PURPOSE This study was undertaken to analyze our cohort of patients with vulvar cancer, the factors affecting their survival, and to review published Indian literature. MATERIALS AND METHODS A prospectively maintained database of 18 patients with vulvar cancer who were amenable to up-front surgery and treated between 2013 and 2021 was analyzed. Patients were studied and evaluated for clinicopathological features, risk factors, stage of disease, surgical modalities, and disease outcome. This study critiques the 16 studies identified in the English literature relating to vulvar carcinoma from 1996 to 2021 from India. RESULTS The mean age of our patients was 63.7 years. All 18 patients underwent up-front radical surgery with primary closure. Postoperative histology was squamous cell carcinoma with negative margins in all and lymph-vascular space involvement negative in 17 of 18 patients. Of the 10 patients who underwent inguinofemoral lymphadenectomy, four patients had positive lymph nodes. The estimated mean survival was 61.6 months. We reviewed the Indian literature for over 25 years. As the majority of patients presented with a locally advanced stage of the disease, the primary mode of treatment was chemoradiotherapy. Lymph node positivity and lack of appropriate management at relapse increased the risk of poor survival. CONCLUSION This review clearly emphasizes the unmet need for more prospective multicentric trials not only to increase our understanding about the disease but also to define better treatment protocols for various stages of the disease, thereby improving disease-free and overall survival.
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.
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