers express a mutation in isocitrate dehydrogenase 1 (<i>IDH1</i>). The phase III <a href="https://www.cancer.gov/clinicaltrials/NCT02989857" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">ClarIDHy</a> (<a href="https://clinicaltrials.gov/show/NCT02989857" title="Study NCT02989857" ref="pagearea=body&targetsite=external&targetcat=link&targettype=clinical-trial">NCT02989857</a>) trial, reported in abstract form, randomly assigned 185 patients with <i>IDH1</i>-mutated cholangiocarcinoma to the IDH1 inhibitor ivosidenib or placebo and observed the following:[<a class="bk_pop" href="#CDR0000062905_rl_501_9">9</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335124/" class="def">Level of evidence: 1iDiii</a>] <ul id="CDR0000062905__463"><li class="half_rhythm"><div> Median PFS was improved among patients treated with ivosidenib (2.7 months) compared with placebo (1.4 months) (HR, 0.37; 95% CI, 0.25−0.54; <i>P</i> < .001). PFS rates at 6 months and 12 months were 32% and 21.9%, respectively, in the ivosidenib arm. No patients in the placebo group were progression-free at 6 months.</div></li><li class="half_rhythm"><div> In the intention-to-treat analysis, median OS was 10.8 months in the ivosidenib group compared with 9.7 months for placebo (HR, 0.60; one-sided <i>P</i> = .06), despite crossover of 57% of placebo patients to ivosidenib.</div></li><li class="half_rhythm"><div> Grades 3 and 4 toxicities occurred in 46% of patients in the ivosidenib group compared with 36% in the placebo group. </div></li><li class="half_rhythm"><div>Although ivosidenib has previously been approved in treatment of leukemia, there is currently no U.S. Food and Drug Administration (FDA) approval for bile duct cancers. Patients with <i>IDH1</i>-mutated disease should be encouraged to enroll in a clinical trial.</div></li></ul></div></li><li class="half_rhythm"><div>Fibroblast growth factor receptor 2 (FGFR2) inhibitors: FGFR2 fusions are present in approximately 15% of intrahepatic cholangiocarcinoma. Multiple phase II trials, some reported in abstract form, in patients who progressed after or were ineligible for first-line chemotherapy have suggested activity of FGFR inhibitors in cholangiocarcinoma with FGFR2 fusion.[<a class="bk_pop" href="#CDR0000062905_rl_501_10">10</a>-<a class="bk_pop" href="#CDR0000062905_rl_501_13">13</a>] At this time, there are no FDA-approved agents, pending more robust evidence. Patients with FGFR2 fusion-positive disease should be encouraged to enroll in a clinical trial.</div></li></ol><p id="CDR0000062905__464">All patients are encouraged to enroll in clinical trials for adjuvant therapies. Information about ongoing clinical trials is available from the <a href="https://www.cancer.gov/about-cancer/treatment/clinical-trials" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">NCI website</a>.</p></div><div id="CDR0000062905__TrialSearch_501_sid_6"><h3>Current Clinical Trials</h3><p id="CDR0000062905__TrialSearch_501_22">Use our <a href="https://www.cancer.gov/about-cancer/treatment/clinical-trials/advanced-search" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">advanced clinical trial search</a> to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. <a href="https://www.cancer.gov/about-cancer/treatment/clinical-trials/" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">General information</a> about clinical trials is also available.</p></div><div id="CDR0000062905_rl_501"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000062905_rl_501_1">Nordback IH, Pitt HA, Coleman J, et al.: Unresectable hilar cholangiocarcinoma: percutaneous versus operative palliation. Surgery 115 (5): 597-603, 1994. [<a href="https://pubmed.ncbi.nlm.nih.gov/7513906" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 7513906</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_501_2">Levy MJ, Baron TH, Gostout CJ, et al.: Palliation of malignant extrahepatic biliary obstruction with plastic versus expandable metal stents: An evidence-based approach. Clin Gastroenterol Hepatol 2 (4): 273-85, 2004. [<a href="https://pubmed.ncbi.nlm.nih.gov/15067620" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15067620</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_501_3">Barney BM, Olivier KR, Miller RC, et al.: Clinical outcomes and toxicity using stereotactic body radiotherapy (SBRT) for advanced cholangiocarcinoma. Radiat Oncol 