Background: A female adnexal tumor of probable Wolffian origin (FATWO) is a rare neoplasm which is considered to have a low malignant potential.
Case: In 2006, a 60-year-old patient was admitted to hospital with solid tumor on the vaginal cuff confirmed by abdominal/pelvic CT, 6 years after her first FATWO was diagnosed and hysterectomy, bilateral adnexectomy, and omentectomia were done. No chemotherapy was administrated after her first surgery. Pelvic washings, lysis of adhesions, and excision of tumor mass were performed. Pathologic examination revealed malignant epithelial neoplasm of Wolffian origin with morphologic features similar to those of the patient's prior tumor. Immunohistochemical staining was positive for vimentin and partially low positive for cytokeratin 19. No chemotherapy was administrated. Two years after second surgery, the patient was asymptomatic and her clinical, laboratory, CT, and chest X-ray findings were unremarkable.
Conclusion: Surgical debulking with hysterectomy and bilateral adnexectomy appears as the most successful initial treatment for FATWOs.