gms | German Medical Science

Objectives: Recent guidelines for the treatment of desmoid-type fibromatosis have suggested watchful waiting as primary therapy of choice for most patients. Surgical resection has been regarded a preferred treatment option over a long time, its role becoming more debatable with increasing evidence for an unpredictable course of the disease, frequently unrelated to the quality of margins achieved during surgery. In this context, we aimed to assess our surgical results within a historical group of patients undergoing resection of extra-abdominal fibromatosis.

Methods: Out of the Vienna Bone and Soft Tissue Tumor Registry we have identified all patients with fibromatosis, excluding patients with palmar and plantar lesions, patients not undergoing surgery and patients with inadequate follow-up: this left 63 cases for further analysis including 23 males (37%) and 40 females (63%) with a mean age of 34 years, ranging from 7 to 72. Twenty tumors were located in the trunk (32%), while the other 43 were in the extremities (68%). All except one patient with primary interscapulothoracic amputation (2%) underwent tumor resection. Ten patients (16%) had an additional resection of bony structures, three of them requiring endoprosthetic reconstruction (5%).

Results and conclusion: The median follow-up was 52 months. None of the patients died of disease related causes. Overall, 23 patients developed a local recurrence (LR) after a median time of 19 months, representing a LR rate of 37%. After repeated resection eleven of these patients (48%) had one to three further recurrences. Hence, the 5- and 10-year LR-free survival was 55%. Female patients showed a significantly better LR-free survival compared to males (p=0.016). Lesions in the extremities showed a significantly worse LR-free outcome, as compared to central tumors (p=0.002). Radiation had no significant impact on LR, while the surgical margin according to Enneking trended to have better outcomes with wider margins.

Facing a high LR rate and repeated recurrence rate after surgical resection of fibromatosis, we conclude to currently advocate a more conservative approach by watchful waiting. Surgery should be restricted to selected symptomatic patients. In case of surgery, a wide margin should be attempted, whenever feasible.