BackgroundDesmoid-type fibromatosis (DF) is a aggressive (myo)fibroblastic neoplasm with an infiltrative growth and a tendency to local recurrence. Resection of the tumour and/or radiation were proposed as principal treatment. The aim of this retrospective study was to analyze the local control rates focusing on the effect of surgical margins and radiotherapy.MethodsFrom 1981 to 2014, 44 patients had been treated. Fifty four therapies had been applied, in 50 cases surgery +/− radiation therapy, NSAIDs or chemotherapy. In 4 cases a conservative approach was chosen. Thirty seven patients had primary, 17 recurrent disease. Endpoint was either local recurrence (LR), progression of residual disease or rare non-metastatic secondary lesions at the same extremity.ResultsThe mean age was 39,4 years. In 17 cases a R0, in 27 a R1 and in 6 cases a R2 resection was achieved. Four patients were treated conservatively. All together in 21 cases radiotherapy, in 5 NSAIDs, in 3 imatinib and in 2 cases each tamoxifen or chemotherapy had been applied. The median follow-up was 119 months. 5-year recurrence free survival after resection was 78%. 10 (20.4%) patients developed LR between 5 and 42 months after therapy. Recurrent disease was a negative factor on LR. Margins, radiotherapy, sex, or size of the tumour had no significant impact on LR. Patients younger than 40 years had a significant higher risk of LR.ConclusionsSurgical margins are less important than keeping function. Radiotherapy might be an option in unresectable lesions, the role of adjuvant radiotherapy is controversially discussed.

Background Desmoid-type fibromatosis (DF) is an aggressive (myo)fibroblastic neoplasm with an infiltrative growth pattern and a tendency for local recurrence. It is rarely seen at the foot. The aim of this retrospective study was to analyze clinical presentation, therapy, and outcomes in a consecutive series of four patients with DF at the foot. Methods From 1994 to 2014, four patients had been surgically treated. The resection margin was marginal or even intralesional in all. One patient already had local recurrence at first presentation. The end point was either local recurrence or progression of residual disease. Results The mean patient age was 27 years. In one patient, marginal excision healed the disease. In another patient, local recurrence after marginal resection necessitated distal phalanx amputation. Two other patients showed stable disease after either adjuvant radiotherapy or treatment with nonsteroidal anti-inflammatory drugs and tamoxifen. Conclusions If surgery is necessary, operative margins are less important than keeping function for the patient. Radiotherapy might be an option to avoid major amputation. The role of adjuvant radiotherapy is controversially discussed. A watchful wait-and-see policy seems to be justified by the published data but may be difficult for DF at the foot.