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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="#__NBK550986_dtls__">Show details</a><div style="display:none" class="ui-widget" id="__NBK550986_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="_NBK550986_"><span class="title" itemprop="name">Childhood Testicular Cancer Treatment (PDQ®)</span></h1><div class="subtitle whole_rhythm">Health Professional Version</div><p class="contrib-group"><span itemprop="author">PDQ Pediatric Treatment Editorial Board</span>.</p><p class="small">Published online: December 17, 2021.</p><p class="small">Created: <span itemprop="datePublished">December 12, 2019</span>.</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="CDR0000799768__1556">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of pediatric testicular cancer. 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="CDR0000799768__1557">This summary is reviewed regularly and updated as necessary by the PDQ Pediatric 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="CDR0000799768__2217"><h2 id="_CDR0000799768__2217_">Incidence and Clinical Presentation</h2><p id="CDR0000799768__2218">Testicular tumors are very rare in young boys and account for an incidence of 1% to 2% of all childhood tumors.[<a class="bk_pop" href="#CDR0000799768_rl_2217_1">1</a>,<a class="bk_pop" href="#CDR0000799768_rl_2217_2">2</a>] The most common testicular tumors are benign teratomas followed by malignant nonseminomatous germ cell tumors. (Refer to the PDQ summary on <a href="/books/n/pdqcis/CDR0000062854/">Childhood Extracranial Germ Cell Tumors Treatment</a> for more information.)</p><p id="CDR0000799768__2219">Non–germ cell tumors such as sex cord–stromal tumors are exceedingly rare in prepubertal boys. In a small series, gonadal stromal tumors accounted for 8% to 13% of pediatric testicular tumors.[<a class="bk_pop" href="#CDR0000799768_rl_2217_3">3</a>,<a class="bk_pop" href="#CDR0000799768_rl_2217_4">4</a>] Most gonadal stromal tumors present as a painless testicular mass, while 10% to 20% of patients may have endocrine manifestations such as precocious puberty.[<a class="bk_pop" href="#CDR0000799768_rl_2217_5">5</a>] In newborns and infants, juvenile granulosa cell and Sertoli cell tumors are the most common stromal cell tumor. Juvenile granulosa cell tumors usually present early in infancy (median age, 6 days) and Sertoli cell tumors present later in infancy (median age, 7 months). In older males, Leydig cell tumors are more common.[<a class="bk_pop" href="#CDR0000799768_rl_2217_6">6</a>] In a report of 12 patients with Leydig cell tumors (aged 4.2–14.7 years), precocious puberty was the presenting symptom in 7 of 12 patients.[<a class="bk_pop" href="#CDR0000799768_rl_2217_7">7</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335144/" class="def">Level of evidence: 3iiA</a>] Testicular Sertoli cell tumors and, possibly, Leydig cell tumors are associated with the <i>DICER1</i> syndrome. Patients with these tumors should undergo genetic testing for <i>DICER1</i> germline mutations.[<a class="bk_pop" href="#CDR0000799768_rl_2217_8">8</a>] Large cell calcifying Sertoli cell tumors may indicate an underlying genetic predisposition, such as Peutz-Jeghers syndrome or Carney complex.[<a class="bk_pop" href="#CDR0000799768_rl_2217_9">9</a>] These tumors are usually benign, may occur in both testes, and some patients may have a slow and indolent course.[<a class="bk_pop" href="#CDR0000799768_rl_2217_10">10</a>]</p><p id="CDR0000799768__2391">The European Cooperative Study Group for Pediatric Rare Tumors within the PARTNER project (Paediatric Rare Tumours Network - European Registry) has published comprehensive recommendations for the diagnosis and treatment of sex cord–stromal tumors in children and adolescents.[<a class="bk_pop" href="#CDR0000799768_rl_2217_11">11</a>]</p><div id="CDR0000799768_rl_2217"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799768_rl_2217_1">Hartke DM, Agarwal PK, Palmer JS: Testicular neoplasms in the prepubertal male. J Mens Health Gend 3 (2): 131-8, 2006.