46XX pure gonadal dysgenesis with dysgerminoma along with leydig cells: a unique presentation

Authors report a unique case of 46XX gonadal dysgenesis, with dysgerminoma in one ovary and other streak ovary with hilar nests of leydig cells. It is exceptionally rare to find dysgerminoma in a dysgenetic gonad with no Y chromosome and so is the presence of leydig cells in the contralateral streak ovary in a patient with 46XX pure gonadal dysgenesis.

Sex cord stromal tumor of ovary masquerading as polycystic ovarian syndrome

Virilization is a portentous sign that suggests the possibility of an ovarian or adrenal neoplasm. Diagnosis may be delayed in some patients due to nonspecific symptoms and overlapping symptoms with that of polycystic ovarian syndrome (PCOS). However, it must be remembered that PCOS usually causes mild to moderate elevation of serum testosterone with hirsutism whereas serum testosterone levels are many times elevated in cases of androgen secreting tumors and virilization is a norm. So high testosterone level with new onset virilization rule out PCOS. Authors are reporting two cases of Sertoli Leydig cell tumor despite their similar histopathology and equivalent levels of serum testosterone had a varied clinical spectrum of virilization.