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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="#__NBK65908_dtls__">Show details</a><div style="display:none" class="ui-widget" id="__NBK65908_dtls__"><div>Bethesda (MD): <a href="http://www.cancer.gov/" ref="pagearea=page-banner&targetsite=external&targetcat=link&targettype=publisher">National Cancer Institute (US)</a>; 2002-.</div></div><div class="half_rhythm"></div><div class="bk_noprnt"><form method="get" action="/books/n/pdqcis/" id="bk_srch"><div class="bk_search"><label for="bk_term" class="offscreen_noflow">Search term</label><input type="text" title="Search this book" id="bk_term" name="term" value="" data-jig="ncbiclearbutton" /> <input type="submit" class="jig-ncbibutton" value="Search this book" submit="false" style="padding: 0.1em 0.4em;" /></div></form></div></div></div></div></div>
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<div class="main-content lit-style" itemscope="itemscope" itemtype="http://schema.org/CreativeWork"><div class="meta-content fm-sec"><h1 id="_NBK65908_"><span class="title" itemprop="name">Extragonadal Germ Cell Tumors Treatment (PDQ®)</span></h1><div class="subtitle whole_rhythm">Health Professional Version</div><p class="contrib-group"><span itemprop="author">PDQ Adult Treatment Editorial Board</span>.</p><p class="small">Published online: February 25, 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="CDR0000062939__118">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of extragonadal germ cell tumors. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.</p><p id="CDR0000062939__119">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="CDR0000062939__1"><h2 id="_CDR0000062939__1_">General Information About Extragonadal Germ Cell Tumors</h2><div id="CDR0000062939__115"><h3>Incidence and Mortality</h3><p id="CDR0000062939__3">Extragonadal germ cell tumors are rare and account for only a small percentage
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of all germ cell tumors. However, the true incidence of these tumors may
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conceivably be higher than originally thought because of failure to diagnose
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|
them properly.
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</p></div><div id="CDR0000062939__116"><h3>Related Summaries</h3><ul id="CDR0000062939__112"><li class="half_rhythm"><div>
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|
<a href="/books/n/pdqcis/CDR0000062935/">Ovarian Germ Cell Tumors Treatment</a>
|
|
</div></li><li class="half_rhythm"><div>
|
|
<a href="/books/n/pdqcis/CDR0000062899/">Testicular Cancer Treatment</a>
|
|
</div></li></ul></div></div><div id="CDR0000062939__68"><h2 id="_CDR0000062939__68_">Cellular Classification of Extragonadal Germ Cell Tumors</h2><p id="CDR0000062939__113">Extragonadal germ cell tumors can be benign (teratoma) or malignant. The
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latter group can be divided into seminoma and nonseminoma germ cell tumors, which include the following:</p><ul id="CDR0000062939__114"><li class="half_rhythm"><div>Embryonal carcinomas.</div></li><li class="half_rhythm"><div>Malignant teratomas.</div></li><li class="half_rhythm"><div>Endodermal sinus tumors.</div></li><li class="half_rhythm"><div>Choriocarcinomas.</div></li><li class="half_rhythm"><div>Mixed germ cell tumors.</div></li></ul><p id="CDR0000062939__4"> Extragonadal germ cell tumors occur much more commonly in males than in females [<a class="bk_pop" href="#CDR0000062939_rl_68_1">1</a>] and are usually seen in young adults. They are aggressive neoplasms and can arise virtually anywhere, but typically
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the site of origin is in the midline (mediastinum, retroperitoneum, or pineal
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gland). Gonadal origin should be excluded by careful testicular examination
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and ultrasound. The diagnosis can be difficult and should be considered in any
|
|
patient with a poorly defined epithelial malignancy, particularly young
|
|
individuals with midline masses.[<a class="bk_pop" href="#CDR0000062939_rl_68_2">2</a>,<a class="bk_pop" href="#CDR0000062939_rl_68_3">3</a>]
|
|
</p><p id="CDR0000062939__12">An international germ cell tumor prognostic classification has been developed
|
|
based on a retrospective analysis of 5,202 patients with metastatic
|
|
nonseminomatous germ cell tumors and 660 patients with metastatic seminomatous germ cell
|
|
tumors.[<a class="bk_pop" href="#CDR0000062939_rl_68_4">4</a>] All patients received treatment with cisplatin-containing or carboplatin-containing therapy as their first chemotherapy course. The prognostic
|
|
classification, shown below, was agreed on in early 1997 by all major clinical
|
|
trial groups worldwide and should be used for the reporting of clinical trials'
|
|
results of patients with extragonadal germ cell tumors.
