Extralobar pulmonary sequestration as a cause of recurrent pulmonary hemorrhage in a neonate

Akdag, Arzu; Arici, Sule; Kaya, Mete; Turgut, Ahmet Tuncay

doi:10.5546/aap.2016.eng.e21

Cited by 1 publication

(1 citation statement)

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“…The characteristic position of the lesion and recurrent lung infections without significant radiographic resolution in childhood or young adulthood makes intrapulmonary sequestration a possible diagnosis. On the contrary, EPS due to lack of communication with the tracheobronchial tree may remain asymptomatic for many years or less frequently present in the neonatal period with more intense clinical manifestations such as respiratory failure, cyanosis, infections and/or recurrent pulmonary hemorrhage (8). Contrastenhanced helical CT scanning with 3-dimensional reconstruction can confirm the suspected diagnosis (9).…”

Section: Discussionmentioning

confidence: 99%

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

Kampolis

Damaskos

Loukeri

et al. 2019

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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.

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Section: Discussionmentioning

confidence: 99%