INDIVIDUALIZING MANAGEMENT OF AGGRESSIVE FIBROMATOSES

Purpose: To examine prognostic indicators in aggressive fibromatoses t hat may be used to optimize case-specific management strategy. Methods and Materials: One hundred and seven fibromatoses presenting between 1971 and 1992 were analyzed. The following treatment modalities were u tilized: (a) surgery alone for 51 tumors; (b) radiation alone for 15 t umors; and (c) radiation and surgery (combined modality) for 41 tumors . Outcome analysis was based on 5-year actuarial local control rates. Results: Control rates among surgery, radiation therapy, and combined modality groups were 69%, 93%, and 72%. Multivariate analysis identifi ed age <18 years, recurrent disease, positive surgical margins, and tr eatment with surgery alone as predictors for failure. Patients treated with surgery alone had control rates of 50% (3 of 6) for gross residu al, 56% for microscopically positive margins, and 77% for negative mar gins. Radiation and surgery resulted in rates of 59% for gross residua l, 78% for microscopically positive margins, and 100% (6 of 6) for neg ative margins. For recurrent vs. primary tumors, control was achieved in 48% vs. 77%, 90% vs. 100% (5 of 5), and 67% vs. 79% in the Surgery, Radiation, and Combined modality Groups, respectively. Patients prese nting with multiple disease sites tended to have aggressive disease. A radiation dose-control relation to >60 Gy was seen in patients with u nresected or gross residual disease. Of the patients, 23 with disease involving the plantar region had a control rate of 62%, with significa ntly worse outcomes in children. Conclusions: These results are consis tent with those found in the relevent literature. They support primary resection with negative margins when feasible. Radiation is a highly effective alternative in situations where surgery would result in majo r functional or cosmetic defects. When negative surgical margins are n ot achieved in recurrent tumors, radiation is recommended. Perioperati ve radiation should be considered in other high-risk groups (recurrent disease, positive margins, and plantar tumors in young patients). Dos es of 60-65 Gy for gross disease and 50-60 Gy for microscopic residual are recommended. Observation may be considered for primary tumors wit h disease remaining in situ when they are located such that progressio n mould not cause significant morbidity. Although plantar lesions in c hildren may represent a group at high risk for recurrence or aggressiv e behavior, the greater potential for radiation-induced morbidity in t his group must also temper its use. Given the inconsistent nature and treatment response of this tumor, it is fundamental that treatment rec ommendations should be made based on the risk:benefit analysis for the individual patient, dependent on tumor characteristics and location, as well as patient characteristics and preferences. (C) 1998 Elsevier Science Inc.