CASE AND RESEARCH LETTERS Angiosarcoma in Chronic Lymphedema (Stewart-Treves Syndrome) Angiosarcoma en linfedema crónico (síndrome de Stewart-Treves)

Angiosarcoma is a malignant endothelial tumor that can form in any part of the body, including the skin. It is known as hemangiosarcoma or lymphangiosarcoma, depending on whether it derives from the endothelium of the blood vessels or the lymph vessels, respectively. It was first described in 1948 by Stewart and Treves in a series of 6 cases of lymphangiosarcoma after chronic postmastectomy lymphedema; since then, more than 400 cases have been reported of angiosarcoma associated with chronic lymphedema at different sites. We report the case of a patient with angiosarcoma of the right arm, associated with chronic lymphedema due to non-Hodgkin lymphoma. The only relevant history reported by the 84-year-old woman was a nodule in the lower right cervical region; results of a fine-needle aspiration biopsy of the nodule were negative for malignancy. Because of the growth of the nodule, a new fine-needle aspiration procedure was performed a year later and cytology results indicated suspected lymphoma; resection and biopsy were performed and a diagnosis of non-Hodgkin large B cell (CD20) lymphoma was established. Computed tomography (CT) revealed several bilateral enlarged axillary lymph nodes; the largest of these, measuring 2.5-3 cm, was on the right side and in contact with the rib. The CT scan also revealed multiple enlarged hilar, mediastinal, para-aortic, and retroesophageal lymph nodes, and a 4.3-cm solid mass in the anterior segment of the right upper lobe of the lung. An atypical segment resection of the right upper lobe and a mediastinal lymphadenectomy were performed, followed by adjuvant chemotherapy with cyclophosphamide, epirubicin, vincristine, and prednisone (6 cycles). Treatment