7: 67, 2012. [<a href="/pmc/articles/PMC3464963/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3464963</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/22553982" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22553982</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_501_4">Hyder O, Marsh JW, Salem R, et al.: Intra-arterial therapy for advanced intrahepatic cholangiocarcinoma: a multi-institutional analysis. Ann Surg Oncol 20 (12): 3779-86, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/23846786" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 23846786</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_501_5">Valle J, Wasan H, Palmer DH, et al.: Cisplatin plus gemcitabine versus gemcitabine for biliary tract cancer. N Engl J Med 362 (14): 1273-81, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/20375404" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20375404</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_501_6">Kim ST, Kang JH, Lee J, et al.: Capecitabine plus oxaliplatin versus gemcitabine plus oxaliplatin as first-line therapy for advanced biliary tract cancers: a multicenter, open-label, randomized, phase III, noninferiority trial. Ann Oncol 30 (5): 788-795, 2019. [<a href="https://pubmed.ncbi.nlm.nih.gov/30785198" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 30785198</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_501_7">Sicklick JK, Kato S, Okamura R, et al.: Molecular profiling of cancer patients enables personalized combination therapy: the I-PREDICT study. Nat Med 25 (5): 744-750, 2019. [<a href="/pmc/articles/PMC6553618/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC6553618</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/31011206" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 31011206</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_501_8">Marabelle A, Le DT, Ascierto PA, et al.: Efficacy of Pembrolizumab in Patients With Noncolorectal High Microsatellite Instability/Mismatch Repair-Deficient Cancer: Results From the Phase II KEYNOTE-158 Study. J Clin Oncol 38 (1): 1-10, 2020. [<a href="/pmc/articles/PMC8184060/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC8184060</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/31682550" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 31682550</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_501_9">Abou-Alfa GK, Macarulla Mercade T, Javle M, et al.: ClarlDHy: a global, phase III, randomized, double-blind study of ivosidenib (IVO) vs placebo in patients with advanced cholangiocarcinoma (CC) with an isocitrate dehydrogenase 1 (IDH1) mutation. [Abstract] Ann Oncol 30 (Suppl 5): A-LBA10_PR, 2019.</div></li><li><div class="bk_ref" id="CDR0000062905_rl_501_10">Javle M, Lowery M, Shroff RT, et al.: Phase II Study of BGJ398 in Patients With FGFR-Altered Advanced Cholangiocarcinoma. J Clin Oncol 36 (3): 276-282, 2018. [<a href="/pmc/articles/PMC6075847/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC6075847</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/29182496" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 29182496</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_501_11">Mazzaferro V, El-Rayes BF, Droz Dit Busset M, et al.: Derazantinib (ARQ 087) in advanced or inoperable FGFR2 gene fusion-positive intrahepatic cholangiocarcinoma. Br J Cancer 120 (2): 165-171, 2019. [<a href="/pmc/articles/PMC6342954/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC6342954</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/30420614" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 30420614</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_501_12">Vogel A, Sahai V, Hollebecque A, et al.: FIGHT-202: a phase II study of pemigatinib in patients (pts) with previously locally advanced or metastatic cholangiocarcinoma (CCA). [Abstract] Ann Oncol 30 (Suppl 5): A-LBA40, 2019.</div></li><li><div class="bk_ref" id="CDR0000062905_rl_501_13">Droz Dit Busset M, Braun S, El-Rayes B, et al.: Efficacy of derazantinib (DZB) in patients (pts) with intrahepatic cholangiocarcinoma (ICCA) expressing FGFR2-fusion or FGFR2 mutations/amplifications. [Abstract] Ann Oncol 30 (Suppl 5): A-721P, 2019.</div></li></ol></div></div><div id="CDR0000062905__71"><h2 id="_CDR0000062905__71_">Changes to This Summary (03/27/2020)</h2><p id="CDR0000062905__72">The PDQ cancer information summaries are reviewed regularly and updated as
