Vulvar squamous cell carcinoma associated with Fanconi's anemia

Fanconi's anemia is a rare autosomal recessive disorder which is rarely associated with squamous cell carcinoma (SCC) of the vulva. We report a 23-year-old virgin female with Fanconi's anemia and diabetes mellitus who presented with a history of 6-month ulcerative lesions of the vulva. Gynecologic examination disclosed a 1 x 2 cm ulcerated tumor lesion at the right labia minor near to the urethra. The biopsy showed a high-grade vulvar intraepithelial neoplasia (VIN III). She underwent wide local excision for this lesion. Pathologic examination of the surgically removed specimen revealed SCC of the vulva. Therefore, radical vulvectomy and bilateral inguino-femoral lymphadenectomy were performed. Due to the involvement of right inguinal lymph node, radiotherapy with a total dose of 45 Gy was delivered to mid-pelvis through antero-posterior/postero-anterior fields with 18 mV photon energies. Until her last follow-up about 1 year after the treatment, the patient was free of disease or any recurrence at the site of operation. Patients with Fanconi's anemia have the risk of developing SCC of the genital tract. Radical vulvectomy and lymphadenectomy along with radiotherapy were associated with a satisfactory outcome in 1-year follow-up period in the presented patient.