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 ?
Morris et al., "External beam radiotherapy for primary and adjuvant management of aggressive fibromatosis," International Journal of Radiation Oncology Biology Physics, vol.
van Laarhoven et al., "Aggressive fibromatosis in the head and neck region: benign tumor with often mutilating effects," Head and Neck, vol.
Vaidya, "Aggressive fibromatosis (desmoid tumour) of the head and neck: a benign neoplasm with high recurrence," BMJ Case Reports, vol.
Aggressive fibromatosis is a diagnostic dilemma which remains in grey zone between benign and malignant lesions.
The role of APC and beta-catenin in the aetiology of aggressive fibromatosis (desmoid tumors) European Journal of Surgical Oncology.
Patho-mechanism Diagnoses Congenital and/or Familial adenomatous polyposis or genetic Gardner's syndromedesmoid tumours (= aggressive fibromatosis) Infective and/or Abscess inflammatory Myositis Neoplastic Metastatic disease Granular cell tumour Lymphoma Sarcomas Neurofibromas and nerve sheath tumours Traumatic and/or Haematoma vascular Varices Pseudo-aneurysm of inferior epigastric artery Iatrogenic Injection granuloma or suture granuloma Surgical scar endometriosis Incisional hernia TABLE 2: MRI features of the major differential diagnoses.
The tumour demonstrated histological features of aggressive fibromatosis. After histopathological confirmation of the disease, the patient received chemotherapy followed by surgical removal of the remnant mass.
Treatment of aggressive fibromatosis: the experience of a single institution.
Aggressive fibromatosis of the neck initiated after thyroidectomy.
Desmoid tumors (also known as desmoid-type fibromatosis or aggressive fibromatosis) can arise anywhere in the body and are locally aggressive, leading to significant morbidity due to their large size and pain caused by compression and infiltration of surrounding nerves and normal structures [1].
DISCUSSION: Desmoid tumor, also known as aggressive fibromatosis, (5) is a rare tumor.

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