Aggressive Fibromatosis

(redirected from Extra-Abdominal Fibromatosis)

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.
Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved.
References in periodicals archive ?
The principal sites of involvement for extra-abdominal fibromatosis are the shoulder, chest wall and back, thigh and head and neck region.
High frequency of beta-catenin heterozygous mutations in extra-abdominal fibromatosis: a potential molecular tool for disease management.
In the head and neck region, AF accounts for 7%-25% of all cases of extra-abdominal fibromatosis (1).
Histology showed the lesion to have the morphologic aspects of extra-abdominal fibromatosis.
Lacroix et al., "High frequency of beta-catenin heterozygous mutations in extra-abdominal fibromatosis: a potential molecular tool for disease management," British Journal of Cancer, vol.
Clinically, the extra-abdominal fibromatosis appears as a deep-seated and circumscribed mass growing insidiously, often with no pain [6] and a variable clinical course [9].
Extra-abdominal fibromatosis (extra-abdominal desmoid fibromatosis).
According to the anatomical site, desmoid-type fibromatosis can be divided into (i) extra-abdominal fibromatosis (60%); (ii) fibromatosis of the abdominal wall (25%); (iii) intra-abdominal fibromatosis (8-15%).
Head and neck lesions are more aggressive than extra-abdominal fibromatosis elsewhere and are capable of massive destruction of adjacent bone, erosion of base of skull and occasional encroach on the trachea, sometimes with fatal outcome [2, 14]
Histology confirmed a diagnosis of extra-abdominal fibromatosis. Two unusual features of this case were the site of the lesion and its rapid growth.
In this article, we report a case of extra-abdominal fibromatosis that was unusual not only in terms of its site, but also because of its rapid growth.