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A Rare Ovarian Tumour Presenting as a Pathologist's Dilemma and a Surgeon's Nightmare

Satinder Kaur*, Megha Nandwani, Hemlata Garg, Shilpa Jamwal

Gynandroblastoma with juvenile Granulosa cell and Sertoli Leydig cell as components is an extremely rare tumor of sex-cord stromal variety. It’s occurrence as a recurrent poorly differentiated Sertoli Leydig cell tumor with heterologous rhabdomyosarcoma component is even a rarer event. A 14 years old girl presented to us in our outpatient department with complaints of a pelvic mass and masculinizing symptoms. She underwent primary cytoreductive surgery at our hospital. The histopathology of the ovarian mass showed both juvenile granulosa cell and Sertoli Leydig differentiation and hence diagnosed as gynandroblastoma. She was given adjuvant chemotherapy and was on regular follow up. The patient developed first recurrence after a disease free interval of 23 months, with an abdominopelvic mass. She underwent secondary cytoreductive surgery for the same. The second histopathology report was suggestive of rhabdomyosarcoma-embryonal type (heterologous differentiation in sex cord stromal tumor). She had multiple relapses and each time the complexity of surgery further increased, and pathology varied. The case presented as a pathologist’s dilemma as adjuvant chemotherapeutic regimen 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.