[Persistent Müllerian duct syndrome with seminoma: report of a case]

Persistent Müllerian duct syndrome is a male hermaphroditism in which remnants of Müllerian ducts occur from abnormality of Müllerian inhibitory factor and often cause transverse testicular ectopia or hernia uteri inguinalis. A 22-year-old-man was admitted with the chief complaint of painless swelling of the right scrotal content. Preoperative diagnosis was right testicular tumor and left undescended testis. High orchiectomy was done. At operation left transverse testicular ectopia and a remnant Müllerian duct was found incidentally. The right testicular tumor was seminoma pT1. Sixty-seven cases of transverse testicular ectopia have been reported in Japan, 37 of which are reports of a remnant Müllerian duct. Ten of these cases were accompanied by tumors. The pathogenesis and treatment of persistent Müllerian duct syndrome were discussed with review of the literature.