|
|
</p><div id="CDR0000062939__13"><h3>Good Prognosis</h3><div id="CDR0000062939__41"><h4>Nonseminoma</h4><ul id="CDR0000062939__15"><li class="half_rhythm"><div>Testis/retroperitoneal primary </div><div><i>and</i></div></li><li class="half_rhythm"><div>No nonpulmonary visceral metastases </div><div><i>and</i>
|
|
</div></li><li class="half_rhythm"><div>Good markers - all of:
|
|
<dl id="CDR0000062939__16" class="temp-labeled-list"><dt>-</dt><dd><p class="no_top_margin">AFP less than 1,000 ng/mL </p><p><i>and</i></p></dd><dt>-</dt><dd><p class="no_top_margin">hCG less than 5,000 iu/L (1,000 ng/mL) </p><p><i>and</i></p></dd><dt>-</dt><dd><p class="no_top_margin">LDH less than 1.5 x upper limit of normal
|
|
</p></dd></dl>
|
|
</div></li></ul><p id="CDR0000062939__17">56% of nonseminomas
|
|
</p><p id="CDR0000062939__18">5-year progression-free survival (PFS) rate of 89%</p><p id="CDR0000062939__47">
|
|
5-year survival rate of 92%
|
|
</p></div><div id="CDR0000062939__42"><h4>Seminoma</h4><ul id="CDR0000062939__20"><li class="half_rhythm"><div>Any primary site </div><div><i>and</i>
|
|
</div></li><li class="half_rhythm"><div>No nonpulmonary visceral metastases</div><div><i>and</i>
|
|
</div></li><li class="half_rhythm"><div>Normal AFP, any hCG, any LDH
|
|
</div></li></ul><p id="CDR0000062939__21">90% of seminomas
|
|
</p><p id="CDR0000062939__22">5-year PFS rate of 82%
|
|
</p><p id="CDR0000062939__48">5-year survival rate of 86%</p></div></div><div id="CDR0000062939__23"><h3>Intermediate Prognosis</h3><div id="CDR0000062939__43"><h4>Nonseminoma</h4><ul id="CDR0000062939__25"><li class="half_rhythm"><div>Testis/retroperitoneal primary </div><div><i>and</i>
|
|
</div></li><li class="half_rhythm"><div>No nonpulmonary visceral metastases </div><div><i>and</i></div></li><li class="half_rhythm"><div>Intermediate markers - any of:
|
|
<dl id="CDR0000062939__26" class="temp-labeled-list"><dt>-</dt><dd><p class="no_top_margin">AFP 1,000 ng/mL or greater and 10,000 ng/mL or less</p><p><i>or</i>
|
|
</p></dd><dt>-</dt><dd><p class="no_top_margin">hCG 5,000 iu/L or greater and 50,000 iu/L or less </p><p><i>or</i>
|
|
</p></dd><dt>-</dt><dd><p class="no_top_margin">LDH 1.5 × N or greater and 10 × N
|
|
or less</p></dd></dl>
|
|
</div></li></ul><p id="CDR0000062939__27">28% of nonseminomas
|
|
</p><p id="CDR0000062939__28">5-year PFS rate of 75%
|
|
</p><p id="CDR0000062939__49">
|
|
5-year survival rate of 80%</p></div><div id="CDR0000062939__44"><h4>Seminoma</h4><ul id="CDR0000062939__30"><li class="half_rhythm"><div>Any primary site </div><div><i>and</i></div></li><li class="half_rhythm"><div>Nonpulmonary visceral metastases </div><div><i>and</i>
|
|
</div></li><li class="half_rhythm"><div>Normal AFP, any hCG, any LDH
|
|
</div></li></ul><p id="CDR0000062939__31">10% of seminomas
|
|
</p><p id="CDR0000062939__32">5-year PFS rate of 67%
|
|
</p><p id="CDR0000062939__50">5-year survival rate of 72%
|
|
</p></div></div><div id="CDR0000062939__33"><h3>Poor Prognosis</h3><div id="CDR0000062939__45"><h4>Nonseminoma</h4><ul id="CDR0000062939__35"><li class="half_rhythm"><div>Mediastinal primary </div><div><i>or</i>
|
|
</div></li><li class="half_rhythm"><div>Nonpulmonary visceral metastases </div><div><i>or</i>
|
|
</div></li><li class="half_rhythm"><div>Poor markers - any of:
|
|
<dl id="CDR0000062939__36" class="temp-labeled-list"><dt>-</dt><dd><p class="no_top_margin">AFP greater than 10,000 ng/mL </p><p><i>or</i>
|
|
</p></dd><dt>-</dt><dd><p class="no_top_margin">hCG greater than 50,000 iu/L (1,000 ng/mL) </p><p><i>or</i></p></dd><dt>-</dt><dd><p class="no_top_margin">LDH greater than 10 × upper limit of normal
|
|
</p></dd></dl>
|
|
</div></li></ul><p id="CDR0000062939__37">16% of nonseminomas
|
|
</p><p id="CDR0000062939__38">5-year PFS rate of 41%
|
|