</div></li><li><div class="bk_ref" id="CDR0000799768_rl_2217_2">Ahmed HU, Arya M, Muneer A, et al.: Testicular and paratesticular tumours in the prepubertal population. Lancet Oncol 11 (5): 476-83, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/20434716" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20434716</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_2217_3">Pohl HG, Shukla AR, Metcalf PD, et al.: Prepubertal testis tumors: actual prevalence rate of histological types. J Urol 172 (6 Pt 1): 2370-2, 2004. [<a href="https://pubmed.ncbi.nlm.nih.gov/15538270" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15538270</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_2217_4">Schwentner C, Oswald J, Rogatsch H, et al.: Stromal testis tumors in infants. a report of two cases. Urology 62 (6): 1121, 2003. [<a href="https://pubmed.ncbi.nlm.nih.gov/14665372" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 14665372</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_2217_5">Cecchetto G, Alaggio R, Bisogno G, et al.: Sex cord-stromal tumors of the testis in children. A clinicopathologic report from the Italian TREP project. J Pediatr Surg 45 (9): 1868-73, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/20850634" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20850634</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_2217_6">Carmignani L, Colombo R, Gadda F, et al.: Conservative surgical therapy for leydig cell tumor. J Urol 178 (2): 507-11; discussion 511, 2007. [<a href="https://pubmed.ncbi.nlm.nih.gov/17561156" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 17561156</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_2217_7">Luckie TM, Danzig M, Zhou S, et al.: A Multicenter Retrospective Review of Pediatric Leydig Cell Tumor of the Testis. J Pediatr Hematol Oncol 41 (1): 74-76, 2019. [<a href="https://pubmed.ncbi.nlm.nih.gov/29554024" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 29554024</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_2217_8">Golmard L, Vasta LM, Duflos V, et al.: Testicular Sertoli cell tumour and potentially testicular Leydig cell tumour are features of DICER1 syndrome. J Med Genet : , 2021. [<a href="/pmc/articles/PMC9743800/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC9743800</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/33782093" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 33782093</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_2217_9">Gourgari E, Saloustros E, Stratakis CA: Large-cell calcifying Sertoli cell tumors of the testes in pediatrics. Curr Opin Pediatr 24 (4): 518-22, 2012. [<a href="/pmc/articles/PMC4132931/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC4132931</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/22732638" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22732638</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_2217_10">Lai JP, Lee CC, Crocker M, et al.: Isolated Large Cell Calcifying Sertoli Cell Tumor in a Young Boy, not Associated with Peutz-Jeghers Syndrome or Carney Complex. Ann Clin Lab Res 3 (1): 2, 2015. [<a href="/pmc/articles/PMC4648613/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC4648613</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/26587565" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 26587565</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_2217_11">Schneider DT, Orbach D, Ben-Ami T, et al.: Consensus recommendations from the EXPeRT/PARTNER groups for the diagnosis and therapy of sex cord stromal tumors in children and adolescents. Pediatr Blood Cancer 68 (Suppl 4): e29017, 2021. [<a href="https://pubmed.ncbi.nlm.nih.gov/33760357" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 33760357</span></a>]</div></li></ol></div></div><div id="CDR0000799768__2220"><h2 id="_CDR0000799768__2220_">Prognosis</h2><p id="CDR0000799768__2221">The prognosis for sex cord–stromal tumors is usually excellent after orchiectomy.[<a class="bk_pop" href="#CDR0000799768_rl_2220_1">1</a>-<a class="bk_pop" href="#CDR0000799768_rl_2220_3">3</a>]; [<a class="bk_pop" href="#CDR0000799768_rl_2220_4">4</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335150/" class="def">Level of evidence: 3iiiA</a>] In a review of the literature, 79 patients younger than 12 years were identified. No patient had high-risk pathological findings after orchiectomy, and none had evidence of occult metastatic disease, suggesting a role for a limited surveillance strategy.