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Sarcoma originating from the pulmonary veins represents one of the rarest subtypes of sarcoma. We report the case of a 52-year-old woman presenting with cough and scant hemoptysis and ultimately diagnosed with a primary intimal sarcoma of the left inferior pulmonary vein extending into the left lower lobe of the lung. To our knowledge, this is the first reported case of such pathology in the literature. We present the case and critically appraise the literature for sarcomas of the great vessels.

Primary Intimal Sarcoma of the Pulmonary Vein with Extension into the Left Atrium: A Case Report