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Childhood Extracranial Germ Cell Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment of Malignant Gonadal GCTs

Childhood Malignant Testicular GCTs

Testicular GCTs in young boys

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General Information About Gastrointestinal Carcinoid Tumors

Epidemiology The age-adjusted incidence of carcinoid tumors worldwide is approximately 2 per 100,000 persons.[1,2] The average age at diagnosis is 61.4 years.[3] Carcinoid tumors represent about 0.5% of all newly diagnosed malignancies.[2,3] Anatomy Carcinoid tumors are rare, slow-growing tumors that originate in cells of the diffuse neuroendocrine system. They occur most frequently in tissues derived from the embryonic gut. Foregut tumors, which account for up to 25% of cases, arise...

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Testicular germ cell tumors (GCTs) in children occur almost exclusively in boys younger than 4 years.[1,2] The initial approach to evaluate a testicular mass in a young boy is important because a transscrotal biopsy can risk inguinal node metastasis.[3,4] Radical inguinal orchiectomy with initial high ligation of the spermatic cord is the procedure of choice.[5] Retroperitoneal dissection of lymph nodes is not beneficial in the staging of testicular GCTs in young boys. Computed tomography or magnetic resonance imaging evaluation, with the additional information provided by elevated tumor markers, appears adequate for staging.[3,4] Therefore, there is no reason to risk the potential morbidity (e.g., impotence and retrograde ejaculation) related to lymph node dissection.[6,7]

A Children's Cancer Group (CCG)/Pediatric Oncology Group (POG) clinical trial evaluated surgery followed by observation for boys aged 10 years or younger with stage I testicular tumors. This treatment strategy resulted in a 6-year event-free survival (EFS) of 82%; those boys who developed recurrent disease were salvaged with four cycles of standard-dose cisplatin, etoposide, and bleomycin (PEB), with a 6-year survival of 100%.[3,4] Boys younger than 10 years with stage II tumors were treated after diagnosis with four cycles of PEB.[8] Boys and adolescents (age not limited to 10 years or younger) with stage III and IV testicular tumors were treated with surgical resection followed by four cycles of standard or high-dose (HD)-PEB therapy. The 6-year survival outcome for males younger than 15 years with stage III and IV tumors was 100%, with 6-year EFS of 100% and 94%, respectively.[9] The use of HD-PEB therapy did not improve the outcome for these boys but did cause increased incidence of ototoxicity. Excellent outcomes for boys with testicular GCTs using surgery and observation for stage I tumors and carboplatin, etoposide, and bleomycin (JEB) and other cisplatin-containing chemotherapy regimens for stage II, III, and IV tumors have also been reported by European investigators.[6,10] Thus, surgery followed by standard-dose platinum-based chemotherapy is the recommended approach for stages II, III, and IV testicular GCTs in children younger than 15 years.

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