[<a class="bk_pop" href="#CDR0000799768_rl_2220_5">5</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335150/" class="def">Level of evidence: 3iiiA</a>]</p><div id="CDR0000799768_rl_2220"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799768_rl_2220_1">Agarwal PK, Palmer JS: Testicular and paratesticular neoplasms in prepubertal males. J Urol 176 (3): 875-81, 2006. [<a href="https://pubmed.ncbi.nlm.nih.gov/16890643" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 16890643</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_2220_2">Dudani R, Giordano L, Sultania P, et al.: Juvenile granulosa cell tumor of testis: case report and review of literature. Am J Perinatol 25 (4): 229-31, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18548396" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18548396</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_2220_3">Cecchetto G, Alaggio R, Bisogno G, et al.: Sex cord-stromal tumors of the testis in children. A clinicopathologic report from the Italian TREP project. J Pediatr Surg 45 (9): 1868-73, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/20850634" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20850634</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_2220_4">Hofmann M, Schlegel PG, Hippert F, et al.: Testicular sex cord stromal tumors: analysis of patients from the MAKEI study. Pediatr Blood Cancer 60 (10): 1651-5, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/23733594" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 23733594</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_2220_5">Rove KO, Maroni PD, Cost CR, et al.: Pathologic Risk Factors in Pediatric and Adolescent Patients With Clinical Stage I Testicular Stromal Tumors. J Pediatr Hematol Oncol 37 (8): e441-6, 2015. [<a href="https://pubmed.ncbi.nlm.nih.gov/26479987" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 26479987</span></a>]</div></li></ol></div></div><div id="CDR0000799768__1864"><h2 id="_CDR0000799768__1864_">Special Considerations for the Treatment of Children With Cancer</h2><p id="CDR0000799768__1865">Cancer in children and adolescents is rare, although the overall incidence has been slowly increasing since 1975.[<a class="bk_pop" href="#CDR0000799768_rl_1864_1">1</a>] Referral to medical centers with multidisciplinary teams of cancer specialists experienced in treating cancers that occur in childhood and adolescence should be considered. This multidisciplinary team approach incorporates the skills
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of the following health care professionals and others to ensure that children receive treatment, supportive care, and rehabilitation
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that will achieve optimal survival and quality of life:</p><ul id="CDR0000799768__1866"><li class="half_rhythm"><div>Primary care physicians.</div></li><li class="half_rhythm"><div>Pediatric surgeons.</div></li><li class="half_rhythm"><div>Radiation
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oncologists.</div></li><li class="half_rhythm"><div>Pediatric medical oncologists/hematologists.</div></li><li class="half_rhythm"><div> Rehabilitation
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specialists.</div></li><li class="half_rhythm"><div>Pediatric nurse specialists.</div></li><li class="half_rhythm"><div>Social workers.</div></li><li class="half_rhythm"><div>Child-life professionals.</div></li><li class="half_rhythm"><div>Psychologists.</div></li></ul><p id="CDR0000799768__1867"> (Refer to the PDQ <a href="https://www.cancer.gov/publications/pdq/information-summaries/supportive-care" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Supportive and Palliative Care</a> summaries for specific information about supportive care for children and adolescents with cancer.)</p><p id="CDR0000799768__1868">The American Academy of Pediatrics has outlined guidelines for
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pediatric cancer centers and their role in the treatment of pediatric patients
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with cancer.[<a class="bk_pop" href="#CDR0000799768_rl_1864_2">2</a>] At
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these pediatric cancer centers, clinical trials are available for most types of cancer that occur in children and adolescents, and the opportunity
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to participate is offered to most patients and their families. Clinical
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trials for children and adolescents diagnosed with cancer are generally
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designed to compare potentially better therapy with current standard therapy. Most of the progress made in identifying curative
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therapy for childhood cancers has been achieved through clinical trials.
