Aggressive Fibromatosis

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Aggressive Fibromatosis

A 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.
References in periodicals archive ?
Brandi, "Treatment of desmoids and mesenteric fibromatosis in familial adenomatous polyposis with raloxifene," Tumori Journal, vol.
Stromal tumors like leiomyomas, leiomyosarcomas, desmoid tumors, mesenteric fibromatosis and fibrosarcomas are considered in differential diagnosis.
Melorheostosis with associated minimal change nephritic syndrome, mesenteric fibromatosis and capillary haemangiomas.
Grigoraki et al., "Isolated giant mesenteric fibromatosis (intra-abdominal desmoid tumors).
Malignant melanomas, synovial sarcomas, mesenteric fibromatosis (desmoid tumors), and schwannomas have been reported as positive for this marker.
Mesenteric fibromatosis most commonly arises in the mesentery and omentum of middle-aged adults but can subsequently involve the wall and/or lumen of the gut, mimicking a GIST.

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