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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&amp;targetsite=external&amp;targetcat=link&amp;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>
<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 Treatment (PDQ&#x000ae;)</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: September 9, 2015.</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 is extremely rare; however, the true incidence of bile duct cancer is unknown because establishing an accurate diagnosis is difficult. Traditionally, bile duct tumors located within the liver (intrahepatic cholangiocarcinomas) have 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 (extrahepatic cholangiocarcinomas) have 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, and the term now includes intrahepatic, perihilar, and distal extrahepatic tumors of the bile ducts.</p><p id="CDR0000062905__2">Bile duct cancer may occur more frequently in patients with a history of primary sclerosing cholangitis, chronic ulcerative colitis, choledochal cysts, or infections with the liver fluke, <i>Clonorchis sinensis</i>.[<a class="bk_pop" href="#CDR0000062905_rl_1_2">2</a>] The most common symptoms caused by bile duct cancer include the following: </p><ul id="CDR0000062905__163"><li class="half_rhythm"><div>Jaundice.</div></li><li class="half_rhythm"><div>Pain.</div></li><li class="half_rhythm"><div>Fever.</div></li><li class="half_rhythm"><div>Pruritis.</div></li></ul><p id="CDR0000062905__3">In most patients, the tumor cannot be completely removed by surgery and is incurable. Bile duct cancer is frequently multifocal. 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 (Figure 1). Bile is produced by the liver and is important for fat digestion. The biliary system is classified by its anatomic location. </p><div class="iconblock whole_rhythm clearfix ten_col fig" id="figCDR00000629056" co-legend-rid="figlgndCDR00000629056"><a href="/books/NBK65869.2/figure/CDR0000062905__6/?report=objectonly" target="object" title="Figure" class="img_link icnblk_img figpopup" rid-figpopup="figCDR00000629056" rid-ob="figobCDR00000629056"><img class="small-thumb" src="/books/NBK65869.2/bin/CDR0000659742.gif" src-large="/books/NBK65869.2/bin/CDR0000659742.jpg" alt="Figure 1" /></a><div class="icnblk_cntnt" id="figlgndCDR00000629056"><h4 id="CDR0000062905__6"><a href="/books/NBK65869.2/figure/CDR0000062905__6/?report=objectonly" target="object" rid-ob="figobCDR00000629056">Figure</a></h4><p class="float-caption no_bottom_margin">Figure 1. Anatomy of the extrahepatic bile duct. </p></div></div><div class="iconblock whole_rhythm clearfix ten_col fig" id="figCDR0000062905226" co-legend-rid="figlgndCDR0000062905226"><a href="/books/NBK65869.2/figure/CDR0000062905__226/?report=objectonly" target="object" title="Figure" class="img_link icnblk_img figpopup" rid-figpopup="figCDR0000062905226" rid-ob="figobCDR0000062905226"><img class="small-thumb" src="/books/NBK65869.2/bin/CDR0000765897.gif" src-large="/books/NBK65869.2/bin/CDR0000765897.jpg" alt="Figure 2" /></a><div class="icnblk_cntnt" id="figlgndCDR0000062905226"><h4 id="CDR0000062905__226"><a href="/books/NBK65869.2/figure/CDR0000062905__226/?report=objectonly" target="object" rid-ob="figobCDR0000062905226">Figure</a></h4><p class="float-caption no_bottom_margin">Figure 2. Anatomy of the intrahepatic bile ducts. </p></div></div><p id="CDR0000062905__85">The bile ducts located within the liver are called intrahepatic bile ducts. Tumors of the intrahepatic bile ducts, also known as intrahepatic cholangiocarcinomas, originate from small intrahepatic ductules or large intrahepatic ducts that are proximal to the bifurcation of the right and left hepatic ducts. </p><p id="CDR0000062905__86"> 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 perihilar region and distal bile ducts.</p><div id="CDR0000062905__159"><h4>Perihilar bile ducts (hilum)</h4><p id="CDR0000062905__160">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 (Figure 3).</p><div class="iconblock whole_rhythm clearfix ten_col fig" id="figCDR000006290537" co-legend-rid="figlgndCDR000006290537"><a href="/books/NBK65869.2/figure/CDR0000062905__37/?report=objectonly" target="object" title="Figure" class="img_link icnblk_img figpopup" rid-figpopup="figCDR000006290537" rid-ob="figobCDR000006290537"><img class="small-thumb" src="/books/NBK65869.2/bin/CDR0000742477.gif" src-large="/books/NBK65869.2/bin/CDR0000742477.jpg" alt="Figure 3" /></a><div class="icnblk_cntnt" id="figlgndCDR000006290537"><h4 id="CDR0000062905__37"><a href="/books/NBK65869.2/figure/CDR0000062905__37/?report=objectonly" target="object" rid-ob="figobCDR000006290537">Figure</a></h4><p class="float-caption no_bottom_margin">Figure 3. Klatskin tumor anatomy. </p></div></div></div><div id="CDR0000062905__161"><h4>Distal extrahepatic bile ducts</h4><p id="CDR0000062905__162">This region includes the common bile duct and inserts into the small intestine. Tumors of this region are also known as extrahepatic cholangiocarcinomas.</p><p id="CDR0000062905__90">Approximately 50% of cholangiocarcinomas arise in the perihilar region, 40% arise in the distal extrahepatic region, and 10% arise in the intrahepatic region. </p></div></div><div id="CDR0000062905__91"><h3>Risk Factors and Clinical Presentation</h3><p id="CDR0000062905__92">Bile duct cancer may occur more frequently in patients with
a history of primary sclerosing cholangitis, chronic ulcerative colitis,
choledochal cysts, or infections with the liver fluke, <i>Clonorchis sinensis</i>.[<a class="bk_pop" href="#CDR0000062905_rl_1_2">2</a>] Extrahepatic and perihilar bile duct cancers frequently cause biliary tract obstruction, leading to symptoms of jaundice, weight loss, abdominal pain, fever, and pruritus. Intrahepatic bile duct cancer may be relatively indolent and difficult to clinically differentiate from metastatic adenocarcinoma deposits to the liver. </p></div><div id="CDR0000062905__93"><h3>Clinical Evaluation and Staging</h3><p id="CDR0000062905__94">Clinical staging is dependent on radiographic imaging, including computed tomography, magnetic resonance imaging, and magnetic resonance cholangiopancreatography, which demonstrates the extent of the primary tumor and assesses for the presence or absence of distant metastases. 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 performed to establish definitive pathologic staging. </p></div><div id="CDR0000062905__7"><h3>Prognosis</h3><p id="CDR0000062905__8">Complete surgical resection with negative surgical margins offers the only chance of cure for a diagnosis of bile duct cancer. Prognosis depends in part on the tumor&#x02019;s anatomic location, which affects its resectability. Because of their close proximity to major blood vessels and diffuse extension within the liver, bile duct tumors can be difficult to resect. Total resection is possible in 25% to 30% of lesions that originate in the distal bile duct, which is better than the resectability rate for lesions that occur in more proximal sites.[<a class="bk_pop" href="#CDR0000062905_rl_1_3">3</a>] </p><p id="CDR0000062905__151">Achievement of a negative surgical margin at the time of resection may be difficult but is associated with improved patient outcomes. For local, resectable tumors, a negative surgical margin, presence of involved lymph nodes, and presence of perineural invasion are significant 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, an underlying risk factor of primary sclerosing cholangitis, elevated CA 19-9, periductal infiltrating tumor growth pattern, and the presence of hepatic venous invasion 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></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&amp;targetsite=entrez&amp;targetcat=link&amp;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&amp;targetsite=entrez&amp;targetcat=link&amp;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&amp;targetsite=entrez&amp;targetcat=link&amp;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&amp;targetsite=entrez&amp;targetcat=link&amp;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&amp;targetsite=entrez&amp;targetcat=link&amp;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&amp;targetsite=entrez&amp;targetcat=link&amp;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&amp;targetsite=entrez&amp;targetcat=link&amp;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&amp;targetsite=entrez&amp;targetcat=link&amp;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&amp;targetsite=entrez&amp;targetcat=link&amp;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&amp;targetsite=entrez&amp;targetcat=link&amp;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&amp;targetsite=entrez&amp;targetcat=link&amp;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__203"><h3>Extrahepatic Bile Duct Cancer</h3><p id="CDR0000062905__204">Adenocarcinomas are the most common type of extrahepatic bile duct tumors. The histologic types include the following:[<a class="bk_pop" href="#CDR0000062905_rl_202_1">1</a>]</p><ul id="CDR0000062905__205"><li class="half_rhythm"><div>Carcinoma <i>in situ</i>.</div></li><li class="half_rhythm"><div>Adenocarcinoma, not otherwise specified (NOS).
