3.P.49 PROGNOSIS AND TREATMENT OF PERISCAPULAR FIBROMATOSIS

Abstract

Desmoid tumour is an histological benign tumour. Nevertheless, peri-scapular relapses can decrease the function and intra thoracic progression threaten life. To prevent these complications, damaging treatment (radiotherapy, amputation) are sometimes proposed. To precise the optimal indications of treatment, we reviewed our cases.

Patients: from 1984 to 2008, we treated 11 patients with peri-scapula fibromatosis (mean age 42 (13–58)). Only 4 patients were seen at first hand, 7 for relapses (3 of them after radiotherapy).

Treatment was adapted to each patient, in function of age, history of illness, and risks of spontaneous evolution. En bloc extratumoral resection was performed each time, when it didn’t expose to heavy functional risk (8). The other patients were treated by contaminated resection, but never invaliding. 4 patients received pre or/and post-operative chemotherapy. 1 received Interferon alpha, and 7 tamoxifen.

Results: with a median follow-up 15 years 3 months, 7 patients suffered of recurrence. No patient died from disease (thoracic complications) or therapeutic complication9 patients are in complete remission and 2 in stable disease. Following radiotherapy, local relapses (7 cases) and repeated surgery, functional sequellaes are numerous: 2 circumflex nerve palsies, 3 articular stiffness. Major functional sequellaes came from radiotherapy (limb discrepancy, lung and thorax deformity, skin and muscle atrophy.

Conclusion: in this non predictable illness, therapeutic indications should individually balance risks of spontaneous evolution and of complications of treatment. Besides surgery, needed in fast all cases, but often insufficient, it must be considered the value of interferon, tamoxifen and/or chemotherapy. The most important concept is the necessity to treatment avoiding late sequellaes and particularly radiotherapy or mutilating surgery.