</p><p id="CDR0000062939__51">5-year survival rate of 48%</p></div><div id="CDR0000062939__46"><h4>Seminoma</h4><p id="CDR0000062939__40">No patients are classified as poor prognosis.
|
|
</p></div></div><div id="CDR0000062939_rl_68"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000062939_rl_68_1">Mayordomo JI, Paz-Ares L, Rivera F, et al.: Ovarian and extragonadal malignant germ-cell tumors in females: a single-institution experience with 43 patients. Ann Oncol 5 (3): 225-31, 1994. [<a href="https://pubmed.ncbi.nlm.nih.gov/7514435" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 7514435</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062939_rl_68_2">Greco FA, Vaughn WK, Hainsworth JD: Advanced poorly differentiated carcinoma of unknown primary site: recognition of a treatable syndrome. Ann Intern Med 104 (4): 547-53, 1986. [<a href="https://pubmed.ncbi.nlm.nih.gov/3006571" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 3006571</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062939_rl_68_3">Hainsworth JD, Greco FA: Extragonadal germ cell tumors and unrecognized germ cell tumors. Semin Oncol 19 (2): 119-27, 1992. [<a href="https://pubmed.ncbi.nlm.nih.gov/1313190" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 1313190</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062939_rl_68_4">International Germ Cell Consensus Classification: a prognostic factor-based staging system for metastatic germ cell cancers. International Germ Cell Cancer Collaborative Group. J Clin Oncol 15 (2): 594-603, 1997. [<a href="https://pubmed.ncbi.nlm.nih.gov/9053482" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 9053482</span></a>]</div></li></ol></div></div><div id="CDR0000062939__71"><h2 id="_CDR0000062939__71_">Benign Teratoma</h2><p id="CDR0000062939__5">Benign teratomas are treated with surgical excision only. These tumors are
|
|
frequently very large, and the surgical procedure can be formidable.
|
|
</p><div id="CDR0000062939__TrialSearch_71_sid_3"><h3>Current Clinical Trials</h3><p id="CDR0000062939__TrialSearch_71_22">Use our <a href="https://www.cancer.gov/research/participate/clinical-trials-search/advanced" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">advanced clinical trial search</a> to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. <a href="https://www.cancer.gov/research/participate/clinical-trials" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">General information</a> about clinical trials is also available.</p></div></div><div id="CDR0000062939__72"><h2 id="_CDR0000062939__72_">Seminoma</h2><p id="CDR0000062939__6">The diagnosis of seminoma requires that the serum alpha fetoprotein (AFP) be
|
|
normal, and no other germ cells be present. Management decisions in patients presenting with these tumors can sometimes be difficult. </p><p id="CDR0000062939__85">As in testicular seminoma, these tumors
|
|
are very radiosensitive. About 60% to 80% of patients will remain disease free after
|
|
treatment with radiation therapy.[<a class="bk_pop" href="#CDR0000062939_rl_72_1">1</a>] Craniospinal radiation therapy for intracranial germinomas (the
|
|
intracranial counterpart of seminoma) is associated with relapse-free and
|
|
overall survival rates of 90% to 95% at 5 years, as evidenced in the GER-GPO-MAKEI-86/89 trial, for example.[<a class="bk_pop" href="#CDR0000062939_rl_72_2">2</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000810035/" class="def">Level of evidence C1</a>]
|
|
</p><p id="CDR0000062939__7">Initial chemotherapy with regimens used in nonseminoma testicular cancer is also
|
|
very efficacious. Practically speaking, patients with localized relatively
|
|
small tumors are usually treated initially with radiation, while those with
|
|
very bulky tumors or nonlocalized tumors are treated with etoposide-based and
|
|
cisplatin-based chemotherapy regimens.
