Leiomyosarcoma of the pulmonary veins

Primary sarcomas of the great vessels are rare, but the most common site is the inferior vena cava. Herein are reported five new cases arising from the pulmonary veins with clinicopathologic correlation and comparison to previously reported cases. All new cases occurred in women ranging in age from 23 to 64 years at diagnosis (mean, 56 years). They had symptoms suggestive of left heart failure, including three patients with dyspnea, one with hemoptysis, and one with cough. Three cases showed tumor extension along the pulmonary veins into the left atrium. Tumors ranged in size from 2.8 to 7 cm in greatest dimension. Histologically, all were leiomyosarcomas. They were highly cellular tumors. Three cases had predominantly spindle cell morphology and two were predominantly epithelioid; one had foci of calcification. Most showed extensive necrosis. All tumors were reactive with antibodies to actin and desmin. Two cases were reactive with antibodies to MIC-2 (dotlike); two cases showed reactivity to keratin antibodies; and two showed reactivity for estrogen, progesterone receptor protein, or both. None were positive for antibodies to S-100 protein. All cases were treated with surgical excision. Follow-up ranged from 2 months to 21 years (mean, 4.8 years). Two patients were alive and well; two were alive with metastases; and one died of disease. Pulmonary vein sarcomas represent intermediate- to high-grade leiomyosarcoma. Although often lethal, complete surgical excision can lead to long-term survival. They occur predominantly in women and may express hormone receptors. Therefore, hormonal manipulation may offer promise as adjuvant therapy.