ABSTRACT
Prepubertal testicular tumors are less common than other pediatric urologic tumors such as neuroblastoma and Wilms’ tumor, and testicular tumors are far more common in adults than in children. Historically, the management of pediatric testicular tumors has been based on experience in adults. Indeed, testicular tumors in adults and children have many similarities. Both usually present with a testicular mass and are treated initially with excision of the primary tumor. In both children and adults, testicular tumors are particularly sensitive to platinum-based chemotherapy, which has revolutionized the management of testicular cancer throughout the age spectrum.1,2 However, there are important differences between testis tumors occurring in children and adults. These differences occur in the tumor histopathology, malignant potential, and pattern of metastatic spread. The patients themselves are also dissimilar, with different concerns regarding surgical morbidity and preservation of testicular function. The introduction of a Prepubertal Testicular Tumor Registry by the Section on Urology of the American Academy of Pediatrics (AAP) in 1980 allowed for the accrual of valuable epidemiologic information.3 Recent multicenter and prospective clinical trials have further advanced our understanding of these tumors and their management.4-6
There is a bimodal age distribution for testis tumors, with one peak occurring in the first 2 years of life, and a second larger peak occurring in young adulthood. The incidence of pediatric testis tumors is 0.5-2.0 per 100 000 children, accounting for 1-2% of all pedi-
atric tumors.7 Testis tumors are categorized, based on the presumed cell of origin, into stromal tumors and germ cell tumors. In the Prepubertal Testicular Tumor Registry of the Section on Urology of the AAP, the majority of primary testis tumors were yolk sac tumors followed by teratomas and stromal tumors.3 However, selection bias can lead to an overrepresentation of malignant tumors in registries. Indeed several recent studies have found teratoma to be more common than yolk sac tumor.8-12 Table 76.1 summarizes the distribution of testis tumors in the Prepubertal Testicular Tumor Registry and a multicenter review of prepubertal testicular tumor pathology specimens. Because teratomas and most stromal tumors are benign in children, the percentage of prepubertal testis tumors that have malignant potential is much lower than the 90% of tumors in adults.