|
|
</p><p id="CDR0000062939__8">As in testicular seminoma, many patients will be left with a residual mass posttreatment. If the residual mass is smaller than 3.0 cm,
|
|
the majority of experts agree that observation is appropriate. In those with larger residual
|
|
masses, some experts favor surgical excision while others favor
|
|
observation.[<a class="bk_pop" href="#CDR0000062939_rl_72_3">3</a>,<a class="bk_pop" href="#CDR0000062939_rl_72_4">4</a>]
|
|
</p><div id="CDR0000062939__TrialSearch_72_sid_4"><h3>Current Clinical Trials</h3><p id="CDR0000062939__TrialSearch_72_22">Use our <a href="https://www.cancer.gov/research/participate/clinical-trials-search/advanced" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">advanced clinical trial search</a> to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. <a href="https://www.cancer.gov/research/participate/clinical-trials" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">General information</a> about clinical trials is also available.</p></div><div id="CDR0000062939_rl_72"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000062939_rl_72_1">Clamon GH: Management of primary mediastinal seminoma. Chest 83 (2): 263-7, 1983. [<a href="https://pubmed.ncbi.nlm.nih.gov/6185280" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 6185280</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062939_rl_72_2">Bamberg M, Kortmann RD, Calaminus G, et al.: Radiation therapy for intracranial germinoma: results of the German cooperative prospective trials MAKEI 83/86/89. J Clin Oncol 17 (8): 2585-92, 1999. [<a href="https://pubmed.ncbi.nlm.nih.gov/10561326" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 10561326</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062939_rl_72_3">Motzer R, Bosl G, Heelan R, et al.: Residual mass: an indication for further therapy in patients with advanced seminoma following systemic chemotherapy. J Clin Oncol 5 (7): 1064-70, 1987. [<a href="https://pubmed.ncbi.nlm.nih.gov/3598610" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 3598610</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062939_rl_72_4">Schultz SM, Einhorn LH, Conces DJ, et al.: Management of postchemotherapy residual mass in patients with advanced seminoma: Indiana University experience. J Clin Oncol 7 (10): 1497-503, 1989. [<a href="https://pubmed.ncbi.nlm.nih.gov/2778480" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 2778480</span></a>]</div></li></ol></div></div><div id="CDR0000062939__73"><h2 id="_CDR0000062939__73_">Nonseminoma</h2><p id="CDR0000062939__9">Patients with nonseminomas should receive chemotherapy at diagnosis. These
|
|
patients tend to have a very large tumor volume at diagnosis and are usually
|
|
symptomatic. Initial debulking surgery is rarely useful. Many high-risk
|
|
patients qualify for clinical trials.
