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Primary tumors of the great vessels are very rare. Primary leiomyosarcomas of the pulmonary vein are extremely rare and little is known about their clinical manifestation and treatment. We report the case of a 34-year-old patient with primary leiomyosarcoma of the pulmonary vein extending into the left atrium. A review of the clinical manifestation and treatment of 24 cases including our own is provided.
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