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Home  List of Disease  Aggressive fibromatosis

Disease Name:Aggressive fibromatosis
Aggressive fibromatosis
Alternate Names:Desmoid Tumor
Virus Name:Aggressive fibromatosis is a locally invasive tumour caused by changes resulting in ?-catenin protein stabilisation. Apc1638N mice are predisposed to developing aggressive fibromatosis tumours, and male mice develop greater numbers of tumours than female mice, suggesting a role for androgens in this tumour type.
Treatment:Treatment may consist of watching and waiting, complete surgical removal, radiation therapy, antiestrogens and NSAIDs, or chemotherapy. Current experimental studies are being done with Gleevec for treatment of Desmoid tumors, and show promising success rates.
Description:Aggressive fibromatosis is a rare situation marked by the presence of desmoid tumors, which are benign, slow-growing tumors with no any metastatic potential. Despite their benign nature, they can damage nearby structures causing organ dysfunction. Most cases are sporadic, but some are associated with familial adenomatous polyposis . just about 10% of individuals with Gardner's syndrome, a type of FAP with extracolonic features, have desmoid tumors.

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