|
|
Standard therapy would generally be considered to be four courses of
|
|
BEP (bleomycin, etoposide, and cisplatin).[<a class="bk_pop" href="#CDR0000062939_rl_73_1">1</a>,<a class="bk_pop" href="#CDR0000062939_rl_73_2">2</a>] </p><p id="CDR0000062939__89">A randomized study comparing
|
|
four courses of BEP with four courses of VIP (etoposide, ifosfamide, and cisplatin)
|
|
showed similar overall survival (OS) and time-to-treatment failure for the two
|
|
regimens in patients with advanced disseminated germ cell tumors who had not
|
|
received previous chemotherapy.[<a class="bk_pop" href="#CDR0000062939_rl_73_3">3</a>,<a class="bk_pop" href="#CDR0000062939_rl_73_4">4</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000810017/" class="def">Level of evidence A1</a>] Of the 304 patients on this study, 66 patients had extragonadal primary tumors, and in this subset
|
|
of patients, responses were similar on the two regimens. Hematologic toxic effects in OS were substantially worse with the VIP regimen than with the BEP
|
|
regimen. </p><p id="CDR0000062939__86">Patients with a residual mass after
|
|
chemotherapy may achieve long-term disease-free survival after postchemotherapy surgery with resection of all
|
|
residual disease.[<a class="bk_pop" href="#CDR0000062939_rl_73_5">5</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000810037/" class="def">Level of evidence C2</a>] Patients with nonseminomatous extragonadal germ cell tumors
|
|
who relapse after front-line chemotherapy generally have poor prognoses with
|
|
poor responses to salvage chemotherapy regimens, including autologous bone
|
|
marrow transplantation, that have had success for recurrent testicular
|
|
cancer.[<a class="bk_pop" href="#CDR0000062939_rl_73_6">6</a>-<a class="bk_pop" href="#CDR0000062939_rl_73_8">8</a>] Such patients, therefore, are candidates for studies of new
|
|
approaches.
|
|
</p><div id="CDR0000062939__74"><h3>Mediastinal Nonseminoma</h3><p id="CDR0000062939__10">Mediastinal nonseminomas have certain unique aspects. The tumors are more frequent in
|
|
individuals with Klinefelter syndrome and are associated with a risk of
|
|
subsequent development of hematologic neoplasia that is not treatment related.[<a class="bk_pop" href="#CDR0000062939_rl_73_9">9</a>,<a class="bk_pop" href="#CDR0000062939_rl_73_10">10</a>] Approximately 50% of patients with mediastinal nonseminomas will survive with appropriate
|
|
management.[<a class="bk_pop" href="#CDR0000062939_rl_73_11">11</a>] High risk is partially related to tumor bulk, to chemotherapy
|
|
resistance, and to a predisposition to develop hematologic neoplasia and other
|
|
nongerm cell malignancies.
|
|
In an uncontrolled study, some patients with a postchemotherapy residual mediastinal mass achieved long-term disease-free survival after complete resection, even when serum tumor markers were elevated.[<a class="bk_pop" href="#CDR0000062939_rl_73_5">5</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000810037/" class="def">Level of evidence C2</a>] Patient selection factors may play a role in these favorable outcomes.</p></div><div id="CDR0000062939__75"><h3>Retroperitoneal Nonseminoma</h3><p id="CDR0000062939__11">The prognosis of retroperitoneal nonseminoma is reasonably good and, similar to
|
|
the situation with nodal metastasis from a testicular primary, is related to
|
|
tumor volume.
|
|
</p></div><div id="CDR0000062939__TrialSearch_73_sid_5"><h3>Current Clinical Trials</h3><p id="CDR0000062939__TrialSearch_73_22">Use our <a href="https://www.cancer.gov/research/participate/clinical-trials-search/advanced" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">advanced clinical trial search</a> to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. <a href="https://www.cancer.gov/research/participate/clinical-trials" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">General information</a> about clinical trials is also available.</p></div><div id="CDR0000062939_rl_73"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000062939_rl_73_1">Williams SD, Birch R, Einhorn LH, et al.: Treatment of disseminated germ-cell tumors with cisplatin, bleomycin, and either vinblastine or etoposide. N Engl J Med 316 (23): 1435-40, 1987. [<a href="https://pubmed.ncbi.nlm.nih.gov/2437455" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 2437455</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062939_rl_73_2">Bosl GJ, Gluckman R, Geller NL, et al.: VAB-6: an effective chemotherapy regimen for patients with germ-cell tumors. J Clin Oncol 4 (10): 1493-9, 1986. [<a href="https://pubmed.ncbi.nlm.nih.gov/2428948" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 2428948</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062939_rl_73_3">Nichols CR, Catalano PJ, Crawford