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Desmoid fibromatosis

Desmoid fibromatosis, also known as desmoid tumours or aggressive fibromatosis, is a rare type of soft tissue tumour that arises from fibroblasts, which are cells that produce the connective tissue in the body. These tumours are locally aggressive, meaning they have the potential to grow and invade nearby tissues but do not metastasize to other parts of the body.

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Desmoid fibromatosis tumours can occur in various parts of the body, including the abdominal wall, intra-abdominal region, extremities, and other soft tissue areas. They are categorized as intermediate-grade tumours between benign and malignant due to their invasive nature.

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Symptoms of desmoid fibromatosis can vary depending on the location and size of the tumour. Common symptoms may include a palpable mass, pain, limited range of motion, and compression of nearby structures.

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Treatment options for desmoid fibromatosis depend on several factors, such as the size and location of the tumour, symptoms, and individual patient considerations. Treatment may include observation with regular monitoring, surgery to remove the tumour, radiation therapy, targeted therapy, or systemic therapy. A multidisciplinary approach involving oncologists, surgeons, and other healthcare professionals is often employed to develop a comprehensive treatment plan based on the specific needs of the patient.

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Due to the locally aggressive nature of desmoid fibromatosis, ongoing monitoring and follow-up care are important to detect any recurrence or progression of the tumour. It is essential for individuals with desmoid fibromatosis to work closely with a healthcare team specialized in managing this rare condition.

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