</div></li><li class="half_rhythm"><div>Adenocarcinoma, intestinal type.
</div></li><li class="half_rhythm"><div>Mucinous adenocarcinoma.</div></li><li class="half_rhythm"><div>Clear cell adenocarcinoma.
</div></li><li class="half_rhythm"><div>Signet-ring cell carcinoma.
</div></li><li class="half_rhythm"><div>Adenosquamous carcinoma.
</div></li><li class="half_rhythm"><div>Squamous cell carcinoma.
</div></li><li class="half_rhythm"><div>Small cell (oat cell) carcinoma.
</div></li><li class="half_rhythm"><div>Undifferentiated carcinoma.<ul id="CDR0000062905__206"><li class="half_rhythm"><div>Spindle and giant cell types.</div></li><li class="half_rhythm"><div>Small cell types.</div></li></ul></div></li><li class="half_rhythm"><div>Papillomatosis.</div></li><li class="half_rhythm"><div>Papillary carcinoma, noninvasive.</div></li><li class="half_rhythm"><div>Papillary carcinoma, invasive.</div></li><li class="half_rhythm"><div>Carcinoma, NOS.</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 tumors. The histologic types 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>Adenocarcinoma, not otherwise specified (NOS).
</div></li><li class="half_rhythm"><div>Adenocarcinoma, intestinal type.
</div></li><li class="half_rhythm"><div>Mucinous adenocarcinoma.
</div></li><li class="half_rhythm"><div>Clear cell adenocarcinoma.
</div></li><li class="half_rhythm"><div>Signet-ring cell carcinoma.
</div></li><li class="half_rhythm"><div>Adenosquamous carcinoma.
</div></li><li class="half_rhythm"><div>Squamous cell carcinoma.
</div></li><li class="half_rhythm"><div>Small cell (oat cell) carcinoma.</div></li><li class="half_rhythm"><div>Undifferentiated carcinoma<ul id="CDR0000062905__210"><li class="half_rhythm"><div>Spindle and giant cell types.</div></li><li class="half_rhythm"><div>Small cell types.</div></li></ul></div></li><li class="half_rhythm"><div>Papillomatosis.</div></li><li class="half_rhythm"><div>Papillary carcinoma, noninvasive.</div></li><li class="half_rhythm"><div>Papillary carcinoma, invasive.</div></li><li class="half_rhythm"><div>Carcinoma, NOS</div></li></ul></div><div id="CDR0000062905__211"><h3>Intrahepatic Bile Duct Cancer </h3><p id="CDR0000062905__212">The most common histologic types of intrahepatic bile duct tumors include the following:[<a class="bk_pop" href="#CDR0000062905_rl_202_3">3</a>]</p><ul id="CDR0000062905__213"><li class="half_rhythm"><div>Intrahepatic cholangiocarcinoma.<ul id="CDR0000062905__214"><li class="half_rhythm"><div>Mass-forming tumor growth pattern. </div></li><li class="half_rhythm"><div> Periductal-infiltrating tumor growth pattern. </div></li><li class="half_rhythm"><div>Mixed mass-forming and periductal-infiltrating growth pattern. </div></li></ul></div></li><li class="half_rhythm"><div>Mixed hepatocellular. </div></li></ul></div><div id="CDR0000062905_rl_202"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000062905_rl_202_1">Distal bile duct. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 227-33.</div></li><li><div class="bk_ref" id="CDR0000062905_rl_202_2">Perihilar bile ducts. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 219-22.</div></li><li><div class="bk_ref" id="CDR0000062905_rl_202_3">Intrahepatic bile ducts. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 201-9.</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">Extrahepatic bile duct cancer is localized (resectable) or unresectable, with obvious prognostic importance. The TNM staging system is used for staging bile duct cancer, commonly following surgery and pathologic examination of the resected specimen. Evaluation of the extent of disease at laparotomy is most important for staging.</p><div id="CDR0000062905__28"><h4>Localized bile duct cancer</h4><p id="CDR0000062905__29">Localized bile duct cancer may be removed completely by the surgeon. These tumors represent a very small minority of cases and usually are distal common bile duct lesions. Patients undergoing surgery for this type of cancer have a 5-year survival rate of 25%. 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 should be determined. </p></div><div id="CDR0000062905__30"><h4>Unresectable bile duct cancer</h4><p id="CDR0000062905__31">Unresectable bile duct cancer represents the majority of bile duct tumors. Often the cancer invades directly into the portal vein, the adjacent liver, along the common bile duct, and to adjacent lymph nodes. Spread to distant parts of the body is uncommon but intra-abdominal metastases, particularly peritoneal metastases, do occur. For patients with unresectable bile duct cancer, management is directed at palliation.</p></div></div><div id="CDR0000062905__26"><h3>Definitions of TNM for Bile Duct Cancer </h3><div id="CDR0000062905__34"><h4>Definitions of TNM for extrahepatic bile duct cancer</h4><p id="CDR0000062905__35">The American Joint Committee on Cancer (AJCC) has designated staging by TNM classification to define extrahepatic bile duct cancer.[<a class="bk_pop" href="#CDR0000062905_rl_25_1">1</a>] Stages defined by TNM classification apply to all primary carcinomas arising in the distal extrahepatic 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 1, 2, 3, and 4 pertain to the distal bile duct group. </p><div id="CDR0000062905__43" class="table"><h3><span class="title">Table 1. Primary Tumor (T)<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.2/table/CDR0000062905__43/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__43_lrgtbl__"><table class="no_margin"><tbody><tr><td colspan="1" rowspan="1" style="vertical-align:top;">TX</td><td colspan="1" rowspan="1" style="vertical-align:top;">Primary tumor cannot be assessed.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T0</td><td colspan="1" rowspan="1" style="vertical-align:top;">No evidence of primary tumor.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">Tis</td><td colspan="1" rowspan="1" style="vertical-align:top;">Carcinoma<i> in situ</i>.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T1</td><td colspan="1" rowspan="1" style="vertical-align:top;">Tumor confined to the bile duct histologically.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T2</td><td colspan="1" rowspan="1" style="vertical-align:top;">Tumor invades beyond the wall of the bile duct.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T3</td><td colspan="1" rowspan="1" style="vertical-align:top;">Tumor invades the gallbladder, pancreas, duodenum, or other adjacent organs without involvement of the celiac axis or the superior mesenteric artery.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T4</td><td colspan="1" rowspan="1" style="vertical-align:top;">Tumor involves the celiac axis or the superior mesenteric artery.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Distal bile duct. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 227-33.</p></div></dd></dl></div></div></div><div id="CDR0000062905__44" class="table"><h3><span class="title">Table 2. Regional Lymph Nodes (N)<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.2/table/CDR0000062905__44/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__44_lrgtbl__"><table class="no_margin"><tbody><tr><td colspan="1" rowspan="1" style="vertical-align:top;">NX</td><td colspan="1" rowspan="1" style="vertical-align:top;">Regional lymph nodes cannot be assessed.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0</td><td colspan="1" rowspan="1" style="vertical-align:top;">No regional lymph node metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N1</td><td colspan="1" rowspan="1" style="vertical-align:top;">Regional lymph node metastasis.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Distal bile duct. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 227-33. </p></div></dd></dl></div></div></div><div id="CDR0000062905__45" class="table"><h3><span class="title">Table 3. Distant Metastasis (M)<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.2/table/CDR0000062905__45/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__45_lrgtbl__"><table class="no_margin"><tbody><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">No distant metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M1</td><td colspan="1" rowspan="1" style="vertical-align:top;">Distant metastasis.