510 Diagnostic criteria, management, and outcome of three rare vulval tumors: mansoura university experience

Abstract

<h3>Introduction/Background</h3> Vulval cancer represents 5% of Gynecologic cancers. The commonest histology is squamous cell carcinoma. Epithelioid sarcoma (ES) of the vulva is a rare and an aggressive malignant soft tissue neoplasm. It is classified into distal, conventional, or classic type and proximal type. Aggressive angiomyxoma is a rare, benign, slowly growing, locally invasive mesenchymal tumor which carries a high risk for local relapse. Primary surgical resection is the mainstay of treatment. Granular cell tumors are rare, usually benign, soft tissue neoplasms of neural origin. They can occur anywhere in the body with 15% situated in the vulva. The commonest presentation is as an asymptomatic mass. The vulvar tumors were malignant in 2% of granular cell tumors. <h3>Methodology</h3> Retrospective reporting of Three rare types of vulval tumors who were managed at the Mansoura Gynecologic Oncology Unit the last 3 years. Diagnostic criteria, management, and outcome will be presented. <h3>Results</h3> The first case is a 25-year-old lady, medically free; referred to our hospital following an excisional biopsy of a painless 7X5 cm-sized lump just outside the right labia majora, at a private hospital. Histopathologic examination revealed proximal type ES with infiltrated skin margin. Chest/Abdomen/Pelvis CT with contrast was free. After MDT discussion; wide local excision and bilateral inguinal lymphadenectomy were performed. Pathology revealed presence of a Yolk sac element with unilateral lymph node metastasis. The MDT recommended adjuvant chemotherapy. The second case is a 28-year-old virgin female presenting with a large pedunculated cauliflower swelling on right labia majora measuring 25X15X 10 cm. Wide local excision with safety margin was performed. Histopathology revealed aggressive angiomyxoma. The third case was diagnosed as recurrent vulval granular cell tumor in 31 years old female who was managed by wide local excision with plastic reconstruction using V-Y skin flap. <h3>Conclusion</h3> Centralization and multidisciplinary management of rare vulval tumors is essential. <h3>Disclosures</h3> No conflicts of interest.