Chronic lymphedema due to surgery, radiation, or parasitic infection can lead to lymphangiosarcoma of the extremity. The most common clinical setting is the postmastectomy patient with lymphangiosarcoma of the ipsilateral upper extremity (Steward-Treves syndrome). While postoperative lymphedema alone can lead to lym-phangiosarcoma, it is much less common than in patients with lymphedema and a history of prior radiation exposure. Modern surgical and radiotherapeutic techniques have led to a decreased incidence of severe lymphedema of the extremity and subsequently, a decreased incidence of the disease.

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