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.