Case report offers insight into treatment of rare FATWO tumor.
Usually considered to have low malignant potential, most female adnexal tumors of probable wolffian origin (FATWOs) arise on the broad ligaments, but other reported sites include the rate ovary, paravaginal area, and retroperitoneum.
The patient described by Dr. Steed was the youngest reported to date: a 15-year-old girl who presented with abdominal pain that was at first misdiagnosed as cystitis, and then, following ultrasound, as a dermoid cyst. Further tests revealed a large retroperitoneal and broad ligament mass; at the time of resection 1 month later the tumor was 14 cm by 11 cm by 8 cm.
The pathology revealed a characteristic pattern for FATWO tumors, including solid neoplastic epithelial cells in "closely packed branching tubules," said Dr. Steed, a subspecialty fellow in gynecologic oncology at the University of Toronto.
The tumor recurred at the same site within 2 years and was treated with numerous chemotherapy regimens followed by surgery a year later for progressive disease. Another recurrence 18 months later led physicians to try Gleevac therapy, a tyrosine kinase inhibitor, because the tumor was positive for "c-kit" oncogene (CD117).
Persistent pelvic pain prompted a laparotomy 2 months after the therapy was initiated. Pathologic specimens obtained during the debulking of retroperitoneal masses and hysterectomy showed evidence of tumor necrosis, suggesting a partial response. The patient was maintained on Gleevac therapy and was free of disease 1 year after surgery. A 2-cm nodule below the right lobe of her liver evident on her postoperative CT scan has resolved.
A review of the literature by Dr. Steed and her associates revealed 72 reported FATWO cases, including 8 that recurred.
The median age of a patient diagnosed with FATWO was 50, with a range from 15 to 83 years. Abdominal or pelvic pain is the most common chief presenting complaint although some patients present with urge/frequency bladder symptoms.
Recurrences are uncommon but do happen, usually within 48 months, and close long-term follow-up is necessary.
BY BETSY BATES
Los Angeles Bureau