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Information about ongoing clinical trials is available from the <a href="https://www.cancer.gov/about-cancer/treatment/clinical-trials" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">NCI website</a>.</p><p id="CDR0000799768__1869">Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2010, childhood cancer mortality decreased by more than 50%.[<a class="bk_pop" href="#CDR0000799768_rl_1864_3">3</a>] Childhood and adolescent cancer survivors require close monitoring because side effects of cancer therapy may persist or develop months or years after treatment. (Refer to the PDQ summary on <a href="/books/n/pdqcis/CDR0000343584/">Late Effects of Treatment for Childhood Cancer</a> for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.)</p><p id="CDR0000799768__1870">Childhood cancer is a rare disease, with about 15,000 cases diagnosed annually in the United States in individuals younger than 20 years.[<a class="bk_pop" href="#CDR0000799768_rl_1864_4">4</a>] The U.S. <a href="https://www.congress.gov/107/plaws/publ280/PLAW-107publ280.pdf" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Rare Diseases Act of 2002</a> defines a rare disease as one that affects populations smaller than 200,000 people. Therefore, all pediatric cancers are considered rare.</p><p id="CDR0000799768__1888">The designation of a rare tumor is not uniform among pediatric and adult groups. In adults, rare cancers are defined as those with an annual incidence of fewer than six cases per 100,000 people. They account for up to 24% of all cancers diagnosed in the European Union and about 20% of all cancers diagnosed in the United States.[<a class="bk_pop" href="#CDR0000799768_rl_1864_5">5</a>,<a class="bk_pop" href="#CDR0000799768_rl_1864_6">6</a>] Also, the designation of a pediatric rare tumor is not uniform among international groups, as follows:</p><ul id="CDR0000799768__1871"><li class="half_rhythm"><div class="half_rhythm">A consensus effort between the European Union Joint Action on Rare Cancers and the European Cooperative Study Group for Rare Pediatric Cancers estimated that 11% of all cancers in patients younger than 20 years could be categorized as very rare. This consensus group defined very rare cancers as those with annual incidences of fewer than 2 cases per 1 million people. However, three additional histologies (thyroid carcinoma, melanoma, and testicular cancer) with incidences of more than 2 cases per 1 million people were also included in the very rare group because there is a lack of knowledge and expertise in the management of these tumors.[<a class="bk_pop" href="#CDR0000799768_rl_1864_7">7</a>]</div></li><li class="half_rhythm"><div class="half_rhythm">The Children's Oncology Group defines rare pediatric cancers as those listed in the International Classification of Childhood Cancer subgroup XI, which includes thyroid cancer, melanoma and nonmelanoma skin cancers, and multiple types of carcinomas (e.g., adrenocortical carcinoma, nasopharyngeal carcinoma, and most adult-type carcinomas such as breast cancer, colorectal cancer, etc.).[<a class="bk_pop" href="#CDR0000799768_rl_1864_8">8</a>] These diagnoses account for about 4% of cancers diagnosed in children aged 0 to 14 years, compared with about 20% of cancers diagnosed in adolescents aged 15 to 19 years.[<a class="bk_pop" href="#CDR0000799768_rl_1864_9">9</a>]</div><div class="half_rhythm">Most cancers in subgroup XI are either melanomas or thyroid cancer, with other types accounting for only 1.3% of cancers in children aged 0 to 14 years and 5.3% of cancers in adolescents aged 15 to 19 years.</div></li></ul><p id="CDR0000799768__1872">These rare cancers are extremely challenging to study because of the low number of patients with any individual diagnosis, the predominance of rare cancers in the adolescent population, and the lack of clinical trials for adolescents with rare cancers.</p><p id="CDR0000799768__1837">Information about these tumors may also be found in sources relevant to
