Definition:
Desmoid tumor is a tendon-like benign fibrous neoplasms. It originates from the musculoaponeurotic structures found in the entire body. It appears as firm overgrowths of tissues, usually fibrous, locally aggressive, well differentiated and infiltrative. Other terms used for this disorder are deep fibromatosis, nonmentastasizing fibrosarcoma or aggressive fibromatosis. It mostly grows from the abdomen and rectus muscle after giving birth or in scars from cuts during abdominal surgery. These tumors may also grow in bone muscles.
Diagnosis:
Differential diagnosis of desmoid tumors with the use of immunostaining using vimentin, muscle actin, desmin, and alpha smooth muscle actin to distinguish the tumors is used. A CT scan and MRI is also used to diagnose and also follow-up the tumors. These methods allow the examining physician to know the extent of the tumor and how it is related to nearby structures. MRI defines the pattern and also to examine if there is a recurrence of the tumor.
Treatment:
Complete surgery with negative margins to remove desmoid tumors is the most effective treatment for this disease as positive margins have high risk or recurrence. Radiation therapy may be helpful to treat recurrence. Other treatments also include administration of anti-estrogens and chemotherapy.
Symptoms and Signs:
Often times, Desmoid tumors grow in areas where there are tissues that form the tendons and ligaments like in the arms, abdomen, and legs and in the chest in some cases. It is usually soft sarcoma and does not spread to other parts of the body.
Causes:
Although there is not a known cause of this disease at present, studies associate this to trauma or hormonal factors such as estrogen or it may also be genetic in nature as some tend to occur in people who have history of polyposis coli.
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