Pathophysiology: Desmoid tumors are cytologically bland fibrous neoplasms originating from the musculoaponeurotic structures throughout the body. Desmoid tumors often appear as infiltrative, usually well-differentiated, firm overgrowths of fibrous tissue, and they are locally aggressive. The synonym aggressive fibromatosis describes the marked cellularity and aggressive local behavior.
Treatment: The primary treatment for desmoid tumor is primary surgery with negative surgical margins. In those patients who refuse surgery or are not surgical candidates, the treatment used is radiation therapy. Pharmacologic therapy with antiestrogens and prostaglandin inhibitors may also be used.
Major research: Management of sporadic desmoid-type fibromatosis: A European consensus approach based on patients' and professionals' expertise - A Sarcoma Patients EuroNet and European Organisation for Research and Treatment of Cancer/Soft Tissue and Bone Sarcoma Group initiative. 2) Isolated limb perfusion using tumour necrosis factor α and melphalan in patients with advanced aggressive fibromatosis.