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

Abstract

Background/Aim: 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. Case Report: A 65-yearold woman, with dyspnea and pleuritic chest pain, was referred for further investigation. Radiological evaluation demonstrated a well-circumscribed mass above the right hemidiaphragm, receiving its arterial supply from the descending thoracic aorta. The patient underwent a right posterolateral thoracotomy and a middle lobectomy. The intraoperative finding was a well-encapsulated solid mass. The histological evaluation described HPC. Results: The patient remains fit and healthy. Conclusion: Pulmonary HPC can arise in EPS. Surgical excision is the treatment of choice. Extralobar pulmonary sequestration (EPS) is an unusual congenital defect characterized by the presence of nonfunctioning lung tissue that receives its arterial supply from the systemic arteries, most commonly the thoracic aorta. It is separated from the bronchial tree and has its own pleural covering. Primary hemangiopericytoma (HPC) is an uncommon potentially malignant tumor of vascular origin that usually involves the soft tissue of extremities or retroperitoneum, but extremely rarely affects the lung. We herein present the interesting case of a 65-year-old female patient with primary pulmonary HPC arising in an EPS, thereby implying a possible association which has not been previously reported.

The clinical management of extralobar pulmonary sequestration in children

“…Choe et al 15 reported ELS torsion occurred in a 10‐year‐old boy with hemorrhagic infarction led to acute abdominal pain and fever. The Christos group confirmed that primary pulmonary hemangiopericytoma may arise in ELS 3 . Thus, the conservative treatment of ELS has potential risks to some extent.…”

“…ILS shares visceral pleura with the adjacent normal lung lobe. In contrast, ELS has its own visceral pleura outside the normal lung, which accounts for approximately 14%–25% of PS 1–3 . The majority of ELS are located in the thoracic cavity, especially near the lower lung lobes and spine.…”