Also known as Aggressive fibromatosis
ABOUT THE DISEASE
Desmoid tumor, a rare type of soft tissue neoplasm, commonly develops in the fibrous (connective) tissue of the body that forms tendons and ligaments, usually in the arms, legs or midsection, and also in the head and neck. Sometimes they are called aggressive fibromatosis due to its similarities with a malignant tumor called fibrosarcoma. However, it is considered benign because it does not metastasize (spread) to other parts of the body. Although they typically don`t spread to other parts of the body, they can quickly invade nearby tissues and organs.
CAUSES
The myofibroblast is the cell considered to be responsible for the development of Desmoid tumor and usually very difficult to remove because they intertwine extensively with the surrounding tissues. Desmoid tumors sometimes occur in people who have a family history of colon cancer.
SYMPTOMS
- Firm, mobile, soft tissue growth, with normal overlying skin; may appear on leg, foot or breast
- Intra abdominal masses may produce late symptoms due to compression of surrounding organs
DIAGNOSIS
These tumors look like dense scar tissue adhering tenaciously to surrounding structures and organs. Although desmoid tumors can arise in any skeletal muscle, they most commonly develop in the anterior abdominal wall and shoulder girdle.
Immunostaining with vimentin, alpha smooth muscle actin, muscle actin, and desmin are helpful in differential diagnosis of desmoid tumors.
TREATMENT METHODS
Surgery with negative surgical margins is the main mode of therapy. Up to 70% of these tumors recur after surgery. In patients where surgery is not possible Radiotherapy or Pharmacologic therapy with chemotherapeutic agents, antiestrogens and prostaglandin inhibitors may also be used.
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