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My Child Has:
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Desmoid Tumor
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Desmoid tumor, also called aggressive fibromatosis, is a tumor that develops in the fibrous tissue that forms tendons and ligaments, usually in the arms, legs or midsection, but also sometimes in the head and neck. Locally, a Desmoid tumor is very similar to a malignant (cancerous) tumor called fibrosarcoma in that local recurrence is very high. However, a Desmoid tumor is considered benign because it does not metastasize (spread) to other parts of the body.
Regardless of its scientific classification, a Desmoid tumor can be invasive to surrounding tissues and difficult to control. It can adhere to and intertwine with surrounding structures and organs.
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The exact cause of Desmoid tumor remains unknown. Researchers have found that Desmoid tumors may occur in patients who have a history of polyposis coli, a hereditary colon cancer syndrome. Often there is a history of repetitive trauma.
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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. The following are the most common symptoms of Desmoid tumors. However, each child may experience symptoms differently. The symptoms of Desmoid tumors vary greatly depending on size, location, and spread of the tumor. Symptoms may include:
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- 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
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The symptoms of Desmoid tumors may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis.
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In addition to a complete medical and physical examination, the most conclusive diagnostic procedure for Desmoid tumor is a biopsy, a tissue sample from the tumor taken through a simple surgical procedure.
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Specific treatment for Desmoid tumor will be determined by your child's physician based on:
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- your child's age, overall health, and medical history
- extent of the disease
- your child's tolerance for specific medications, procedures, or therapies
- how your child's doctor expects the disease may progress
- your opinion or preference
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Treatment may include:
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- surgery - surgical removal of the tumor by a surgeon is the primary treatment; because Desmoid tumor rarely metastasizes, often surgery alone is the only treatment. There is a high recurrence rate with Desmoid tumor, so often more than one surgery is needed.
- anti-inflammatory medication - given to help manage pain and swelling. In some cases these medications will cause the tumor to slowly shrink.
- radiation therapy - using high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors; used alone or in conjunction with surgery
- hormone therapy - because some hormones seem to increase the growth of Desmoid tumors, anti-hormonal medications are sometimes used in treatment
- chemotherapy - if surgeons are unable to remove the tumor because of the size or location, researchers are testing some types of chemotherapy to reduce the tumor
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Children's Hospital and Dana-Farber Cancer Institute are conducting numerous research studies that will help clinicians better understand and treat Desmoid tumors.
Types of treatment currently being studied include:
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- angiogenesis inhibitors - substances that may be able to prevent the growth of tumors by blocking the formation of new blood vessels that feed the tumors
- chemotherapy agents - researchers are testing which chemotherapy drugs, or combination of drugs, is most effective in treating Desmoid tumors
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