Leiomyosarcoma of the pulmonary veins

Primary sarcomas of the gnat vessels are rare, but the most common sire is the inferior vena cava. Herein are reported five new cases arising from the pulmonary veins with clinicopathologic correlation and comparison to previ ously reported cases. All new cases occurred in women ranging in age from 2 3 to 64 years at diagnosis (mean, 56 years). They had symptoms sugggestive of left heart failure, including three patients with dyspnea, one with hemo ptysis, and one with cough. Three cases showed tumor extension along th pul monary veins into thp left atrium. Tumors ranged in size from 2.8 to 7 cm i n greatest dimension. Histologically, all were leiomyosarcomas. They were h ighly cellular tumors. Three cases had predominantly spindle cell morpholog y and two were predominantly epithelioid; one had foci of calcification. Mo st showed extensive necrosis. All tumors were reactive with antibodies to a ctin and desmin. Two cases were reactive with antibodies to MIC-2 (dotlike) ; two cases showed reactivity to keratin antibodies; and two showed reactiv ity for estrogen, progesterone receptor protein, or both. None were positiv e for antibodies to S-100 protein. All cases were treated with surgical exc ision. Follow-up ranged from 2 months to 21 years (mean, 4.8 years). Two pa tients were alive and well; two were alive with metastases; and one died of disease. Pulmonary vein sarcomas represent intermediate- to high-grade lei omyosarcoma. Although often lethal, complete surgical excision can lead to long-term survival. They occur predominantly in women and may express hormo ne receptors. Therefore, hormonal manipulation may offer promise as adjuvan t therapy.