Review Question - QID 103800

Axillary lymph node dissection is associated with development of chronic lymph edema, an important risk factor for developing angiosarcoma or lymphangiosarcoma.

Lymphangiosarcoma is a rare, malignant tumor arising from or resembling constituents of blood vessels. Risk factors for the development of lymphangiosarcoma include radiation, chronic lymphedema, inflammation and immunosuppression. Overall prognosis for angiosarcoma is poor, with a reported 5-year survival rate of 10-35%.

O'Brien et al., in a review of the treatment of lymphedema, note that lymphedema after axillary node dissection affects up to 80% of patients. Chronic lymphedema with resultant inflammation can predispose patients to the development of angiosarcoma. There is hope that the advent of breast-conserving surgery and sentinel lymph-node biopsy will reduce the incidence of these adverse events.

Peinemann et al., in a Cochrane review, reported that overall survival after high-dose chemotherapy (HDCT) followed by autologous hematopoietic stem cell transplant is not statistically significantly different from standard-dose chemotherapy for treatment of sarcoma. The authors recommend against these high-burden treatments.

Illustration A shows the contrast between an normal arm and one affected by lymphedema secondary to axillary dissection.
Illustration B shows an example of lymphangiosarcoma, in this case resulting from long-standing primary lymphedema.

Incorrect Answers:
Answer 1: The BRCA gene produces a protein responsible for repairing damaged DNA. While inheriting this gene leads to increased risk for developing breast cancer, it is not associated with angiosarcoma.
Answer 2: While there is an association between environmental exposures such as vinyl chloride (used in production of synthetic rubber) and arsenic (used in pesticides) with the development of angiosarcoma, asbestos exposure is not a known risk factor.
Answer 3: There is no known association between chemotherapy and development of angiosarcoma.
Answer 5: While there is an association between certain preexisting conditions (includin neurofibromatosis, Maffucci syndrome, Rb1 deletion) and angiosarcoma, the association is much weaker than that shown for radiation therapy.