Surgical management of desmoid tumors of the female pelvis

Background: Desmoid tumors are neoplasms characterized by proliferation of fibroblasts without cytologic features of malignancy. Although desmoid tumo rs are not uncommon, pelvic desmoid tumors are rare and therapeutically cha llenging. We describe the surgical management of seven patients with histol ogically confirmed desmoid tumors arising in the pelvis. Study Design: All patients, six with primary and one with recurrent desmoid tumors of the female pelvis, were treated at Mayo Clinic Rochester between 1976 and 1997. The mean age of one African-American and six Caucasian pati ents was 29.8 years (range 17 to 59 years). No history of previous relevant pelvic trauma was identified; six patients experienced pelvic or leg pain (or both). All patients presented with isolated masses; five of seven were located left of the midline, generally involving the obturator internis or levator muscles (or both). The surgical approach was transabdominal, with a vaginal component in one patient. In addition to this retrospective review of these seven patients, literature addressing the management of desmoid t umors of the female pelvis is reviewed. Results: Extirpative surgery necessitated resection of musculoskeletal, vas cular, or adjacent soft tissue structures. Mean tumor diameter was 13.9 cm (range 5 to 27 cm). Although the median recorded blood loss was 1,425 mL, o ne patient who refused blood products died secondary to surgical hemorrhage . The median followup for the remaining evaluable patients was 57 months (r ange 13 to 249 months). Recurrent disease developed in 3 of 6 patients at 3 , 11, and 13 months after surgical resection and was successfully managed w ith subsequent reoperations, with or without adjuvant radiotherapy. Conclusions: Although recent reports demonstrate selective regression of de smoid tumors with antiestrogen therapy, management of symptomatic pelvic de smoid tumors in young women may necessitate an operation. In our series, al l primary and recurrent tumors were managed with resection of involved bone and other adjacent soft tissues. The role of adjunctive radiotherapy or ho rmonal therapy remains to be defined. (J Am Coll Surg 2000;191:175-183. (C) 2000 by the American College of Surgeons).