Pogoriler Jennifer, Vargas Sara O
Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia and Perelman School of Medicine, University of Pennsylvania, 3401 Civic Center Blvd, Philadelphia, PA, 19104, USA.
Department of Pathology, Boston Children's Hospital & Harvard Medical School, 300 Longwood Avenue, Boston, MA, 02115, USA.
Virchows Arch. 2025 Jan;486(1):177-188. doi: 10.1007/s00428-024-04019-9. Epub 2024 Dec 31.
Localized cystic lung lesions in pediatric patients encompass a spectrum of benign and rare malignant conditions that are quite distinct from cystic lung disease arising in adulthood. The majority have historically fallen under the diagnostic category of "congenital pulmonary airway malformation," a term that has been used to denote a diverse group of diseases ranging in etiology from ectopia to bronchial atresia to mosaic oncogenic mutation or neoplasia. This article reviews the clinical characteristics, gross and histologic features, and pathogenetic underpinnings of congenital pulmonary airway malformation as well as lesions that enter its histologic differential diagnosis. In light of ongoing advances in the field, previously proposed pathology-based classification schemes are critically appraised, and a new diagnostic framework is considered.
儿科患者的局限性肺囊性病变包括一系列良性和罕见的恶性疾病，这些疾病与成人期出现的囊性肺病截然不同。从历史上看，大多数病变都属于“先天性肺气道畸形”这一诊断类别，该术语一直用于表示病因多样的一组疾病，范围从异位到支气管闭锁，再到镶嵌性致癌突变或肿瘤形成。本文综述了先天性肺气道畸形的临床特征、大体和组织学特征、发病机制基础以及进入其组织学鉴别诊断的病变。鉴于该领域的不断进展，对先前提出的基于病理学的分类方案进行了批判性评估，并考虑了一个新的诊断框架。
