Lymphangiosarcoma

Lymphangiosarcoma is a rare form of cancer that occurs in long-standing cases of primary or secondary lymphedema. It is also known as Stewart-Treves syndrome, particularly when it is associated with lymphedema following mastectomy for breast cancer.

Etiology

The exact cause of lymphangiosarcoma is unknown, but it is often associated with chronic lymphedema. This condition, which involves swelling due to a blockage in the lymphatic system, can occur after surgery for breast cancer, particularly mastectomy with removal of axillary lymph nodes. The association between lymphedema and lymphangiosarcoma is known as Stewart-Treves syndrome.

Symptoms

Symptoms of lymphangiosarcoma may include a red or purple patch of skin that gradually enlarges and may eventually become a raised nodule. The lesion is often painless, but may be associated with a heavy or aching sensation. Other symptoms may include fever, weight loss, and general malaise.

Diagnosis

Diagnosis of lymphangiosarcoma is often challenging due to its rarity and nonspecific symptoms. It is typically confirmed through a biopsy of the lesion. Imaging studies such as MRI or CT scan may also be used to assess the extent of the disease.

Treatment

Treatment of lymphangiosarcoma typically involves surgery to remove the tumor, often followed by chemotherapy or radiation therapy. However, the prognosis is generally poor due to the aggressive nature of the disease and its tendency to recur or metastasize.

See also

• Lymphedema
• Stewart-Treves syndrome
• Lymphatic system
• Cancer

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