Aggressive Fibromatosis

(redirected from Intra-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 ?
Intra-abdominal fibromatosis arises in the mesentery or pelvis while abdominal tumours arise from musculo-aponeurotic structures of the abdominal wall, especially the rectus and internal oblique muscles and their fascial coverings.
However, intra-abdominal fibromatosis shows no gender difference or age predilection.
The recurrence rate is 15-30% for intra-abdominal fibromatosis. Therefore, completeness of resection is an important prognostic factor.
Helwig, "Intra-abdominal fibromatosis. A pathologic analysis of 130 tumors with comparison of clinical subgroups," The American Journal of Surgical Pathology, vol.
Hou et al., "Subsequent intra-abdominal fibromatosis mimicking recurrent gastrointestinal stromal tumor," Diagnostic Pathology, vol.
[11] The other differential includes intra-abdominal fibromatosis, inflammatory fibroid polyp, paragangliomas, and metastatic malignant melanoma.
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%).
Intra-abdominal fibromatosis can remain silent or it can appear in association with a fistula, abdominal pain, small-bowel obstruction, ureteric obstruction, and, in rare cases, bowel perforation.
Schwannomas, Lymphoma, Intra-Abdominal Fibromatosis, Inflammatory Fibroid Polyps, Paragangliomas, Carcinoid Tumours, Sarcomas and Adenocarcinomas.
These abnormal characteristics have been limited to use in pathogenesis and are currently now used as diagnostic characteristic adjuncts [2] The differential diagnosis for GISTs currently include intra-abdominal fibromatosis, inflammatory fibroid polyp, paragangliomas and metastatic malignant melanoma.