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new information becomes available. This section describes the latest
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changes made to this summary as of the date above.</p><p id="CDR0000062905__508">This summary was comprehensively reviewed and extensively revised.</p><p id="CDR0000062905__disclaimerHP_3">This summary is written and maintained by the <a href="https://www.cancer.gov/publications/pdq/editorial-boards/adult-treatment" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PDQ Adult Treatment Editorial Board</a>, which is
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editorially independent of NCI. The summary reflects an independent review of
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the literature and does not represent a policy statement of NCI or NIH. More
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information about summary policies and the role of the PDQ Editorial Boards in
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maintaining the PDQ summaries can be found on the <a href="#CDR0000062905__AboutThis_1">About This PDQ Summary</a> and <a href="https://www.cancer.gov/publications/pdq" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PDQ® - NCI's Comprehensive Cancer Database</a> pages.
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</p></div><div id="CDR0000062905__AboutThis_1"><h2 id="_CDR0000062905__AboutThis_1_">About This PDQ Summary</h2><div id="CDR0000062905__AboutThis_2"><h3>Purpose of This Summary</h3><p id="CDR0000062905__AboutThis_3">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of bile duct cancer. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.</p></div><div id="CDR0000062905__AboutThis_4"><h3>Reviewers and Updates</h3><p id="CDR0000062905__AboutThis_5">This summary is reviewed regularly and updated as necessary by the <a href="https://www.cancer.gov/publications/pdq/editorial-boards/adult-treatment" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PDQ Adult Treatment Editorial Board</a>, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).</p><p id="CDR0000062905__AboutThis_22"> Board members review recently published articles each month to determine whether an article should:</p><ul id="CDR0000062905__AboutThis_6"><li class="half_rhythm"><div>be discussed at a meeting,</div></li><li class="half_rhythm"><div>be cited with text, or</div></li><li class="half_rhythm"><div>replace or update an existing article that is already cited.</div></li></ul><p id="CDR0000062905__AboutThis_7">Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.</p><p>The lead reviewers for Bile Duct Cancer (Cholangiocarcinoma) Treatment are:</p><ul><li class="half_rhythm"><div>Valerie Lee, MD (Johns Hopkins University)</div></li><li class="half_rhythm"><div>Ari Seifter, MD (University of Illinois at Chicago)</div></li></ul><p id="CDR0000062905__AboutThis_9">Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's <a href="https://www.cancer.gov/contact/email-us" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Email Us</a>. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.</p></div><div id="CDR0000062905__AboutThis_10"><h3>Levels of Evidence</h3><p id="CDR0000062905__AboutThis_11">Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a <a href="/books/n/pdqcis/CDR0000062796/">formal evidence ranking system</a> in developing its level-of-evidence designations.</p></div><div id="CDR0000062905__AboutThis_12"><h3>Permission to Use This Summary</h3><p id="CDR0000062905__AboutThis_13">PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].”</p><p id="CDR0000062905__AboutThis_14">The preferred citation for this PDQ summary is:</p><p id="CDR0000062905__AboutThis_15">PDQ® Adult Treatment Editorial Board. PDQ Bile Duct Cancer (Cholangiocarcinoma) Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: <a href="https://www.cancer.gov/types/liver/hp/bile-duct-treatment-pdq" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">https://www.cancer.gov/types/liver/hp/bile-duct-treatment-pdq</a>. Accessed <MM/DD/YYYY>. [PMID: 26389308]</p><p id="CDR0000062905__AboutThis_16">Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in <a href="https://visualsonline.cancer.gov/" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Visuals Online</a>, a collection of over 2,000 scientific images.
