RESUMO
Aggressive fibromatosis is a rare neoplasm arising from musculoaponeurotic structures. Our aim is to share our experience with this rare tumor in our institute and to discuss the more perplexing recurrence patterns and the management options. This is a retrospective study of the disease, treated in our institute for the past fourteen years. A total of 36 patients were analyzed. The demographic pattern of the disease, various treatment modalities offered and their outcome along with patterns of recurrence were studied. Our study showed a demographic pattern mostly similar to the rest of the world. But the pattern of recurrence and the multicentric and the non-random pattern of presentation observed in our study showed some difference from the other studies. We suggest surgery as the primary modality with radiation reserved for select patients with margin positivity, inoperable tumors, and multiple tumors. Since the disease has a long natural history a wait and watch policy can be observed for giving adjuvant RT. There is need for prospective multi-institutional RCTs to shed light on the unknown facts about this disease.