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Distal bile duct. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 227-33. </p></div></dd></dl></div></div></div><div id="CDR0000062905__46" class="table"><h3><span class="title">Table 4. Anatomic Stage/Prognostic Groups<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.2/table/CDR0000062905__46/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__46_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;">T</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">N</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">M</th></tr></thead><tbody><tr><td colspan="1" rowspan="1" style="vertical-align:top;">0</td><td colspan="1" rowspan="1" style="vertical-align:top;">Tis</td><td colspan="1" rowspan="1" style="vertical-align:top;">N0</td><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">IA</td><td colspan="1" rowspan="1" style="vertical-align:top;">T1</td><td colspan="1" rowspan="1" style="vertical-align:top;">N0</td><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">IB</td><td colspan="1" rowspan="1" style="vertical-align:top;">T2</td><td colspan="1" rowspan="1" style="vertical-align:top;">N0</td><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">IIA</td><td colspan="1" rowspan="1" style="vertical-align:top;">T3</td><td colspan="1" rowspan="1" style="vertical-align:top;">N0</td><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td></tr><tr><td colspan="1" rowspan="3" style="vertical-align:top;">IIB</td><td colspan="1" rowspan="1" style="vertical-align:top;">T1</td><td colspan="1" rowspan="1" style="vertical-align:top;">N1</td><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T2</td><td colspan="1" rowspan="1" style="vertical-align:top;">N1</td><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T3</td><td colspan="1" rowspan="1" style="vertical-align:top;">N1</td><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">III</td><td colspan="1" rowspan="1" style="vertical-align:top;">T4</td><td colspan="1" rowspan="1" style="vertical-align:top;">Any N</td><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">IV</td><td colspan="1" rowspan="1" style="vertical-align:top;">Any T</td><td colspan="1" rowspan="1" style="vertical-align:top;">Any N</td><td colspan="1" rowspan="1" style="vertical-align:top;">M1</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Distal bile duct. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 227-33. </p></div></dd></dl></div></div></div></div><div id="CDR0000062905__133"><h4>Definitions of TNM for perihilar bile duct cancer</h4><p id="CDR0000062905__134">The American Joint Committee on Cancer (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 5, 6, 7, and 8 pertain to the perihilar bile duct group.</p><div id="CDR0000062905__155" class="table"><h3><span class="title">Table 5. Primary Tumor (T)<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.2/table/CDR0000062905__155/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__155_lrgtbl__"><table class="no_margin"><tbody><tr><td colspan="1" rowspan="1" style="vertical-align:top;">TX </td><td colspan="1" rowspan="1" style="vertical-align:top;">Primary tumor cannot be assessed.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T0</td><td colspan="1" rowspan="1" style="vertical-align:top;">No evidence of primary tumor.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">Tis</td><td colspan="1" rowspan="1" style="vertical-align:top;">Carcinoma <i>in situ</i>.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T1</td><td colspan="1" rowspan="1" style="vertical-align:top;">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;">T2a</td><td colspan="1" rowspan="1" style="vertical-align:top;">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</td><td colspan="1" rowspan="1" style="vertical-align:top;">Tumor invades adjacent hepatic parenchyma.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T3</td><td colspan="1" rowspan="1" style="vertical-align:top;">Tumor invades unilateral branches of the portal vein or hepatic artery.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T4</td><td colspan="1" rowspan="1" style="vertical-align:top;">Tumor invades main portal vein or its branches bilaterally; or the common hepatic artery; or the second-order biliary radicals bilaterally; or unilateral second-order biliary radicals with contralateral portal vein or hepatic artery involvement.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Perihilar bile ducts. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 219-25.</p></div></dd></dl></div></div></div><div id="CDR0000062905__156" class="table"><h3><span class="title">Table 6. Regional Lymph Nodes (N)<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.2/table/CDR0000062905__156/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__156_lrgtbl__"><table class="no_margin"><tbody><tr><td colspan="1" rowspan="1" style="vertical-align:top;">NX</td><td colspan="1" rowspan="1" style="vertical-align:top;">Regional lymph nodes cannot be assessed.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0</td><td colspan="1" rowspan="1" style="vertical-align:top;">No regional lymph node metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N1</td><td colspan="1" rowspan="1" style="vertical-align:top;">Regional lymph node metastases (including nodes along the cystic duct, common bile duct, hepatic artery, and portal vein).</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N2</td><td colspan="1" rowspan="1" style="vertical-align:top;">Metastases to periaortic, pericaval, superior mesenteric artery, and celiac artery lymph nodes.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Perihilar bile ducts. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 219-25.</p></div></dd></dl></div></div></div><div id="CDR0000062905__157" class="table"><h3><span class="title">Table 7. Distant Metastasis (M)<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.2/table/CDR0000062905__157/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__157_lrgtbl__"><table class="no_margin"><tbody><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">No distant metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M1</td><td colspan="1" rowspan="1" style="vertical-align:top;">Distant metastasis.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Perihilar bile ducts. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 219-25.</p></div></dd></dl></div></div></div><div id="CDR0000062905__158" class="table"><h3><span class="title">Table 8. Anatomic Stage/Prognostic Groups<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.2/table/CDR0000062905__158/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__158_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;">T</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">N</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">M</th></tr></thead><tbody><tr><td colspan="1" rowspan="1" style="vertical-align:top;">0</td><td colspan="1" rowspan="1" style="vertical-align:top;">Tis</td><td colspan="1" rowspan="1" style="vertical-align:top;">N0</td><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">I</td><td colspan="1" rowspan="1" style="vertical-align:top;">T1</td><td colspan="1" rowspan="1" style="vertical-align:top;">N0</td><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">II</td><td colspan="1" rowspan="1" style="vertical-align:top;">T2a&#x02013;b</td><td colspan="1" rowspan="1" style="vertical-align:top;">N0</td><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">IIIA</td><td colspan="1" rowspan="1" style="vertical-align:top;">T3</td><td colspan="1" rowspan="1" style="vertical-align:top;">N0</td><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">IIIB</td><td colspan="1" rowspan="1" style="vertical-align:top;">T1&#x02013;3</td><td colspan="1" rowspan="1" style="vertical-align:top;">N1</td><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">IVA</td><td colspan="1" rowspan="1" style="vertical-align:top;">T4</td><td colspan="1" rowspan="1" style="vertical-align:top;">N0&#x02013;1</td><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td></tr><tr><td colspan="1" rowspan="2" style="vertical-align:top;">IVB</td><td colspan="1" rowspan="1" style="vertical-align:top;">Any T</td><td colspan="1" rowspan="1" style="vertical-align:top;">N2</td><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">Any T</td><td colspan="1" rowspan="1" style="vertical-align:top;">Any N</td><td colspan="1" rowspan="1" style="vertical-align:top;">M1</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Perihilar bile ducts. