Wednesday, August 29, 2012

Desmoid Tumours: About The Rare Condition

What exactly are desmoid tumours?

Aggressive fibromatosis is a rare condition marked by the presence of desmoid tumors, which are benign, slow-growing tumours 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

The growths form from fibroblasts, a type of cell that helps heal wounds and protects the lung, liver, blood vessels, heart, kidneys and other vital organs, according to the Desmoid Tumour Research Foundation.

The prognosis for desmoid tumours depends on how aggressively they grow and whether the growths compress organs such as the intestines, kidneys and lungs or blood vessels or nerves.

More aggressive desmoids can be fatal, according to the foundation.

Unlike some cancers, desmoid tumors do not metastatize, meaning they don't spread from organ to organ or through the blood.

Desmoid tumors are extremely rare, with 900 people diagnosed with the condition each year, according to the foundation, although more people may have the condition due to difficulties diagnosing the growths.

Anyone can become afflicted with desmoid tumors, but they commonly occur in people in their 30s and 40s. Women are twice as likely to be diagnosed with desmoid tumours as men, according to the foundation.

There may also be a link between desmoid tumours and people who have a family history of colon cancer, according to the Mayo Clinic.

The growths are diagnosed through a biopsy and are treated either through surgery (as in Rounds' case), chemotherapy, radiation, medications and hormone therapy.

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