Case Report: Diffuse pulmonary lymphangiomatosis in a child

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.

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Publication Details

Journal
Frontiers in Pediatrics
Published
2026-01-05
DOI
https://doi.org/10.3389/fped.2025.1591611
Primary Topic
Vascular Malformations and Hemangiomas
Type
article
Field-Weighted Citation Impact
0.00

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article

Case Report: Diffuse pulmonary lymphangiomatosis in a child

Fu Li, Zhen Yuan, Qing-Xin Li, Jin-chen Du et al.
Frontiers in Pediatrics
Vascular Malformations and Hemangiomas
article

Case Report: Diffuse pulmonary lymphangiomatosis in a child

Fu Li, Zhen Yuan, Qing-Xin Li, Jin-chen Du, Ya-nan Zhang, Shou-Cai Hu, Bin Liu
article en

Abstract

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.

Frontiers in PediatricsVol. 13
Gansu University of Traditional Chinese Medicine (CN), People's Liberation Army 401 Hospital (CN)
National Natural Science Foundation of China
Good health and well-being
Openalex Percentile: Top 97%
Vascular Malformations and Hemangiomas
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