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 219-25.</p></div></dd></dl></div></div></div></div><div id="CDR0000062905__47"><h4>Definitions of TNM 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_3">3</a>]</p><p id="CDR0000062905__55">Tables 9, 10, 11, and 12 pertain to intrahepatic bile duct cancer.</p><div id="CDR0000062905__51" class="table"><h3><span class="title">Table 9. Primary Tumor (T)<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.2/table/CDR0000062905__51/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__51_lrgtbl__"><table class="no_margin"><tbody><tr><td colspan="1" rowspan="1" style="vertical-align:top;">TX</td><td colspan="1" rowspan="1" style="vertical-align:top;">Primary tumor cannot be assessed.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T0</td><td colspan="1" rowspan="1" style="vertical-align:top;">No evidence of primary tumor.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">Tis</td><td colspan="1" rowspan="1" style="vertical-align:top;">Carcinoma <i>in situ</i> (intraductal tumor).</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T1</td><td colspan="1" rowspan="1" style="vertical-align:top;">Solitary tumor without vascular invasion.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T2a</td><td colspan="1" rowspan="1" style="vertical-align:top;">Solitary tumor with vascular invasion.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T2b</td><td colspan="1" rowspan="1" style="vertical-align:top;">Multiple tumors, with or without vascular invasion.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T3</td><td colspan="1" rowspan="1" style="vertical-align:top;">Tumor perforating the visceral peritoneum or involving the local extrahepatic structures by direct invasion.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">T4</td><td colspan="1" rowspan="1" style="vertical-align:top;">Tumor with periductal invasion.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Intrahepatic bile ducts. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 201-9. </p></div></dd></dl></div></div></div><div id="CDR0000062905__52" class="table"><h3><span class="title">Table 10. Regional Lymph Nodes (N)<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.2/table/CDR0000062905__52/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__52_lrgtbl__"><table class="no_margin"><tbody><tr><td colspan="1" rowspan="1" style="vertical-align:top;">NX</td><td colspan="1" rowspan="1" style="vertical-align:top;">Regional lymph nodes cannot be assessed.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N0</td><td colspan="1" rowspan="1" style="vertical-align:top;">No regional lymph node metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">N1</td><td colspan="1" rowspan="1" style="vertical-align:top;">Regional lymph node metastasis present.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Intrahepatic bile ducts. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 201-9. </p></div></dd></dl></div></div></div><div id="CDR0000062905__53" class="table"><h3><span class="title">Table 11. Distant Metastasis (M)<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.2/table/CDR0000062905__53/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__53_lrgtbl__"><table class="no_margin"><tbody><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td><td colspan="1" rowspan="1" style="vertical-align:top;">No distant metastasis.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">M1</td><td colspan="1" rowspan="1" style="vertical-align:top;">Distant metastasis present.</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Intrahepatic bile ducts. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 201-9. </p></div></dd></dl></div></div></div><div id="CDR0000062905__54" class="table"><h3><span class="title">Table 12. Anatomic Stage/Prognostic Groups<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65869.2/table/CDR0000062905__54/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000062905__54_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;">T</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">N</th><th colspan="1" rowspan="1" style="text-align:center;vertical-align:top;">M</th></tr></thead><tbody><tr><td colspan="1" rowspan="1" style="vertical-align:top;">0</td><td colspan="1" rowspan="1" style="vertical-align:top;">Tis</td><td colspan="1" rowspan="1" style="vertical-align:top;">N0</td><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">I</td><td colspan="1" rowspan="1" style="vertical-align:top;">T1</td><td colspan="1" rowspan="1" style="vertical-align:top;">N0</td><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">II</td><td colspan="1" rowspan="1" style="vertical-align:top;">T2</td><td colspan="1" rowspan="1" style="vertical-align:top;">N0</td><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">III</td><td colspan="1" rowspan="1" style="vertical-align:top;">T3</td><td colspan="1" rowspan="1" style="vertical-align:top;">N0</td><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td></tr><tr><td colspan="1" rowspan="2" style="vertical-align:top;">IVA</td><td colspan="1" rowspan="1" style="vertical-align:top;">T4</td><td colspan="1" rowspan="1" style="vertical-align:top;">N0</td><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">Any T</td><td colspan="1" rowspan="1" style="vertical-align:top;">N1</td><td colspan="1" rowspan="1" style="vertical-align:top;">M0</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">IVB</td><td colspan="1" rowspan="1" style="vertical-align:top;">Any T</td><td colspan="1" rowspan="1" style="vertical-align:top;">Any N</td><td colspan="1" rowspan="1" style="vertical-align:top;">M1</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Reprinted with permission from AJCC: Intrahepatic bile ducts. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 201-9. </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">Distal bile duct. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 227-33.</div></li><li><div class="bk_ref" id="CDR0000062905_rl_25_2">Perihilar bile ducts. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 219-22.</div></li><li><div class="bk_ref" id="CDR0000062905_rl_25_3">Intrahepatic bile ducts. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 201-9.</div></li></ol></div></div><div id="CDR0000062905__56"><h2 id="_CDR0000062905__56_">Localized Bile Duct Cancer</h2><div id="CDR0000062905__215"><h3>Localized Extrahepatic Bile Duct Cancer</h3><p id="CDR0000062905__216">Complete surgical resection with negative surgical margins offers the only chance of cure for extrahepatic bile duct cancer. Because of the close proximity to major blood vessels and diffuse infiltration of adjacent bile ducts, bile duct tumors can be difficult to resect. Total resection is possible in 25% to 30% of lesions that originate in the distal bile duct, which is better than the resectability rate for lesions that occur in more proximal sites.[<a class="bk_pop" href="#CDR0000062905_rl_56_1">1</a>]</p><p id="CDR0000062905__217">No randomized trial data of adjuvant therapy for patients with localized disease are currently available. However, radiation therapy (external-beam radiation therapy [EBRT] with or without brachytherapy) has been reported to improve local control.[<a class="bk_pop" href="#CDR0000062905_rl_56_2">2</a>,<a class="bk_pop" href="#CDR0000062905_rl_56_3">3</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335158/" class="def">Level of evidence: 3iiiDiii</a>]</p><p id="CDR0000062905__218"><b>Standard treatment options:</b></p><ol id="CDR0000062905__219"><li class="half_rhythm"><div class="half_rhythm"><b>Surgery.</b> The optimum surgical procedure for carcinoma of the extrahepatic
bile duct will vary according to its location 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 are assessed at the time of surgery because they have prognostic significance. Surgery for bile duct cancer is usually extensive and has a high
