Abstract
Gonadblastoma is a rare tumor that may occur in the gonad of Y-chromosome-related disorders of sex development (DSD). Although gonadoblastoma is a benign tumor, dysgerminoma could occur from gonadoblastoma. Therefore, preventive gonadal resection is recommended. A relevant period of gonadal resection was discussed based on our study on 5 cases with Y-chromosome-related DSD and other reported Japanese cases since 2000.
Among our 5 cases, gonadoblastoma was observed in 2 cases that were 8 and 4 years old at the time of surgery; no neoplastic lesion was observed in the other 3 cases (less than 2 years old). No cases had dysgerminoma. In the reported Japanese cases, gonadoblastoma was observed in 17 cases, and dysgerminoma in 10 cases (9 cases: overlapping). In our cases and reported cases, the youngest case with gonadoblastoma was 2 years old, and the other with dysgerminoma was 15 years old.
It is possible for children with Y-chromosome-related DSD to suffer gnadoblastoma at 2 years of age or older, and additionally dysgerminoma at 15 years old or older. Therefore, preventive gonadal resection should be considered for cases more than 2 years old.
