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Aggressive Fibromatosis

Definition


Definition of Aggressive Fibromatosis
Aggressive fibromatosis is a rare condition marked by the presence of desmoid tumors, which are benign, slow-growing tumors without any metastatic potential. However, aggressive fibromatosis is locally aggressive. Despite their benign nature, they can damage nearby structures causing organ dysfunction. Most cases are sporadic, but some are associated with familial adenomatous polyposis (FAP). Approximately 10% of individuals with Gardner's syndrome, a type of FAP with extracolonic features, have desmoid tumors.

Symptoms


Symptoms of Aggressive Fibromatosis
The list of signs and symptoms for Aggressive fibromatosis includes the 2 symptoms listed below:

  1. Firm mass
  2. Compression of adjacent structures

Subtypes


Subtypes of Aggressive Fibromatosis
The list below shows the known subtypes of Aggressive fibromatosis: juvenile fibromatosis fibromatosis colli infantile/immature digital fibromatosis infantile/immature myofibromatosis ipofibromatosis fibromatosis hyalinica multiplex plantar fibromatosis penile fibromatosis (Peyronie's disease) palmar fibromatosis (Dupuytren's contracture).

Diagnosis


Diagnosis of Aggressive Fibromatosis
Many times, this condition is diagnosed through the use of routine diagnostic tests or after symptoms begin to develop.

Treatment


Treatment of Aggressive Fibromatosis
Treatment may consist of watching and waiting, complete surgical removal, radiation therapy, antiestrogens and NSAIDs, or chemotherapy.

A biopsy is always indicated as the de?nitive method to determine nature of the tumour. Management of these lesions is complex, the main problem being the high rates of recurrence. Wide surgical resection with clear margins is the most widely practiced technique with radiation, chemotherapy, or hormonal therapy being used to reduce recurrence rates.

Complications


Complications of Aggressive Fibromatosis
Complications and sequelae of Aggressive fibromatosis from the Diseases Database include:

  • Subcutaneous nodules


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