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adults with cancer, such as the PDQ summary on adult <a href="/books/n/pdqcis/CDR0000062899/">Testicular Cancer Treatment</a>.</p><div id="CDR0000799768_rl_1864"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799768_rl_1864_1">Smith MA, Seibel NL, Altekruse SF, et al.: Outcomes for children and adolescents with cancer: challenges for the twenty-first century. J Clin Oncol 28 (15): 2625-34, 2010. [<a href="/pmc/articles/PMC2881732/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC2881732</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/20404250" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20404250</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_1864_2">American Academy of Pediatrics: Standards for pediatric cancer centers. Pediatrics 134 (2): 410-4, 2014. <a href="https://pediatrics.aappublications.org/content/134/2/410" ref="pagearea=cite-ref&targetsite=external&targetcat=link&targettype=uri">Also available online</a>. Last accessed February 1, 2022.</div></li><li><div class="bk_ref" id="CDR0000799768_rl_1864_3">Smith MA, Altekruse SF, Adamson PC, et al.: Declining childhood and adolescent cancer mortality. Cancer 120 (16): 2497-506, 2014. [<a href="/pmc/articles/PMC4136455/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC4136455</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/24853691" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24853691</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_1864_4">Ward E, DeSantis C, Robbins A, et al.: Childhood and adolescent cancer statistics, 2014. CA Cancer J Clin 64 (2): 83-103, 2014 Mar-Apr. [<a href="https://pubmed.ncbi.nlm.nih.gov/24488779" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24488779</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_1864_5">Gatta G, Capocaccia R, Botta L, et al.: Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet-a population-based study. Lancet Oncol 18 (8): 1022-1039, 2017. [<a href="https://pubmed.ncbi.nlm.nih.gov/28687376" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28687376</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_1864_6">DeSantis CE, Kramer JL, Jemal A: The burden of rare cancers in the United States. CA Cancer J Clin 67 (4): 261-272, 2017. [<a href="https://pubmed.ncbi.nlm.nih.gov/28542893" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28542893</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_1864_7">Ferrari A, Brecht IB, Gatta G, et al.: Defining and listing very rare cancers of paediatric age: consensus of the Joint Action on Rare Cancers in cooperation with the European Cooperative Study Group for Pediatric Rare Tumors. Eur J Cancer 110: 120-126, 2019. [<a href="https://pubmed.ncbi.nlm.nih.gov/30785015" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 30785015</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_1864_8">Pappo AS, Krailo M, Chen Z, et al.: Infrequent tumor initiative of the Children's Oncology Group: initial lessons learned and their impact on future plans. J Clin Oncol 28 (33): 5011-6, 2010. [<a href="/pmc/articles/PMC3020699/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3020699</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/20956621" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20956621</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_1864_9">Howlader N, Noone AM, Krapcho M, et al., eds.: SEER Cancer Statistics Review, 1975-2012. National Cancer Institute, 2015. <a href="http://seer.cancer.gov/csr/1975_2012/" ref="pagearea=cite-ref&targetsite=external&targetcat=link&targettype=uri">Also available online</a>. Last accessed June 22, 2021.</div></li></ol></div></div><div id="CDR0000799768__978"><h2 id="_CDR0000799768__978_">Treatment of Childhood Testicular Cancer</h2><p id="CDR0000799768__2222">Treatment options for childhood testicular cancer (non-germ cell) include the following:</p><ol id="CDR0000799768__2223"><li class="half_rhythm"><div>Surgery.</div></li></ol><p id="CDR0000799768__979">There are conflicting data about malignant potential in older males. Most case reports suggest that in pediatric patients, these tumors can be treated with surgery alone.[<a class="bk_pop" href="#CDR0000799768_rl_978_1">1</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000561227/" class="def">Level of evidence: 3iii</a>]; [<a class="bk_pop" href="#CDR0000799768_rl_978_2">2</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335150/" class="def">Level of evidence: 3iiiA</a>]; [<a class="bk_pop" href="#CDR0000799768_rl_978_3">3</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335155/" class="def">Level of evidence: 3iiiDii</a>] It is prudent to check alpha-fetoprotein (AFP) levels before surgery. Elevated AFP levels are usually indicative of a malignant germ cell tumor. However, AFP levels and decay in levels are often difficult to interpret in infants younger than 1 year.