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Primary pulmonary intimal sarcoma is rare. Differentiating it from pulmonary thromboembolism is difficult because of similarities in clinical symptoms and imaging findings. Positron-emission tomography-computed tomography has been useful for diagnosing primary pulmonary intimal sarcoma. We describe a rare case of primary pulmonary intimal sarcoma that showed no abnormal 18F-fluorodeoxyglucose uptake on positron-emission tomography. We resected the mass and performed right ventricular outflow tract reconstruction. Proper diagnosis is necessary to determine appropriate therapy, Clinicians must consider the possibility of primary pulmonary intimal sarcoma even if imaging findings are inconsistent with the disease.

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