operative mortality (5%&#x02013;10%) and low curability. Cases with cancer of the
lower end of the duct and regional lymph node involvement may warrant an
extensive resection (Whipple procedure), but bypass operations or endoluminal
stents are alternatives if intraoperatively the tumor is found to be unresectable.</div><div class="half_rhythm">In jaundiced patients, percutaneous transhepatic catheter drainage or endoscopic placement of a stent
for relief of biliary obstruction is considered before surgery, particularly if jaundice is severe, or an
element of azotemia is present.</div><div class="half_rhythm"><b>Adjuvant treatment options:</b><ol id="CDR0000062905__222"><li class="half_rhythm"><div class="half_rhythm"><b>EBRT.</b> Numerous retrospective studies have suggested a benefit of adding of EBRT after complete surgical resection.[<a class="bk_pop" href="#CDR0000062905_rl_56_2">2</a>,<a class="bk_pop" href="#CDR0000062905_rl_56_3">3</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 overall survival (OS) benefit. </div><div class="half_rhythm">One small randomized trial of 207 patients with pancreatic and periampullary cancers demonstrated no survival benefit of administering chemoradiation therapy after surgery. This study is limited, however, in that 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_56_4">4</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 class="half_rhythm"><b>Chemotherapy.</b> Numerous retrospective series have similarly suggested a benefit of adding chemotherapy after complete surgical resection.[<a class="bk_pop" href="#CDR0000062905_rl_56_5">5</a>,<a class="bk_pop" href="#CDR0000062905_rl_56_6">6</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335158/" class="def">Level of evidence: 3iiiDiii</a>] However, no prospective randomized trials have demonstrated an OS benefit. Two randomized trials of pancreaticobiliary tumors, which included distal bile duct cancer, have attempted to address the potential benefit of additional chemotherapy.</div><div class="half_rhythm">A multi-institutional Japanese study compared surgery alone with mitomycin-C and infusional 5-fluorouracil (5-FU) followed by 5-FU until progression.[<a class="bk_pop" href="#CDR0000062905_rl_56_7">7</a>] Among the subset of patients with bile duct cancer (n = 139), no survival benefit was seen.</div><div class="half_rhythm">The <a href="http://cancer.gov/clinicaltrials/search/view?version=healthprofessional&#x00026;cdrid=287023" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">ESPAC-3</a> (<a href="https://clinicaltrials.gov/show/NCT00058201" title="Study NCT00058201" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=clinical-trial">NCT00058201</a>) study randomly assigned 428 patients with periampullary cancer, which included 96 patients with bile duct cancer, to one of three arms: observation, 6 months of 5-FU/leukovorin, or 6 months of gemcitabine. [<a class="bk_pop" href="#CDR0000062905_rl_56_8">8</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335125/" class="def">Level of evidence: 1iiA</a>] In a preplanned subgroup analysis of the 96 patients with bile duct cancer, no benefit was seen among patients treated with chemotherapy. 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.</div></li><li class="half_rhythm"><div class="half_rhythm"><b>Clinical Trials.</b> All patients are encouraged to enroll in clinical trials for adjuvant therapies. </div></li></ol></div></li></ol><div id="CDR0000062905__TrialSearch_215_sid_3"><h4>Current Clinical Trials</h4><p id="CDR0000062905__TrialSearch_215_10">Check the list of NCI-supported cancer clinical trials that are now accepting patients with
<a href="http://www.cancer.gov/search/ClinicalTrialsLink.aspx?Diagnosis=38753&#x00026;tt=1&#x00026;format=2" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">localized extrahepatic bile duct cancer</a>. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.</p><p id="CDR0000062905__TrialSearch_215_18">General information about clinical trials is also available from the <a href="http://www.cancer.gov/about-cancer/treatment/clinical-trials" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">NCI website</a>.</p></div></div><div id="CDR0000062905__167"><h3>Localized Perihilar Bile Duct Cancer</h3><p id="CDR0000062905__168">For perihilar cholangiocarcinomas, 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 routine partial hepatectomy including the caudate lobe has improved long-term outcomes but may also be associated with increased postoperative complications.[<a class="bk_pop" href="#CDR0000062905_rl_56_9">9</a>] With this aggressive surgical approach, 5-year survival rates of 20% to 50% have been reported.[<a class="bk_pop" href="#CDR0000062905_rl_56_10">10</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 (Klatskin tumors) that can be resected.</p><p id="CDR0000062905__169">The primary site of relapse after surgical resection is local; however, distant recurrence has also been frequently reported.[<a class="bk_pop" href="#CDR0000062905_rl_56_11">11</a>] There has been no randomized trial of adjuvant therapy for patients with localized disease. Radiation therapy (EBRT with or without brachytherapy), however, has been reported to improve local control.[<a class="bk_pop" href="#CDR0000062905_rl_56_2">2</a>,<a class="bk_pop" href="#CDR0000062905_rl_56_3">3</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335158/" class="def">Level of evidence: 3iiiDiii</a>]</p><p id="CDR0000062905__170"><b>Standard treatment options:</b></p><ol id="CDR0000062905__171"><li class="half_rhythm"><div class="half_rhythm"><b>Surgery.</b> The optimal surgical procedure for carcinoma of the perihilar bile duct will vary according to its location 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 should be assessed at the time of surgery because of their prognostic significance. Operations for bile duct cancer are usually extensive and have a high operative mortality rate (5%&#x02013;10%) and low curability. </div><div class="half_rhythm">In jaundiced patients, percutaneous transhepatic catheter drainage or endoscopic placement of a stent for relief of biliary obstruction should be considered before surgery, particularly if jaundice is severe or an element of azotemia is present. </div></li></ol><p id="CDR0000062905__173"><b>Adjuvant treatment options:</b></p><ol id="CDR0000062905__174"><li class="half_rhythm"><div class="half_rhythm"><b>EBRT.</b> Numerous retrospective studies have suggested a benefit of adding EBRT after complete surgical resection.[<a class="bk_pop" href="#CDR0000062905_rl_56_2">2</a>,<a class="bk_pop" href="#CDR0000062905_rl_56_3">3</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. </div><div class="half_rhythm">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_56_4">4</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 class="half_rhythm"><b>Chemotherapy.</b> Numerous retrospective series have similarly suggested a benefit of adding chemotherapy after complete surgical resection.[<a class="bk_pop" href="#CDR0000062905_rl_56_5">5</a>,<a class="bk_pop" href="#CDR0000062905_rl_56_6">6</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335158/" class="def">Level of evidence: 3iiiDiii</a>] However, no prospective randomized trials have demonstrated an OS benefit. </div><div class="half_rhythm">One randomized trial of pancreaticobiliary tumors, which included bile duct cancer, attempted to address the potential benefit of additional chemotherapy. In a multi-institutional Japanese study that compared surgery alone with mitomycin-C and infusional 5-FU followed by 5-FU until progression, among the subset of patients with bile duct cancer (n = 139), there was no survival benefit.[<a class="bk_pop" href="#CDR0000062905_rl_56_7">7</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335125/" class="def">Level of evidence: 1iiA</a>] </div></li><li class="half_rhythm"><div class="half_rhythm"><b>Clinical Trials.</b> All patients are encouraged to enroll in clinical trials for adjuvant therapies. </div></li></ol><div id="CDR0000062905__TrialSearch_167_sid_4"><h4>Current Clinical Trials</h4><p id="CDR0000062905__TrialSearch_167_10">Check the list of NCI-supported cancer clinical trials that are now accepting patients with