[<a class="bk_pop" href="#CDR0000799768_rl_978_4">4</a>]</p><p id="CDR0000799768__2224">Evidence (surgery):</p><ol id="CDR0000799768__2225"><li class="half_rhythm"><div>In a study of patients prospectively reported to the German Maligne Keimzelltumoren (MAKEI) registry, 42 patients with sex cord–stromal tumors were identified. All tumors were confined to the testes. Patients were treated with surgery alone, according to specific germ cell tumor guidelines.[<a class="bk_pop" href="#CDR0000799768_rl_978_5">5</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335150/" class="def">Level of evidence: 3iiiA</a>]<ul id="CDR0000799768__2226"><li class="half_rhythm"><div>There were no recurrences.</div></li></ul></div></li><li class="half_rhythm"><div> A French registry identified 11 boys with localized sex cord–stromal testicular tumors. All 11 boys were treated with surgery alone.[<a class="bk_pop" href="#CDR0000799768_rl_978_6">6</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335138/" class="def">Level of evidence: 3iA</a>]<ul id="CDR0000799768__2227"><li class="half_rhythm"><div>There were no recurrences.</div></li></ul></div></li><li class="half_rhythm"><div>The benign behavior of pediatric non–germ cell testicular tumors has led to reports of testis-sparing surgery.[<a class="bk_pop" href="#CDR0000799768_rl_978_7">7</a>-<a class="bk_pop" href="#CDR0000799768_rl_978_11">11</a>] In one series of patients with Leydig cell tumors (aged 4.2–14.7 years), 3 of 12 patients were treated with enucleation alone, and 9 patients were treated with orchiectomy. All patients were alive at the last follow-up.[<a class="bk_pop" href="#CDR0000799768_rl_978_12">12</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335144/" class="def">Level of evidence: 3iiA</a>]</div></li></ol><p id="CDR0000799768__1434"> However, given the rarity of this tumor, the surgical approach in pediatrics has not been well defined.</p><div id="CDR0000799768_rl_978"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799768_rl_978_1">Agarwal PK, Palmer JS: Testicular and paratesticular neoplasms in prepubertal males. J Urol 176 (3): 875-81, 2006. [<a href="https://pubmed.ncbi.nlm.nih.gov/16890643" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 16890643</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_978_2">Thomas JC, Ross JH, Kay R: Stromal testis tumors in children: a report from the prepubertal testis tumor registry. J Urol 166 (6): 2338-40, 2001. [<a href="https://pubmed.ncbi.nlm.nih.gov/11696779" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 11696779</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_978_3">Cecchetto G, Alaggio R, Bisogno G, et al.: Sex cord-stromal tumors of the testis in children. A clinicopathologic report from the Italian TREP project. J Pediatr Surg 45 (9): 1868-73, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/20850634" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20850634</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_978_4">Blohm ME, Vesterling-Hörner D, Calaminus G, et al.: Alpha 1-fetoprotein (AFP) reference values in infants up to 2 years of age. Pediatr Hematol Oncol 15 (2): 135-42, 1998 Mar-Apr. [<a href="https://pubmed.ncbi.nlm.nih.gov/9592840" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 9592840</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_978_5">Hofmann M, Schlegel PG, Hippert F, et al.: Testicular sex cord stromal tumors: analysis of patients from the MAKEI study. Pediatr Blood Cancer 60 (10): 1651-5, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/23733594" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 23733594</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_978_6">Fresneau B, Orbach D, Faure-Conter C, et al.: Sex-Cord Stromal Tumors in Children and Teenagers: Results of the TGM-95 Study. Pediatr Blood Cancer 62 (12): 2114-9, 2015. [<a href="https://pubmed.ncbi.nlm.nih.gov/26206391" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 26206391</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_978_7">Cosentino