What are Desmoid Tumors? Desmoid tumors are tumors that arise from cells called fibroblasts. Fibroblasts are found throughout our body and their main function is to provide structural support and protection to the vital organs such as lung, liver, blood vessels, heart, kidneys, skin, intestines etc. and they also play a critical role in wound healing. When fibroblast cells undergo mutations they can become cancerous and become desmoid tumors (also known as aggressive fibromatosis). Desmoid tumors can arise in virtually any part of the body. These tumors often occur in women in their 30s, but can occur in anyone at any age. Desmoid tumors can be slow growing or extremely aggressive. They do not metastasize (move from one body part to another), and if slow growing they can be carefully watched by your physician. However, when they are aggressive they can cause life threatening problems or even death when they compress vital organs such as intestines, kidney, lungs, blood vessels, nerves etc. Desmoid Tumors Are Rare! In the United States, approximately 900 people are diagnosed with desmoid tumors every year. This means that out of a million people approximately 2 - 4 people are diagnosed with desmoid tumors each year. Experts believe that the numbers are likely to be far greater because of the difficulty in correctly diagnosing this disease. Because of inconsistent and inaccurate reporting procedures, accurate statistics about the number of desmoid tumor cases have not been kept. Individuals between the ages of 15 and 60 are most often affected, but this disease can occur in anyone. The average age is 30s to 40s. They are slightly more common in women than in men (2:1), and there is no significant racial or ethnic distribution. Causes of Desmoid Tumors The cause of most desmoid tumors is unknown and thus they are called sporadic. Most desmoids have mutations in a gene called beta catenin. A minority of desmoid tumors are caused by mutations in a gene called Familial Adenomatous Polyposis or FAP. Patients with mutations in the FAP gene are predisposed to forming hundreds of polyps in the intestines and go on to develop colon cancers. Patients with FAP are often recommended to undergo surgical removal of their intestines. These patients are at a very high risk of developing desmoid tumors which can arise deep in the abdomen or in the abdominal wall. In older scientific literature, the combination of FAP and desmoid tumors is termed Gardners Syndrome. In some rare cases, desmoid tumors can occur in women who are pregnant. This happens during pregnancy or after a surgical delivery. Many believe that this is caused by a combination of elevated hormones and surgery, however, these is no strong scientific evidence to support this claim. The relationship between pregnancy and desmoid tumors is very rare and consists of mostly anecdotes in the scientific literature.
Posted on: Mon, 07 Jul 2014 02:31:02 +0000
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