Lymphedema-related malignant vascular tumors may occur:
(1) following mastectomy for breast cancer
(2) following surgery for malignant melanoma
(3) in a patient with filariasis
(4) in a patient with congenital lymphedema
(5) in a patient with chronic idiopathic lymphedema
Risk factors:
(1) chronic lymphedema, typically for many years (over 10 years)
(2) extensive axillary lymph node dissection
(3) axillary radiation
Clinical findings:
(1) Skin lesions appear in the lymphedematous arm, most often on the medial aspect.
(2) These are purplish-red and may be macules, plaques or nodules. They often are multifocal or may show satellite lesions. The lesions may ulcerate as they become larger.
(3) The lesions may be painful.
(4) Telangiectasias may be present in adjacent skin.
(5) The lesions often grow rapidly with infiltration of the subcutaneous skin.
(6) Metastases may be present at the time of diagnosis.
Early and aggressive therapy is associated with improved outcomes. Delay or inadequate therapy results in a poor long-term survival.