M, Algaba F, Saldaña L, et al.: Juvenile granulosa cell tumor of the testis: a bilateral and synchronous case. Should testis-sparing surgery be mandatory? Urology 84 (3): 694-6, 2014. [<a href="https://pubmed.ncbi.nlm.nih.gov/25053523" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 25053523</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_978_8">Kao CS, Cornejo KM, Ulbright TM, et al.: Juvenile granulosa cell tumors of the testis: a clinicopathologic study of 70 cases with emphasis on its wide morphologic spectrum. Am J Surg Pathol 39 (9): 1159-69, 2015. [<a href="https://pubmed.ncbi.nlm.nih.gov/26076062" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 26076062</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_978_9">Emre S, Ozcan R, Elicevik M, et al.: Testis sparing surgery for Leydig cell pathologies in children. J Pediatr Urol 13 (1): 51.e1-51.e4, 2017. [<a href="https://pubmed.ncbi.nlm.nih.gov/27773621" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 27773621</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_978_10">Bois JI, Vagni RL, de Badiola FI, et al.: Testis-sparing surgery for testicular tumors in children: a 20 year single center experience and systematic review of the literature. Pediatr Surg Int 37 (5): 607-616, 2021. [<a href="https://pubmed.ncbi.nlm.nih.gov/33454815" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 33454815</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_978_11">Woo LL, Ross JH: The role of testis-sparing surgery in children and adolescents with testicular tumors. Urol Oncol 34 (2): 76-83, 2016. [<a href="https://pubmed.ncbi.nlm.nih.gov/26094168" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 26094168</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799768_rl_978_12">Luckie TM, Danzig M, Zhou S, et al.: A Multicenter Retrospective Review of Pediatric Leydig Cell Tumor of the Testis. J Pediatr Hematol Oncol 41 (1): 74-76, 2019. [<a href="https://pubmed.ncbi.nlm.nih.gov/29554024" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 29554024</span></a>]</div></li></ol></div></div><div id="CDR0000799768__1970"><h2 id="_CDR0000799768__1970_">Treatment Options Under Clinical Evaluation for Childhood Testicular Cancer</h2><p id="CDR0000799768__1971">Information about National Cancer Institute (NCI)–supported clinical trials can be found on the <a href="https://www.cancer.gov/about-cancer/treatment/clinical-trials/search" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">NCI website</a>. For information about clinical trials sponsored by other organizations, refer to the <a href="https://clinicaltrials.gov/" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">ClinicalTrials.gov website</a>.</p><p id="CDR0000799768__1972">The following is an example of a national and/or institutional clinical trial that is currently being conducted:</p><ul id="CDR0000799768__1973"><li class="half_rhythm"><div class="half_rhythm"><b><a href="https://www.cancer.gov/clinicaltrials/NCT03155620" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">APEC1621 (NCT03155620)</a></b> (Pediatric MATCH: Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients with Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphomas, or Histiocytic Disorders)<b>:</b> NCI-COG Pediatric Molecular Analysis for Therapeutic Choice (MATCH), referred to as Pediatric MATCH, will match targeted agents with specific molecular changes identified using a next-generation sequencing targeted assay of more than 4,000 different mutations across more than 160 genes in refractory and recurrent solid tumors. Children and adolescents aged 1 to 21 years are eligible for the trial.</div><div class="half_rhythm">Tumor tissue from progressive or recurrent disease must be available for molecular characterization. Patients with tumors that have molecular variants addressed by treatment arms included in the trial will be offered treatment on Pediatric MATCH. Additional information can be obtained on the <a href="https://www.cancer.gov/about-cancer/treatment/clinical-trials/nci-supported/pediatric-match" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">NCI website</a> and <a href="https://clinicaltrials.gov/ct2/show/NCT03155620" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">ClinicalTrials.gov website</a>.
