Management of recurrent dysgerminoma arising in a dysgenetic gonad

Abstract

Gonadoblastomas are rare gonadal tumors, arising from a dysgenetic gonad with a Y chromosome. These tumors may manifest as dysgerminomas and if not appropriately managed can lead to disastrous consequences. 24 year female reported with lump in abdomen and abdominal pain on and off since 6 months and primary amenorrhoea. She underwent laparotomy with removal of an abdominal mass at a hospital 2 years back for similar complaints. Histopathology report of the mass read 'Dysgerminoma/Seminoma with both male and female elements'. Thereafter, patient was referred to a higher oncology centre for further management. 2 years hence she reported to us without any follow up in oncology centre as advised. On clinical and CT examination a diagnosis of recurrent tumor mass was made. Laparotomy revealed a non-resectable mass, whose biopsy report was Dysgerminoma. Patient was put on BEP regime. Second look laparotomy showed a significantly regressed necrotic mass with no evidence of residual malignancy. Till date she has shown no sign of recurrence of disease. Patients with dysgenetic gonads should be investigated judiciously and appropriately managed.

Keywords

• Disorder of sexual development
• dysgenetic gonad
• dysgerminoma
• gonadoblastoma