Recurrent Abdominal Aggressive Fibromatosis with Incapacitating Hip Contracture - A Case Report.

Abstract

Aggressive fibromatosis (AF) is a fibroblastic locally aggressive neoplasm arising from the musculoaponeurotic stroma and has no metastatic potential. The high tendency of recurrence despite complete surgical resections makes the management of the condition onerous. It can result in significant morbidity with major functional loss due to the destruction of adjacent vital structures and organs. AF with hip flexion contracture is a very rare occurrence. A 20-year-old male presented with recurrent abdominal AF with severe hip flexion contracture and an unresectable tumor. He underwent deformity correction and he maintains the full correction achieved along with very good functional improvement at the end of 4 years. This case demonstrates that in a case of AF with an unresectable tumor, good functional outcome can be obtained; it can be maintained over the short term following contracture release with soft-tissue coverage surgery along with chemotherapy with sorafenib.