CASE REPORT article
Front. Pediatr.
Sec. Pediatric Pulmonology
Case Report: Diffuse pulmonary lymphangiomatosis in a child
Provisionally accepted
- 1Gansu University of Chinese Medicine, Lanzhou, China
- 2People's Liberation Army Joint Logistics Support Force 940th Hospital, Lanzhou, Gansu Province, China
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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≈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.
Keywords: Diffuse pulmonary lymphangiomatosis (DPL), generalized lymphatic anomaly (GLA), Pediatric case, Diagnostic Imaging, histopathology, mTOR inhibitors
Received: 11 Mar 2025; Accepted: 28 Nov 2025.
Copyright: © 2025 Zhang, Yuan, DU, Liu, Li, Hu and Li. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Qingxin Li
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