<a href="http://www.cancer.gov/search/ClinicalTrialsLink.aspx?Diagnosis=766943&#x00026;tt=1&#x00026;format=2" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">stage I perihilar bile duct cancer</a> and <a href="http://www.cancer.gov/search/ClinicalTrialsLink.aspx?Diagnosis=766944&#x00026;tt=1&#x00026;format=2" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">stage II perihilar bile duct cancer</a>. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.</p><p id="CDR0000062905__TrialSearch_167_18">General information about clinical trials is also available from the <a href="http://www.cancer.gov/about-cancer/treatment/clinical-trials" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">NCI website</a>.</p></div></div><div id="CDR0000062905__177"><h3>Localized Intrahepatic Bile Duct Cancer</h3><p id="CDR0000062905__178">For intrahepatic bile duct cancer, hepatic resection to achieve negative margins is the curative procedure. If a major liver resection is necessary to achieve negative surgical margins, preoperative portal vein embolization may be considered to increase the volume of the remnant liver.</p><p id="CDR0000062905__179"><b>Standard treatment options:</b></p><ol id="CDR0000062905__180"><li class="half_rhythm"><div><b>Surgery. </b>Partial liver resection or partial hepatectomy to acheive negative margins resection is the mainstay of cure for patients with intrahepatic cholangiocarcinoma. 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. The role of routine portal lymphadenectomy has not been well established because of the risk of common bile duct devascularization. </div></li></ol><p id="CDR0000062905__181"><b>Adjuvant treatment options:</b></p><ol id="CDR0000062905__182"><li class="half_rhythm"><div class="half_rhythm"><b>EBRT.</b> There is little published literature evaluating the role of EBRT after resection for intrahepatic cholangiocarcinoma. </div></li><li class="half_rhythm"><div class="half_rhythm"><b>Chemotherapy.</b> Numerous retrospective series have similarly suggested a benefit to the addition of chemotherapy after complete surgical resection.[<a class="bk_pop" href="#CDR0000062905_rl_56_5">5</a>,<a class="bk_pop" href="#CDR0000062905_rl_56_6">6</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335158/" class="def">Level of evidence: 3iiiDiii</a>] However, to date, no prospective randomized trials have demonstrated an OS benefit. </div><div class="half_rhythm">One randomized trial of pancreaticobiliary tumors, which included bile duct cancer, has attempted to address the potential benefit of additional chemotherapy. In a multi-institutional Japanese study which compared surgery alone with mitomycin-C and infusional 5-FU followed by 5-FU until progression, among the subset of patients with bile duct cancer (n = 139) , no survival benefit was seen.[<a class="bk_pop" href="#CDR0000062905_rl_56_7">7</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335125/" class="def">Level of evidence: 1iiA</a>] </div></li><li class="half_rhythm"><div class="half_rhythm"><b>Clinical Trials.</b> All patients are encouraged to enroll in clinical trials for adjuvant therapies. </div></li></ol><div id="CDR0000062905__TrialSearch_177_sid_5"><h4>Current Clinical Trials</h4><p id="CDR0000062905__TrialSearch_177_10">Check the list of NCI-supported cancer clinical trials that are now accepting patients with
<a href="http://www.cancer.gov/search/ClinicalTrialsLink.aspx?Diagnosis=767110&#x00026;tt=1&#x00026;format=2" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">intrahepatic bile duct cancer</a>. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.</p><p id="CDR0000062905__TrialSearch_177_18">General information about clinical trials is also available from the <a href="http://www.cancer.gov/about-cancer/treatment/clinical-trials" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">NCI website</a>.</p></div></div><div id="CDR0000062905_rl_56"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000062905_rl_56_1">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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 1280374</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_56_2">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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 21497455</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_56_3">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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 17276614</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_56_4">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&amp;targetsite=entrez&amp;targetcat=link&amp;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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 10615932</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_56_5">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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 10919004</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_56_6">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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 19953713</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_56_7">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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 12365016</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_56_8">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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 22782416</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_56_9">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&amp;targetsite=entrez&amp;targetcat=link&amp;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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 9742921</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_56_10">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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 12407338</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_56_11">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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 17453285</span></a>]</div></li></ol></div></div><div id="CDR0000062905__63"><h2 id="_CDR0000062905__63_">Unresectable, Recurrent, or Metastatic Bile Duct Cancer</h2><p id="CDR0000062905__149">Patients with unresectable bile duct cancer have cancer that
cannot be completely removed by the surgeon. These patients represent the
majority of cases of bile duct cancer. Often a proximal bile duct cancer
invades directly into the adjacent liver or into the hepatic artery or portal
vein. Portal hypertension may result. Spread to distant parts of the body is
uncommon, though transperitoneal and hematogenous hepatic metastases do occur
with bile duct cancer of all sites. Invasion along the biliary tree and into
the liver is common.
Moreover, the majority of patients who undergo resection will develop recurrent disease within the hepatobiliary system or, less frequently, at distant sites.</p><p id="CDR0000062905__150">Patients with unresectable, recurrent, or metastatic bile duct cancer should be considered for inclusion in clinical trials whenever possible. Information about ongoing clinical trials is available from the <a href="http://www.cancer.gov/about-cancer/treatment/clinical-trials" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">NCI website</a>.</p><div id="CDR0000062905__184"><h3>Unresectable, Recurrent, or Metastatic Extrahepatic Bile Duct Cancer</h3><p id="CDR0000062905__185"><b>Standard treatment options:</b></p><ol id="CDR0000062905__186"><li class="half_rhythm"><div class="half_rhythm"><b>Palliative therapy.</b> 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 by anastomosis of
the bile duct to the bowel or by the placement of bile duct stents by
operative, endoscopic, or percutaneous techniques.[<a class="bk_pop" href="#CDR0000062905_rl_63_1">1</a>,<a class="bk_pop" href="#CDR0000062905_rl_63_2">2</a>]</div><div class="half_rhythm">Palliative radiation
therapy after biliary bypass or intubation may be beneficial, and patients may be candidates for inclusion in clinical trials that explore ways to improve the effects of radiation therapy with various radiation sensitizers, such as hyperthermia, radiosensitizer drugs, or cytotoxic chemotherapeutic agents.
