試験開腹後27年を経過してセミノーマの発生を見た混合型性腺形成不全症の1例

Abstract

A case of mixed gonadal dysgenesis with anaplastic seminoma is herein reported. A 38-years-old man was admitted to our hospital with the chief complaint of a painful mass, 19.0 cm in long diameter in his lower abdomen. At 11, 13 and 14 years old he had the history of treatment for an ambiguous external genitalia and defect of both scrotal contents. In the concrete, laparotomy, chordectomy and urethroplasty were performed at those times and the laparotomy revealed a right immature abdominal testis and Mullerian structures. Laboratory examination revealed elevated levels of serum lactic dehydrogenase and HCG. The karyotype from the cultured lymphocyte was that of a normal male. Because the histological finding of biopsied abdominal tumor was shown to be an anaplastic seminoma, firstly two courses of chemotherapy using cisplatin vinblastine and bleomycin were induced, and that after an extensive resection for the tumor and Mullerian structure including some portions of surrounding tissue was performed. We reviewed 40 cases of mixed gonadal dysgenesis reported previously in the Japanese literature and 8 cases of that with a gonadal tumor.