aggressive infantile fibromatosis
ag·gres·sive in·fan·tile fi·bro·ma·to·sis
a childhood counterpart of abdominal or extraabdominal desmoid tumors, characterized by firm subcutaneous nodules that grow rapidly in any part of the body that invade locally and recur but do not metastasize.
Aggressive FibromatosisA common (50% of fibroproliferative lesions of musculoaponeuroses of childhood), slowly infiltrative, non-tender, non-metastasising, but locally aggressive myofibroblastic neoplasm of younger (mean age 40) adults that is the most common tumour of mesentery. It arises in the stomach, GI tract, or abdomen and may extend into liver, pancreas, or retroperitoneum, as well as in deep soft tissues (shoulder, pelvic girdle, chest wall, trunk, back, legs, head and neck, breast and spermatic cord) of children to young adults.
Clinical findings Obstruction of bowel, ureter, and blood vessels; fistula formation.
Management Surgery with wide margins, but not always possible or successful if attempted. Radiotherapy may or may not help. Chemotherapy (imatinib/Glivec/Gleevec) or endocrine therapy may or may not help
Prognosis Post-excisional recurrences range from 20–90%; successful therapy requires adequate margins. Young age at diagnosis, mesenteric location and Gardner’s syndrome carry a higher risk of recurrence.