If a percutaneous catheter has been placed, it can be used as a conduit for
placement of brachytherapy sources.[<a class="bk_pop" href="#CDR0000062905_rl_63_3">3</a>,<a class="bk_pop" href="#CDR0000062905_rl_63_4">4</a>] Information about ongoing clinical trials is available from the <a href="http://www.cancer.gov/about-cancer/treatment/clinical-trials" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">NCI website</a>. </div></li><li class="half_rhythm"><div class="half_rhythm"><b>Systemic chemotherapy.</b> Systemic chemotherapy is appropriate for selected patients with adequate performance status and intact organ function. Fluoropyrimidines, gemcitabine, platinum agents, and docetaxel have been reported to produce transient partial remissions in a minority of patients. </div><div class="half_rhythm">A randomized, phase III study (<a href="http://cancer.gov/clinicaltrials/search/view?version=healthprofessional&#x00026;cdrid=455013" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">NCT00262769</a>) of up to 6 months of gemcitabine versus gemcitabine and cisplatin in 410 patients with unresectable, recurrent, or metastatic biliary tract carcinoma demonstrated an improvement in median overall survival (OS) among patients treated with combination therapy (11.7 months vs. 8.1 months; hazard ratio [HR], 0.64; 95% confidence interval [CI], 0.52&#x02013;0.80); <i>P</i> &#x0003c; .001).[<a class="bk_pop" href="#CDR0000062905_rl_63_5">5</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335125/" class="def">Level of evidence: 1iiA</a>] A similar median OS benefit was demonstrated in all subgroups, including 73 patients with extrahepatic bile duct cancer and 57 patients with hilar tumors. Grade 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></ol><p id="CDR0000062905__189">Other drugs and drug combinations await evaluation in randomized trials. </p><div id="CDR0000062905__TrialSearch_184_sid_5"><h4>Current Clinical Trials</h4><p id="CDR0000062905__TrialSearch_184_10">Check the list of NCI-supported cancer clinical trials that are now accepting patients with
<a href="http://www.cancer.gov/search/ClinicalTrialsLink.aspx?Diagnosis=38754&#x00026;tt=1&#x00026;format=2" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">unresectable extrahepatic bile duct cancer</a>, <a href="http://www.cancer.gov/search/ClinicalTrialsLink.aspx?Diagnosis=38755&#x00026;tt=1&#x00026;format=2" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">recurrent extrahepatic bile duct cancer</a> and <a href="http://www.cancer.gov/search/ClinicalTrialsLink.aspx?Diagnosis=709296&#x00026;tt=1&#x00026;format=2" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">metastatic extrahepatic bile duct cancer</a>. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.</p><p id="CDR0000062905__TrialSearch_184_18">General information about clinical trials is also available from the <a href="http://www.cancer.gov/about-cancer/treatment/clinical-trials" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">NCI website</a>.</p></div></div><div id="CDR0000062905__190"><h3>Unresectable, Recurrent, or Metastatic Perihilar Bile Duct Cancer</h3><p id="CDR0000062905__191"><b>Standard treatment options:</b></p><ol id="CDR0000062905__192"><li class="half_rhythm"><div class="half_rhythm"><b>Palliative therapy.</b> Relief of biliary obstruction is warranted when symptoms such as pruritus and hepatic dysfunction outweigh other symptoms from the cancer. When possible, such palliation can be achieved by anastomosis of the bile duct to the bowel or by the placement of bile duct stents by operative, endoscopic, or percutaneous techniques.[<a class="bk_pop" href="#CDR0000062905_rl_63_1">1</a>,<a class="bk_pop" href="#CDR0000062905_rl_63_2">2</a>]</div><div class="half_rhythm">Palliative radiation therapy after biliary bypass or intubation may be beneficial, and patients may be candidates for inclusion in clinical trials that explore ways to improve the effects of radiation therapy with various radiation sensitizers, such as hyperthermia, radiosensitizer drugs, or cytotoxic chemotherapeutic agents. If a percutaneous catheter has been placed, it can be used as a conduit for placement of sources for brachytherapy.[<a class="bk_pop" href="#CDR0000062905_rl_63_3">3</a>,<a class="bk_pop" href="#CDR0000062905_rl_63_4">4</a>]</div></li><li class="half_rhythm"><div class="half_rhythm"><b>Systemic chemotherapy.</b> Systemic chemotherapy is appropriate for selected patients with adequate performance status and intact organ function. Fluoropyrimidines, gemcitabine, platinum agents, and docetaxel have been reported to produce transient partial remissions in a minority of patients. </div><div class="half_rhythm">A randomized, phase III study (<a href="http://cancer.gov/clinicaltrials/search/view?version=healthprofessional&#x00026;cdrid=455013" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">NCT00262769</a>) of up to 6 months of gemcitabine versus gemcitabine and cisplatin in 410 patients with unresectable, recurrent, or metastatic biliary tract carcinoma demonstrated an improvement in median OS among patients treated with combination therapy (11.7 months vs. 8.1 months; HR, 0.64; (95% CI, 0.52&#x02013;0.80); <i>P</i> &#x0003c; .001.[<a class="bk_pop" href="#CDR0000062905_rl_63_5">5</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335125/" class="def">Level of evidence: 1iiA</a>] A similar median OS benefit was demonstrated in all subgroups, including 73 patients with extrahepatic bile duct cancer and 57 patients with hilar tumors. Grade 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><li class="half_rhythm"><div class="half_rhythm"><b>Neoadjuvant chemoradiation and orthotopic liver transplantation.</b> Neoadjuvant chemoradiation and orthotopic liver transplantation have been evaluated in carefully selected patients with locally unresectable perihilar bile duct cancer.[<a class="bk_pop" href="#CDR0000062905_rl_63_6">6</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000593395/" class="def">Level of evidence: 3iiiD</a>] This approach is not considered standard of care and should only be performed in the context of a clinical trial in specialized transplant centers. </div></li></ol><p id="CDR0000062905__195">Other drugs and drug combinations await evaluation in randomized trials. </p><div id="CDR0000062905__TrialSearch_190_sid_6"><h4>Current Clinical Trials</h4><p id="CDR0000062905__TrialSearch_190_10">Check the list of NCI-supported cancer clinical trials that are now accepting patients with
<a href="http://www.cancer.gov/search/ClinicalTrialsLink.aspx?Diagnosis=766945&#x00026;tt=1&#x00026;format=2" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">stage III perihilar bile duct cancer</a> and <a href="http://www.cancer.gov/search/ClinicalTrialsLink.aspx?Diagnosis=766948&#x00026;tt=1&#x00026;format=2" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">stage IV perihilar bile duct cancer</a>. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.</p><p id="CDR0000062905__TrialSearch_190_18">General information about clinical trials is also available from the <a href="http://www.cancer.gov/about-cancer/treatment/clinical-trials" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">NCI website</a>.</p></div></div><div id="CDR0000062905__196"><h3>Unresectable, Recurrent, or Metastatic Intrahepatic Bile Duct Cancer</h3><p id="CDR0000062905__197"><b>Standard treatment options:</b></p><ol id="CDR0000062905__198"><li class="half_rhythm"><div class="half_rhythm"><b>Palliative therapy.</b> 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_63_1">1</a>,<a class="bk_pop" href="#CDR0000062905_rl_63_2">2</a>]</div><div class="half_rhythm">Radiation therapy may be beneficial, and patients may be candidates for inclusion in clinical trials that explore ways to improve the effects of radiation therapy with various radiation sensitizers, such as hyperthermia, radiosensitizer drugs, or cytotoxic chemotherapeutic agents. If a percutaneous catheter has been placed, it can be used as a conduit for placement of brachytherapy sources.[<a class="bk_pop" href="#CDR0000062905_rl_63_3">3</a>,<a class="bk_pop" href="#CDR0000062905_rl_63_4">4</a>] Limited but emerging data are available regarding the potential role for alternative liver-directed therapies, including stereotactic body radiation therapy [<a class="bk_pop" href="#CDR0000062905_rl_63_7">7</a>] and intra-arterial embolization.[<a class="bk_pop" href="#CDR0000062905_rl_63_8">8</a>] </div></li><li class="half_rhythm"><div class="half_rhythm"><b>Systemic chemotherapy.</b> Systemic chemotherapy is appropriate for selected patients with adequate performance status and intact organ function. Fluoropyrimidines, gemcitabine, platinum agents, and docetaxel have been reported to produce transient partial remissions in a minority of patients. </div><div class="half_rhythm">A randomized, phase III study (<a href="http://cancer.gov/clinicaltrials/search/view?version=healthprofessional&#x00026;cdrid=455013" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">NCT00262769</a>) of up to 6 months of gemcitabine versus gemcitabine and cisplatin in 410 patients with unresectable, recurrent, or metastatic biliary tract carcinoma demonstrated an improvement in median OS among patients treated with combination therapy (11.7 months vs. 8.1 months; HR, 0.64; (95% CI, 0.52&#x02013;0.80); <i>P</i> &#x0003c; .001.[<a class="bk_pop" href="#CDR0000062905_rl_63_5">5</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000561227/" class="def">Level of evidence: 3iii</a>] A similar median OS benefit was demonstrated in all subgroups, including 73 patients with extrahepatic bile duct cancer and 57 patients with hilar tumors. Grade 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></ol><p id="CDR0000062905__201">Other drugs and drug combinations await evaluation in randomized trials.</p><div id="CDR0000062905__TrialSearch_196_sid_7"><h4>Current Clinical Trials</h4><p id="CDR0000062905__TrialSearch_196_10">Check the list of NCI-supported cancer clinical trials that are now accepting patients with
