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Extralobar pulmonary sequestration (EPS) is an unusual congenital defect characterized by the presence of non-functioning lung tissue receiving arterial supply from the systemic arteries. Primary hemangiopericytoma (HPC) is an uncommon potentially malignant tumor of vascular origin that usually involves the soft tissue of the extremities or retroperitoneum, but extremely rarely affects the lung. We present the rare case of a primary pulmonary HPC arising in an EPS.

Primary Hemangiopericytoma Arising in Extralobar Pulmonary Sequestration: A Coincidence or Two Rare Disorders?