It is possible that the main title of the report Multiple Endocrine Neoplasia Type 2 is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Testicular germ cell tumors (GCTs) in children occur almost exclusively in boys younger than 4 years.[1,2] The initial surgical 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.
Computed tomography or magnetic resonance imaging evaluation, with the additional information provided by elevated tumor markers, appears adequate for staging. Retroperitoneal dissection of lymph nodes is not beneficial in the staging of testicular GCTs in young boys.[3,4] Therefore, there is no reason to risk the potential morbidity (e.g., impotence and retrograde ejaculation) associated with lymph node dissection.[6,7]
The role of surgery at diagnosis for GCTs is age- and site-dependent and must be individualized. All malignant testicular GCTs should be resected. Primary resection of other areas of disease may be appropriate when feasible, without undue risk of damage to adjacent structures; otherwise, an appropriate strategy is resection of the testis for diagnosis followed by subsequent excision in selected patients who have residual masses after undergoing chemotherapy.
Standard treatment options for malignant GCTs in prepubertal males
Standard treatment options for malignant GCTs in prepubertal males (younger than 15 years) include the following:
Surgery and observation (stage I).
Surgery and chemotherapy (stages II through IV).
The treatment options for malignant GCTs in prepubertal males differ by stage of disease.
Surgery and close follow-up observation are indicated to document that a normalization of the tumor markers occurs after resection.[8,3]
Evidence (surgery and observation for stage I disease):
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.[3,4]
This treatment strategy resulted in a 6-year event-free survival (EFS) of 82%.
Boys who developed recurrent disease received salvage therapy with four cycles of standard-dose cisplatin, etoposide, and bleomycin (PEB), with a 6-year survival of 100%.