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</p></div><div id="CDR0000062905__AboutThis_17"><h3>Disclaimer</h3><p id="CDR0000062905__AboutThis_18">Based on the strength of the available evidence, treatment options may be described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the <a href="https://www.cancer.gov/about-cancer/managing-care" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Managing Cancer Care</a> page.</p></div><div id="CDR0000062905__AboutThis_20"><h3>Contact Us</h3><p id="CDR0000062905__AboutThis_21">More information about contacting us or receiving help with the Cancer.gov website can be found on our <a href="https://www.cancer.gov/contact" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Contact Us for Help</a> page. Questions can also be submitted to Cancer.gov through the website’s <a href="https://www.cancer.gov/contact/email-us" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Email Us</a>.</p></div></div><div style="display:none"><div style="display:none" id="figCDR0000062905254"><img alt="Image CDR0000659742" src-large="/books/NBK65869.10/bin/CDR0000659742.jpg" /></div></div></div></div>
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<div xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance"></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Views</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="PDF_download" id="Shutter"></a></div><div class="portlet_content"><ul xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="simple-list"><li><a href="/books/NBK65869.10/?report=reader">PubReader</a></li><li><a href="/books/NBK65869.10/?report=printable">Print View</a></li><li><a data-jig="ncbidialog" href="#_ncbi_dlg_citbx_NBK65869" data-jigconfig="width:400,modal:true">Cite this Page</a><div id="_ncbi_dlg_citbx_NBK65869" style="display:none" title="Cite this Page"><div class="bk_tt">PDQ Adult Treatment Editorial Board. Bile Duct Cancer (Cholangiocarcinoma) Treatment (PDQ®): Health Professional Version. 2020 Mar 27. In: PDQ Cancer Information Summaries [Internet]. Bethesda (MD): National Cancer Institute (US); 2002-. <span class="bk_cite_avail"></span></div></div></li><li><a href="#" class="toggle-glossary-link" title="Enable/disable links to the glossary">Disable Glossary Links</a></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Version History</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter shutter_closed" title="Show/hide content" remembercollapsed="true" pgsec_name="version_history" id="Shutter"></a></div><div class="portlet_content" style="display: none;"><ul xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="simple-list"><li><span class="bk_col_itm"><a href="/books/NBK65869.22/">NBK65869.22</a></span> March 28, 2024</li><li><span class="bk_col_itm"><a href="/books/NBK65869.21/">NBK65869.21</a></span> March 14, 2024</li><li><span class="bk_col_itm"><a href="/books/NBK65869.20/">NBK65869.20</a></span> 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class="portlet_title"><h3><span>In this Page</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="page-toc" id="Shutter"></a></div><div class="portlet_content"><ul xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="simple-list"><li><a href="#CDR0000062905__1" ref="log$=inpage&link_id=inpage">General Information About Bile Duct Cancer</a></li><li><a href="#CDR0000062905__202" ref="log$=inpage&link_id=inpage">Cellular Classification of Bile Duct Cancer</a></li><li><a href="#CDR0000062905__25" ref="log$=inpage&link_id=inpage">Stage Information for Bile Duct Cancer</a></li><li><a href="#CDR0000062905__261" ref="log$=inpage&link_id=inpage">Treatment Option Overview for Bile Duct Cancer</a></li><li><a href="#CDR0000062905__500" ref="log$=inpage&link_id=inpage">Resectable (Localized) Bile Duct Cancer Treatment</a></li><li><a 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xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="cit">PDQ Cancer Information Summaries. 2002</em></div></div></li><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/26389231" ref="ordinalpos=1&linkpos=2&log$=relatedreviews&logdbfrom=pubmed"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Pruritus (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div class="brieflinkpop offscreen_noflow"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Pruritus (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="author">PDQ Supportive and Palliative Care Editorial Board. </em><em 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