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: 1)Diagnosis and management of the intra-abdominal desmoid tumour. 2)Prevalence of KIT Expression in Human Tumor.
Desmoid tumours comprise 0.03% of all neoplasms, less than 3% of all soft tissue tumours (1), with an incidence of 2-4 cases / 1’000’000 per year (2). They are usually detected relatively early on in life (10-40 years of age), with a slight female predominance.