<a href="http://www.cancer.gov/search/ClinicalTrialsLink.aspx?Diagnosis=767126&#x00026;tt=1&#x00026;format=2" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">stage III intrahepatic bile duct cancer</a>, <a href="http://www.cancer.gov/search/ClinicalTrialsLink.aspx?Diagnosis=767127&#x00026;tt=1&#x00026;format=2" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">stage IV intrahepatic bile duct cancer</a> and <a href="http://www.cancer.gov/search/ClinicalTrialsLink.aspx?Diagnosis=768153&#x00026;tt=1&#x00026;format=2" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">recurrent intrahepatic bile duct cancer</a>. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.</p><p id="CDR0000062905__TrialSearch_196_18">General information about clinical trials is also available from the <a href="http://www.cancer.gov/about-cancer/treatment/clinical-trials" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">NCI website</a>.</p></div></div><div id="CDR0000062905_rl_63"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000062905_rl_63_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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 7513906</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_63_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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 15067620</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_63_3">Fritz P, Brambs HJ, Schraube P, et al.: Combined external beam radiotherapy and intraluminal high dose rate brachytherapy on bile duct carcinomas. Int J Radiat Oncol Biol Phys 29 (4): 855-61, 1994. [<a href="https://pubmed.ncbi.nlm.nih.gov/8040034" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 8040034</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_63_4">Shin HS, Seong J, Kim WC, et al.: Combination of external beam irradiation and high-dose-rate intraluminal brachytherapy for inoperable carcinoma of the extrahepatic bile ducts. Int J Radiat Oncol Biol Phys 57 (1): 105-12, 2003. [<a href="https://pubmed.ncbi.nlm.nih.gov/12909222" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 12909222</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_63_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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 20375404</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_63_6">Rea DJ, Heimbach JK, Rosen CB, et al.: Liver transplantation with neoadjuvant chemoradiation is more effective than resection for hilar cholangiocarcinoma. Ann Surg 242 (3): 451-8; discussion 458-61, 2005. [<a href="/pmc/articles/PMC1357753/" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pmc">PMC free article<span class="bk_prnt">: PMC1357753</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/16135931" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 16135931</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_63_7">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&amp;targetsite=entrez&amp;targetcat=link&amp;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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 22553982</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062905_rl_63_8">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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 23846786</span></a>]</div></li></ol></div></div><div id="CDR0000062905__71"><h2 id="_CDR0000062905__71_">Changes to This Summary (09/09/2015)</h2><p id="CDR0000062905__72">The PDQ cancer information summaries are reviewed regularly and updated as
new information becomes available. This section describes the latest
changes made to this summary as of the date above.</p><p id="CDR0000062905__230"><b><a href="#CDR0000062905__1">General Information About Bile Duct Cancer </a></b></p><p id="CDR0000062905__231">Editorial changes were made to this section.</p><p id="CDR0000062905__disclaimerHP_3">This summary is written and maintained by the <a href="http://www.cancer.gov/publications/pdq/editorial-boards/adult-treatment" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">PDQ Adult Treatment Editorial Board</a>, which is
editorially independent of NCI. The summary reflects an independent review of
the literature and does not represent a policy statement of NCI or NIH. More
information about summary policies and the role of the PDQ Editorial Boards in
maintaining the PDQ summaries can be found on the <a href="#CDR0000062905__AboutThis_1">About This PDQ Summary</a> and <a href="http://www.cancer.gov/publications/pdq" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">PDQ&#x000ae; - NCI's Comprehensive Cancer Database</a> pages.
</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="http://www.cancer.gov/publications/pdq/editorial-boards/adult-treatment" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;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 Treatment are:</p><ul><li class="half_rhythm"><div>Jason E. Faris, MD (Massachusetts General Hospital)</div></li><li class="half_rhythm"><div>Jennifer Wo, MD (Massachusetts General Hospital)</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="http://www.cancer.gov/contact/email-us" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;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 &#x0201c;NCI&#x02019;s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].&#x0201d;</p><p id="CDR0000062905__AboutThis_14">The preferred citation for this PDQ summary is:</p><p id="CDR0000062905__AboutThis_15">National Cancer Institute: PDQ&#x000ae; Bile Duct Cancer Treatment. Bethesda, MD: National Cancer Institute. Date last modified &#x0003c;MM/DD/YYYY&#x0003e;. Available at: <a href="http://www.cancer.gov/types/liver/hp/bile-duct-treatment-pdq" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">http://www.cancer.gov/types/liver/hp/bile-duct-treatment-pdq</a>. Accessed &#x0003c;MM/DD/YYYY&#x0003e;.</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="http://visualsonline.cancer.gov/" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">Visuals Online</a>, a collection of over 2,000 scientific images.
</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 &#x0201c;standard&#x0201d; or &#x0201c;under clinical evaluation.&#x0201d; 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="http://www.cancer.gov/about-cancer/managing-care" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;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="http://www.cancer.gov/contact" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">Contact Us for Help</a> page. Questions can also be submitted to Cancer.gov through the website&#x02019;s <a href="http://www.cancer.gov/contact/email-us" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">Email Us</a>.</p></div></div><div id="CDR0000062905__GetMore_3"><h2 id="_CDR0000062905__GetMore_3_">Get More Information From NCI</h2><p id="CDR0000062905__GetMore_15"><i><b>Call 1-800-4-CANCER</b></i></p><p id="CDR0000062905__GetMore_16">For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 8:00 a.m. to 8:00 p.m., Eastern Time. A trained Cancer Information Specialist is available to answer your questions.</p><p id="CDR0000062905__GetMore_25"><i><b>Chat online
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class="portlet"><div class="portlet_head"><div 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$=inpage&amp;link_id=inpage">General Information About Bile Duct Cancer </a></li><li><a href="#CDR0000062905__202" ref="log$=inpage&amp;link_id=inpage">Cellular Classification of Bile Duct Cancer</a></li><li><a href="#CDR0000062905__25" ref="log$=inpage&amp;link_id=inpage">Stage Information for Bile Duct Cancer</a></li><li><a href="#CDR0000062905__56" ref="log$=inpage&amp;link_id=inpage">Localized Bile Duct Cancer</a></li><li><a href="#CDR0000062905__63" ref="log$=inpage&amp;link_id=inpage">Unresectable, Recurrent, or Metastatic Bile Duct Cancer</a></li><li><a href="#CDR0000062905__71" ref="log$=inpage&amp;link_id=inpage">Changes to This Summary (09/09/2015)</a></li><li><a href="#CDR0000062905__AboutThis_1" ref="log$=inpage&amp;link_id=inpage">About This PDQ Summary</a></li><li><a href="#CDR0000062905__GetMore_3" ref="log$=inpage&amp;link_id=inpage">Get More Information From NCI</a></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Related publications</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="document-links" 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/NBK65851/">Patient Version</a></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Related 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