ED, et al.: Randomized comparison of cisplatin and etoposide and either bleomycin or ifosfamide in treatment of advanced disseminated germ cell tumors: an Eastern Cooperative Oncology Group, Southwest Oncology Group, and Cancer and Leukemia Group B Study. J Clin Oncol 16 (4): 1287-93, 1998. [<a href="https://pubmed.ncbi.nlm.nih.gov/9552027" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 9552027</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062939_rl_73_4">Hinton S, Catalano PJ, Einhorn LH, et al.: Cisplatin, etoposide and either bleomycin or ifosfamide in the treatment of disseminated germ cell tumors: final analysis of an intergroup trial. Cancer 97 (8): 1869-75, 2003. [<a href="https://pubmed.ncbi.nlm.nih.gov/12673712" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 12673712</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062939_rl_73_5">Schneider BP, Kesler KA, Brooks JA, et al.: Outcome of patients with residual germ cell or non-germ cell malignancy after resection of primary mediastinal nonseminomatous germ cell cancer. J Clin Oncol 22 (7): 1195-200, 2004. [<a href="https://pubmed.ncbi.nlm.nih.gov/15051766" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15051766</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062939_rl_73_6">Saxman SB, Nichols CR, Einhorn LH: Salvage chemotherapy in patients with extragonadal nonseminomatous germ cell tumors: the Indiana University experience. J Clin Oncol 12 (7): 1390-3, 1994. [<a href="https://pubmed.ncbi.nlm.nih.gov/8021729" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 8021729</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062939_rl_73_7">Beyer J, Kramar A, Mandanas R, et al.: High-dose chemotherapy as salvage treatment in germ cell tumors: a multivariate analysis of prognostic variables. J Clin Oncol 14 (10): 2638-45, 1996. [<a href="https://pubmed.ncbi.nlm.nih.gov/8874322" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 8874322</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062939_rl_73_8">Loehrer PJ, Gonin R, Nichols CR, et al.: Vinblastine plus ifosfamide plus cisplatin as initial salvage therapy in recurrent germ cell tumor. J Clin Oncol 16 (7): 2500-4, 1998. [<a href="https://pubmed.ncbi.nlm.nih.gov/9667270" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 9667270</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062939_rl_73_9">Nichols CR, Heerema NA, Palmer C, et al.: Klinefelter's syndrome associated with mediastinal germ cell neoplasms. J Clin Oncol 5 (8): 1290-4, 1987. [<a href="https://pubmed.ncbi.nlm.nih.gov/3040921" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 3040921</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062939_rl_73_10">Nichols CR, Roth BJ, Heerema N, et al.: Hematologic neoplasia associated with primary mediastinal germ-cell tumors. N Engl J Med 322 (20): 1425-9, 1990. [<a href="https://pubmed.ncbi.nlm.nih.gov/2158625" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 2158625</span></a>]</div></li><li><div class="bk_ref" id="CDR0000062939_rl_73_11">Nichols CR, Saxman S, Williams SD, et al.: Primary mediastinal nonseminomatous germ cell tumors. A modern single institution experience. Cancer 65 (7): 1641-6, 1990. [<a href="https://pubmed.ncbi.nlm.nih.gov/1690077" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 1690077</span></a>]</div></li></ol></div></div><div id="CDR0000062939__95"><h2 id="_CDR0000062939__95_">Recurrent or Refractory Extragonadal Germ Cell Tumors</h2><p id="CDR0000062939__96">A randomized, controlled trial compared conventional doses of salvage chemotherapy to high-dose chemotherapy with autologous marrow rescue in 263 patients with recurrent or refractory germ cell tumors. Of the 263 patients, 43 of whom had extragonadal primary tumors, more toxic effects and treatment-related deaths were seen in the high-dose arm without any improvement in response rate or overall survival.[<a class="bk_pop" href="#CDR0000062939_rl_95_1">1</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000810017/" class="def">Level of evidence A1</a>]</p><div id="CDR0000062939__TrialSearch_95_sid_6"><h3>Current Clinical Trials</h3><p id="CDR0000062939__TrialSearch_95_22">Use our <a href="https://www.cancer.gov/research/participate/clinical-trials-search/advanced" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">advanced clinical trial search</a> to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. <a href="https://www.cancer.gov/research/participate/clinical-trials" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">General information</a> about clinical trials is also available.</p></div><div id="CDR0000062939_rl_95"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000062939_rl_95_1">Pico JL, Rosti G, Kramar A, et al.: A randomised trial of high-dose chemotherapy in the salvage treatment of patients failing first-line platinum chemotherapy for advanced germ cell tumours. Ann Oncol 16 (7): 1152-9, 2005. [<a href="https://pubmed.ncbi.nlm.nih.gov/15928070" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15928070</span></a>]</div></li></ol></div></div><div id="CDR0000062939__81"><h2 id="_CDR0000062939__81_">Latest Updates to This Summary (02/25/2015)</h2><p id="CDR0000062939__82">The PDQ cancer information summaries are reviewed regularly and updated as
