Mukherjee Raktim, Das Oindrila, Chakravorty Subhankar, Mohanty Suravi, Chatterjee Uttara
Department of Pathology, Institute of Postgraduate Medical Education & Research (IPGME&R), Kolkata, India.
Department of Neonatology, Institute of Postgraduate Medical Education & Research (IPGME&R), Kolkata, India.
Fetal Pediatr Pathol. 2023 Apr;42(2):310-314. doi: 10.1080/15513815.2022.2094513. Epub 2022 Jul 7.
Extralobar pulmonary sequestration (ELS) is a malformation composed of bronchopulmonary tissue outside the lungs that is discontinuous from the main tracheobronchial tree. ELS can present as a suprarenal space occupying lesion. A 1-day old girl presented with a right supra-adrenal mass, first detected in-utero at 34 weeks. The differential included congenital neuroblastoma, but the urinary VMA was normal. At resection, there was lung tissue composed of dilated and tortuous bronchioles lined by columnar epithelium present in a back- to- back arrangement along with thick-walled vessels, features of an ELS with congenital pulmonary airway malformation type II. ELS enters the differential diagnosis of neonatal suprarenal masses with normal urinary catecholamines, and can have the morphology of a type II congenital pulmonary airway malformation.
肺叶外型肺隔离症（ELS）是一种由肺外支气管肺组织构成的畸形，与主气管支气管树不连续。ELS可表现为肾上腺区占位性病变。一名1日龄女婴因右肾上腺区肿块就诊，该肿块在孕34周时于宫内首次被发现。鉴别诊断包括先天性神经母细胞瘤，但尿香草扁桃酸（VMA）正常。手术切除时，发现有由柱状上皮衬里的扩张和迂曲细支气管组成的肺组织，呈背靠背排列，伴有厚壁血管，这是具有II型先天性肺气道畸形特征的ELS表现。ELS应列入尿儿茶酚胺正常的新生儿肾上腺肿块的鉴别诊断，且可具有II型先天性肺气道畸形的形态。
