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 30’s, 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.
The most common symptom of desmoid tumors is pain. Other signs and symptoms, which are often caused by growth of the tumor into surrounding tissue, vary based on the size and location of the tumor. Intra-abdominal desmoid tumors can block the bowel, causing constipation. Extra-abdominal desmoid tumors can restrict the movement of affected joints and cause limping or difficulty moving the arms or legs.
Desmoid tumors are rare, affecting an estimated 1 to 2 per 500,000 people worldwide. In the United States, 900 to 1,500 new cases are diagnosed per year. Sporadic desmoid tumors are more common than those associated with familial adenomatous polyposis.
Desmoid tumors are usually named for the areas where they grow –
- Abdominal desmoid tumors—in the walls of the abdomen (belly)
- Intra-abdominal desmoid tumors—in tissue connecting abdominal organs
- Extra-abdominal desmoid tumors—anywhere else in the body
Causes
The exact cause of desmoid tumors is not known.Typically, tumors develop in just one area, although sometimes they may appear in several locations. Multiple locations of disease can be associated with familial adenomatous polyposis, a genetic abnormality in which potentially malignant polyps develop in the intestinal lining.
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.
This supports the theory that desmoids may have a genetic cause. Other theories have been explored, as well—including hormonal and traumatic causes—with no definitive conclusions.
Symptoms
Desmoid tumors can develop at virtually any site in the body. Superficial desmoids tend to be less aggressive than desmoids found deep inside the body (i.e., abdominal, extra-abdominal, mesenteric). Superficial desmoid tumors usually manifest themselves as a painless or slightly painful lump. Desmoids inside the abdomen can cause severe pain, rupture of intestines, compression of the kidneys or ureters or rectal bleeding. They can compress critical blood vessels such as the mesenteric vessels and the vena cava. Desmoid tumors may have multiple sites of origin on chest, arms or legs.
Because Desmoid tumors affect tissue that is elastic and easily moved, a tumor may exist for a long time before being discovered, growing large and pushing aside surrounding tissue.
Each child may experience symptoms differently, but here’s a list of the most common symptoms of Desmoid tumors –
- A painless swelling or lump
- Pain or soreness caused by compressed nerves or muscles
- Pain and obstruction of the bowels
- Limping or other difficulty using the legs, feet, arms or hands
Treatment
Radiation – Radiation therapy can destroy cancer cells and shrink tumors. In some cases, desmoid tumors may be treated successfully with radiation therapy alone. In other cases, radiation therapy is used in conjunction with surgery. Radiation treatments usually begin two weeks after the surgical procedure.
Hormone therapy – Desmoids are known to have estrogen receptors. This means that their growth may be stimulated by the hormone estrogen. Drugs that block the hormonal stimulation of the tumor are sometimes effective at controlling the tumor growth.
Nonsteroidal anti-inflammatory drugs (NSAIDs) – Medications such as ibuprofen and naproxen, as well as a special kind of NSAID called a COX-II inhibitor, can be effective in treating desmoid tumors.
Chemotherapy – Low doses of chemotherapy drugs are effective in management of desmoids. Long-term use of these drugs is difficult, however, due to their cumulative effects in the body.
Other nonsurgical treatments – Radiofrequency ablation, in which the tumor is heated with a high-frequency electrical current, and direct injection of the tumor with irritating chemicals such as acetic acid have also shown some promise in treating desmoid tumors.
Surgery to remove the tumor is the preferred method of treatment. However, it can be difficult during surgery to determine the outer extent of the tumor. This is because desmoids are not well encapsulated and they often intertwine with other tissues. A tumor may recur after surgery with local rates of recurrence ranging from 25% to 68%.
Alternative Treatment
Reference –
http://www.webmd.com/cancer/desmoid-tumor
http://www.orpha.net/consor4.01/www/cgi-bin/OC_Exp.php?lng=EN&Expert=873
http://radiopaedia.org/articles/desmoid-tumour
http://atlasgeneticsoncology.org/Tumors/DesmoidFibromatosisID5179.html
http://atlasgeneticsoncology.org/Tumors/DesmoidFibromatosisID5179.html
http://ghr.nlm.nih.gov/condition/desmoid-tumor
http://jco.ascopubs.org/content/17/1/158.full
http://www2.mdanderson.org/depts/oncolog/articles/10/11-12-novdec/11-12-10-1.html
http://www.dtrf.org/index.php/about-br-desmoid-tumors/about-desmoid-tumors.html
http://emedicine.medscape.com/article/1060887-overview
http://ghr.nlm.nih.gov/condition/desmoid-tumor