A rare ovarian tumor presenting as a Pathologist's dilemma and a Surgeon's nightmare

Abstract

Gynandroblastoma with juvenile granulosa cell and Sertoli Leydig cell as components is an extremely rare tumor of sex-cord stromal variety and its occurrence as a recurrent poorly differentiated Sertoli Leydig cell tumor with heterologous rhabdomyosarcoma component is even a rarer event. A 14 years old female presented to us in our outpatient department with complaints of a pelvic mass and masculinizing symptoms. She was evaluated at our hospital and underwent primary upfront surgery; laparotomy with left salpingo-oophorectomy (ovarian mass excision) with retroperitoneal lymph node dissection with omentectomy. The histopathology of ovarian mass showed both juvenile granulosa cell and Sertoli Leydig differentiation, diagnosed as gynandroblastoma. The patient developed recurrence with a disease-free interval of 23 months and underwent secondary cytoreductive surgery for the same. The second histopathology report was suggestive of rhabdomyosarcoma; embryonal type (heterologous differentiation in sex cord stromal tumor). The case presented as a pathologist’s dilemma as adjuvant chemotherapeutic regimens changed based on the dominant component of the tumor. Also, as intra-operatively the tumor was extremely vascular, its surgical management proved to be a nightmare for the operating surgeon.