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new information becomes available. This section describes the latest
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changes made to this summary as of the date above.</p><p id="CDR0000062939__117">
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<b>
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<a href="#CDR0000062939__1">General Information About Extragonadal Germ Cell Tumors</a>
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</b>
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</p><p id="CDR0000062939__110">Editorial changes were made to this section.</p><p id="CDR0000062939__disclaimerHP_3">This summary is written and maintained by the <a href="https://www.cancer.gov/publications/pdq/editorial-boards/adult-treatment" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PDQ Adult Treatment Editorial Board</a>, which is
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editorially independent of NCI. The summary reflects an independent review of
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the literature and does not represent a policy statement of NCI or NIH. More
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information about summary policies and the role of the PDQ Editorial Boards in
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maintaining the PDQ summaries can be found on the <a href="#CDR0000062939__AboutThis_1">About This PDQ Summary</a> and <a href="https://www.cancer.gov/publications/pdq" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PDQ® Cancer Information for Health Professionals</a> pages.
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</p></div><div id="CDR0000062939__AboutThis_1"><h2 id="_CDR0000062939__AboutThis_1_">About This PDQ Summary</h2><div id="CDR0000062939__AboutThis_2"><h3>Purpose of This Summary</h3><p id="CDR0000062939__AboutThis_3">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of extragonadal germ cell tumors. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.</p></div><div id="CDR0000062939__AboutThis_4"><h3>Reviewers and Updates</h3><p id="CDR0000062939__AboutThis_5">This summary is reviewed regularly and updated as necessary by the <a href="https://www.cancer.gov/publications/pdq/editorial-boards/adult-treatment" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PDQ Adult Treatment Editorial Board</a>, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).</p><p id="CDR0000062939__AboutThis_22"> Board members review recently published articles each month to determine whether an article should:</p><ul id="CDR0000062939__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="CDR0000062939__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 reviewer for Extragonadal Germ Cell Tumors Treatment is:</p><ul><li class="half_rhythm"><div>Timothy Gilligan, MD (Cleveland Clinic Taussig Cancer Institute)</div></li></ul><p id="CDR0000062939__AboutThis_9">Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's <a href="https://www.cancer.gov/contact/email-us" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Email Us</a>. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.</p></div><div id="CDR0000062939__AboutThis_10"><h3>Levels of Evidence</h3><p id="CDR0000062939__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="CDR0000062939__AboutThis_12"><h3>Permission to Use This Summary</h3><p id="CDR0000062939__AboutThis_13">PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].”</p><p id="CDR0000062939__AboutThis_14">The preferred citation for this PDQ summary is:</p><p id="CDR0000062939__AboutThis_15">PDQ® Adult Treatment Editorial Board. PDQ Extragonadal Germ Cell Tumors Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: <a href="https://www.cancer.gov/types/extragonadal-germ-cell/hp/extragonadal-treatment-pdq" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">https://www.cancer.gov/types/extragonadal-germ-cell/hp/extragonadal-treatment-pdq</a>. Accessed <MM/DD/YYYY>. [PMID: 26389346]</p><p id="CDR0000062939__AboutThis_16">Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in <a href="https://visualsonline.cancer.gov/" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Visuals Online</a>, a collection of over 2,000 scientific images.
