It is an autosomal dominant disorder that is characterized by infiltrative fibromatosis of the mesentery without colonic polyps, osteomas, and sebaceous cysts. It is known to predispose to desmoid tumor but its association with colon cancer is unknown.
Incidence and genetic inheritance
Incidence unknown. Autosomal dominant inheritance.
These are fibroproliferative disorders in which histologically benign tumors of musculoaponeurotic tissue arise and are often locally invasive. They occur more commonly in patients with familial adenomatous polyposis. The fibromatosis may be intraabdominal (mesenteric) or extraabdominal, such as paraspinal, limb, buttock, flank, head, and neck.
Patients receive multidisciplinary treatment, so they may present for surgery after having received chemotherapy or radiotherapy. Patients with tumors of the head and neck may require careful airway management planning according to the site of the tumor(s).
et al: Fibromatoses in childhood: The desmoid/fibromatosis complex. Med Pediatr Oncol