Abstract
Desmoid tumours are known for their varied clinical behaviour. Hence, choosing an ideal treatment protocol remains challenging. This is further complicated by the rarity of these neoplasms and high recurrence rates following treatment. In our study, we tried to answer these questions in the background of Indian scenario. This is a retrospective study of 41 patients with extremity fibromatoses who were operated between June 2002 and November 2012. The mean age for all patients was 29.2 years with 30 females and 11 males. The mean duration of follow-up was 4.37 years. Eight patients were margin-positive on final histopathology. Seventeen developed recurrences (41.4%). Ten of these 17 patients underwent repeat surgery. The remaining 7 patients with inoperable recurrence were put on metronomic chemotherapy. The DFS for patients treated with surgical management was 62.6% at 3 years, 54.4% at 5 years and 44% at 10 years. The enigmacy on the tumour biology, natural history and optimal management of fibromatoses continue. Surgery remains the standard treatment and should be attempted only if R0 resection is possible without much morbidity to the patient. Non-surgical modalities also have their role to play in the management of these neoplasms.