Intimal sarcoma of the pulmonary artery is a rare and highly malignant neoplasm. This report describes a case of a 30-year-old woman with an extensive right pulmonary artery tumor who underwent an emergency operation. The tumor was aggressively resected with right pneumonectomy and reconstruction of the right ventricle outflow tract and left pulmonary artery. Although the resected margin at the left pulmonary artery was positive for disease, as confirmed by mouse double minute type 2 homologue staining, she is doing well and remains free of relapse at 16 months after the operation.
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