Genital/urinary tumors include carcinoma of the bladder, non-germ cell testicular cancer, non-germ cell ovarian cancer, and carcinoma of the cervix and vagina. The prognosis, diagnosis, classification, and treatment of these genital/urinary tumors are discussed below. It must be emphasized that these tumors are seen very infrequently in patients younger than 15 years, and most of the evidence is derived from case series.
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.
Incidence, risk factors, and clinical presentation
Carcinoma of the bladder is extremely rare in children. The most common childhood carcinoma to involve the bladder is papillary urothelial neoplasm of low malignant potential, which generally presents with hematuria.[1,2,3] High-grade, invasive, urothelial carcinomas are extremely rare in young patients.
In contrast to adults, most pediatric bladder carcinomas are low grade, superficial, and have a good prognosis following transurethral resection.[2,3,6,7,8,9] Squamous cell carcinoma and more aggressive carcinomas, however, have been reported and may require a more aggressive surgical approach.[3,10,11,12]
(Refer to the PDQ summary on adult Bladder Cancer Treatment for more information.)
Testicular Cancer (Non-Germ Cell)
Testicular tumors are very rare in young boys and account for an incidence of 1% to 2% of all childhood tumors.[13,14] The most common testicular tumors are benign teratomas followed by malignant nonseminomatous germ cell tumors. (Refer to the PDQ summary on Childhood Extracranial Germ Cell Tumors for more information.) 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.[15,16] In newborns and infants, juvenile granulosa cell and Sertoli cell tumors are the most common stromal cell tumor. Juvenile granulosa cell tumors usually present in infancy (median age, 6 days) and Sertoli cell tumors present later in infancy (median age, 7 months). The prognosis is usually excellent after orchiectomy. In older males, Leydig cell tumors are more common. Stromal cell tumors have been described as benign in young boys.[19,20,21]