Desmoid fibromatosis of submandibular region

Desmoid tumors are benign musculoaponeurotic tumor accounting for almost 0.03% of all neoplasms. Despite their benign nature, they are known as aggressive fibromatosis, with the potential to cause damage to surrounding structures, leading to organ damage. It is caused by mutations of fibroblast cells that are found throughout the body. Fibroblasts play crucial role in wound healing and protection of vital organs such as lungs, liver, blood vessels, heart, and kidneys. When mutations, either sporadic or inherited occur in these cells, they become neoplastic and lead to formation of desmoid tumors. Thus, aggressive fibromatosis is a monoclonal proliferative disease with inability to metastasise. [1] Despite their purely benign histological appearance and insignificant potential to metastasise; the tendency of desmoid to cause infiltration is of a great concern in the context of deformity, morbidity and mortality resulting from pressure effect on cellular level up to the obstruction of vital structures and organs. Besides that, the high prevalence of recurrence makes the treatment of these rare fibrous tumors challenging. Moreover, the depth of invasion of the tumor is associated with the rate of recurrence.