文献类型：期刊文献

英文题名：Case Report: Diffuse pulmonary lymphangiomatosis in a child

作者：Zhang, Ya-nan[1,2];Yuan, Zhen[1];Du, Jin-chen[1];Liu, Bin[1,2];Li, Fu[1,2];Hu, Shou-Cai[1,2];Li, Qing-Xin[1]

第一作者：Zhang, Ya-nan;张亚男

通信作者：Li, QX[1]

机构：[1]940th Hosp Joint Logist Support Force Chinese Peop, Dept Thorac Surg, Lanzhou, Gansu, Peoples R China;[2]Gansu Univ Tradit Chinese Med, Clin Dept 1, Lanzhou, Gansu, Peoples R China

第一机构：940th Hosp Joint Logist Support Force Chinese Peop, Dept Thorac Surg, Lanzhou, Gansu, Peoples R China

通信机构：[1]corresponding author), 940th Hosp Joint Logist Support Force Chinese Peop, Dept Thorac Surg, Lanzhou, Gansu, Peoples R China.

年份：2026

卷号：13

外文期刊名：FRONTIERS IN PEDIATRICS

收录：;Scopus(收录号：2-s2.0-105027839879);WOS:【SCI-EXPANDED(收录号:WOS:001663682800001)】；

基金：The author(s) declared that financial support was received for this work and/or its publication. This work was supported by the Gansu Natural Science Foundation of China (Grant No. 24JRRA006).

语种：英文

外文关键词：diffuse pulmonary lymphangiomatosis (DPL); generalized lymphatic anomaly (GLA); pediatric case; diagnostic imaging; histopathology; mTOR inhibitors

摘要：We report a rare case of generalized lymphatic anomaly (GLA) in a 12-year-old male, presenting with intermittent cough, expectoration, and hemoptysis exacerbated by strenuous activity. Key features included bilateral pleural/pericardial effusions; chest/abdominal CT showed diffuse mediastinal infiltration, bronchovascular/interlobular septal thickening, and extrathoracic extension to periaortic abdominal tissues. Definitive diagnosis was confirmed by thoracoscopic biopsy and immunohistochemistry (CD31+, D2-40+, Ki-67 approximate to 3%), differentiating it from mimics like pulmonary lymphangiectasia. The patient achieved symptomatic remission after pericardiectomy, thoracic catheter drainage, and postoperative sirolimus. This case enriches pediatric GLA literature, highlights multidisciplinary diagnosis value, and supports mTOR inhibitors' role in pediatric GLA management.