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</p></div><div id="CDR0000062939__AboutThis_17"><h3>Disclaimer</h3><p id="CDR0000062939__AboutThis_18">Based on the strength of the available evidence, treatment options may be described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the <a href="https://www.cancer.gov/about-cancer/managing-care" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Managing Cancer Care</a> page.</p></div><div id="CDR0000062939__AboutThis_20"><h3>Contact Us</h3><p id="CDR0000062939__AboutThis_21">More information about contacting us or receiving help with the Cancer.gov website can be found on our <a href="https://www.cancer.gov/contact" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Contact Us for Help</a> page. Questions can also be submitted to Cancer.gov through the website’s <a href="https://www.cancer.gov/contact/email-us" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Email Us</a>.</p></div></div></div></div>
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<div xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance"></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Views</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="PDF_download" id="Shutter"></a></div><div class="portlet_content"><ul xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="simple-list"><li><a href="/books/NBK65908.1/?report=reader">PubReader</a></li><li><a href="/books/NBK65908.1/?report=printable">Print View</a></li><li><a data-jig="ncbidialog" href="#_ncbi_dlg_citbx_NBK65908" data-jigconfig="width:400,modal:true">Cite this Page</a><div id="_ncbi_dlg_citbx_NBK65908" style="display:none" title="Cite this Page"><div class="bk_tt">PDQ Adult Treatment Editorial Board. Extragonadal Germ Cell Tumors Treatment (PDQ®): Health Professional Version. 2015 Feb 25. In: PDQ Cancer Information Summaries [Internet]. Bethesda (MD): National Cancer Institute (US); 2002-. <span class="bk_cite_avail"></span></div></div></li><li><a href="#" class="toggle-glossary-link" title="Enable/disable links to the glossary">Disable Glossary Links</a></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Version History</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter shutter_closed" title="Show/hide content" remembercollapsed="true" pgsec_name="version_history" id="Shutter"></a></div><div class="portlet_content" style="display: none;"><ul xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="simple-list"><li><span class="bk_col_itm"><a href="/books/NBK65908.2/">NBK65908.2</a></span> December 9, 2024</li><li><span class="bk_col_itm">NBK65908.1</span> February 25, 2015 (Displayed Version)</li></ul></div></div><div 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="#CDR0000062939__1" ref="log$=inpage&link_id=inpage">General Information About Extragonadal Germ Cell Tumors</a></li><li><a href="#CDR0000062939__68" ref="log$=inpage&link_id=inpage">Cellular Classification of Extragonadal Germ Cell Tumors</a></li><li><a href="#CDR0000062939__71" ref="log$=inpage&link_id=inpage">Benign Teratoma</a></li><li><a href="#CDR0000062939__72" ref="log$=inpage&link_id=inpage">Seminoma</a></li><li><a href="#CDR0000062939__73" ref="log$=inpage&link_id=inpage">Nonseminoma</a></li><li><a href="#CDR0000062939__95" ref="log$=inpage&link_id=inpage">Recurrent or Refractory Extragonadal Germ Cell Tumors</a></li><li><a href="#CDR0000062939__81" ref="log$=inpage&link_id=inpage">Latest Updates to This Summary (02/25/2015)</a></li><li><a href="#CDR0000062939__AboutThis_1" ref="log$=inpage&link_id=inpage">About This PDQ Summary</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/NBK65770/">Patient Version</a></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Related information</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" 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class="invert">Review</span> Gastrointestinal Stromal Tumors Treatment (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div class="brieflinkpop offscreen_noflow"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Gastrointestinal Stromal Tumors Treatment (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="author">PDQ Adult Treatment Editorial Board. </em><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="cit">PDQ Cancer Information Summaries. 2002</em></div></div></li><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/26389312" ref="ordinalpos=1&linkpos=2&log$=relatedreviews&logdbfrom=pubmed"><span 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class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/26389199" ref="ordinalpos=1&linkpos=4&log$=relatedreviews&logdbfrom=pubmed"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Hypopharyngeal Cancer Treatment (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div class="brieflinkpop offscreen_noflow"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Hypopharyngeal Cancer Treatment (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="author">PDQ Adult Treatment Editorial Board. </em><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="cit">PDQ Cancer Information Summaries. 2002</em></div></div></li><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/26389393" ref="ordinalpos=1&linkpos=5&log$=relatedreviews&logdbfrom=pubmed"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Adrenocortical Carcinoma Treatment (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div class="brieflinkpop offscreen_noflow"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Adrenocortical Carcinoma Treatment (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="author">PDQ Adult Treatment Editorial Board. </em><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" 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