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</div></li></ul></div><div id="CDR0000799768__2228"><h2 id="_CDR0000799768__2228_">Changes to This Summary (12/17/2021)</h2><p id="CDR0000799768__2229">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="CDR0000799768__2392">This summary was comprehensively reviewed.</p><p id="CDR0000799768__disclaimerHP_3">This summary is written and maintained by the <a href="https://www.cancer.gov/publications/pdq/editorial-boards/pediatric-treatment" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PDQ Pediatric 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="#CDR0000799768__AboutThis_1">About This PDQ Summary</a> and <a href="https://www.cancer.gov/publications/pdq" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PDQ® - NCI's Comprehensive Cancer Database</a> pages.
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</p></div><div id="CDR0000799768__AboutThis_1"><h2 id="_CDR0000799768__AboutThis_1_">About This PDQ Summary</h2><div id="CDR0000799768__AboutThis_2"><h3>Purpose of This Summary</h3><p id="CDR0000799768__AboutThis_3">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of pediatric testicular cancer. 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="CDR0000799768__AboutThis_4"><h3>Reviewers and Updates</h3><p id="CDR0000799768__AboutThis_5">This summary is reviewed regularly and updated as necessary by the <a href="https://www.cancer.gov/publications/pdq/editorial-boards/pediatric-treatment" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PDQ Pediatric 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="CDR0000799768__AboutThis_22"> Board members review recently published articles each month to determine whether an article should:</p><ul id="CDR0000799768__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="CDR0000799768__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 Childhood Testicular Cancer Treatment are:</p><ul><li class="half_rhythm"><div>Denise Adams, MD (Children's Hospital Boston)</div></li><li class="half_rhythm"><div>Julie Blatt, MD (University of North Carolina)</div></li><li class="half_rhythm"><div>Karen J. Marcus, MD, FACR (Dana-Farber Cancer Institute/Boston Children's Hospital)</div></li><li class="half_rhythm"><div>Paul A. Meyers, MD (Memorial Sloan-Kettering Cancer Center)</div></li><li class="half_rhythm"><div>Thomas A. Olson, MD (Aflac Cancer and Blood Disorders Center of Children's Healthcare of Atlanta - Egleston Campus)</div></li><li class="half_rhythm"><div>Alberto S. Pappo, MD (St. Jude Children's Research Hospital)</div></li><li class="half_rhythm"><div>Arthur Kim Ritchey, MD (Children's Hospital of Pittsburgh of UPMC)</div></li><li class="half_rhythm"><div>Carlos Rodriguez-Galindo, MD (St. Jude Children's Research Hospital)</div></li><li class="half_rhythm"><div>Stephen J. Shochat, MD (St. Jude Children's Research Hospital)</div></li></ul><p id="CDR0000799768__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="CDR0000799768__AboutThis_10"><h3>Levels of Evidence</h3><p id="CDR0000799768__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 Pediatric 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="CDR0000799768__AboutThis_12"><h3>Permission to Use This Summary</h3><p id="CDR0000799768__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="CDR0000799768__AboutThis_14">The preferred citation for this PDQ summary is:</p><p id="CDR0000799768__AboutThis_15">PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Testicular Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: <a href="https://www.cancer.gov/types/testicular/hp/child-testicular-treatment-pdq" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">https://www.cancer.gov/types/testicular/hp/child-testicular-treatment-pdq</a>. Accessed <MM/DD/YYYY>. </p><p id="CDR0000799768__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="CDR0000799768__AboutThis_17"><h3>Disclaimer</h3><p id="CDR0000799768__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="CDR0000799768__AboutThis_20"><h3>Contact Us</h3><p id="CDR0000799768__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/NBK550986.3/?report=reader">PubReader</a></li><li><a href="/books/NBK550986.3/?report=printable">Print View</a></li><li><a data-jig="ncbidialog" href="#_ncbi_dlg_citbx_NBK550986" data-jigconfig="width:400,modal:true">Cite this Page</a><div id="_ncbi_dlg_citbx_NBK550986" style="display:none" title="Cite this Page"><div class="bk_tt">PDQ Pediatric Treatment Editorial Board. 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(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> Testicular 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/26389243" ref="ordinalpos=1&linkpos=2&log$=relatedreviews&logdbfrom=pubmed"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" 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