Evidence map›Paper›PMID 40615978›Full record

ArticleBMC pediatrics2025

Lymphangiomatosis with pulmonary, mediastinal, and spinal involvement in a pediatric patient: a case report.

Yuxing Sun, Jun Zhou, Xiaoqin Hu, Ming Li, Li Tan

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In one paragraph

Article in BMC pediatrics, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

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4 · The record

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5 · Who and what money

Authors and funding

5 authors.

Yuxing Sun *Department of respiration, Kunming Children's Hospital, 288 Qianxing Road, Kunming, Yunnan, 650028, China.
Jun Zhou *Department of pathology, Kunming Children's Hospital, 288 Qianxing Road, Kunming, Yunnan, 650028, China.
Xiaoqin HuDepartment of respiration, Kunming Children's Hospital, 288 Qianxing Road, Kunming, Yunnan, 650028, China.
Ming LiDepartment of respiration, Kunming Children's Hospital, 288 Qianxing Road, Kunming, Yunnan, 650028, China.
Li TanDepartment of respiration, Kunming Children's Hospital, 288 Qianxing Road, Kunming, Yunnan, 650028, China. 6178903@qq.com.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundLymphangiomatosis is a rare disorder characterized by abnormal proliferation and dilation of lymphatic vessels, resulting in non-specific clinical manifestations. This report describes the case of an 8-year-old male patient with lymphangiomatosis involving the lungs, mediastinum, and spine, with the objective of promoting awareness and understanding of this condition. CASE PRESENTATION: An 8-year-old male patient presented with chest tightness. Two years ago, the patient underwent decortication of the pleural fibrous plate due to empyema. Within two months after the operation, the patient successively developed chylothorax and chylopericardium. Chest CT revealed thickening of the interlobular septa and peribronchovascular interstitial tissue in both lungs. Spinal MRI showed scoliosis in the cervicothoracic region, flattening of several vertebral bodies, and multiple osteolytic lesions. Pathological examination of mucosal tissue obtained from the basal segment of the left lower lobe of the lung via bronchoscopy confirmed a diagnosis of lymphangiomatosis. The patient’s condition improved significantly after three weeks of treatment with sirolimus and bevacizumab, with marked alleviation of the chest tightness symptoms during the 23-month follow-up period.

conclusionLymphangiomatosis is associated with indolent progression and non-specific clinical/radiological features, frequently manifesting as multiorgan involvement. Diagnostic suspicion should arise when chylous effusions coexist with osteolytic lesions, although histopathological verification remains the gold standard. Although there is no current consensus on therapeutic strategies, emerging pharmacological interventions including the use of sirolimus, bevacizumab, propranolol, interferon-α, and tyrosine kinase inhibitors, which demonstrate variable efficacy. This case underscores the importance of multidisciplinary collaboration in the management of complex lymphatic malformations and indicates the potential of targeted therapies in pediatric patients.

Indexed as

Lung NeoplasmsLymphangiectasisLymphangiomaMediastinal NeoplasmsChildChylothoraxHumansMagnetic Resonance ImagingMaleTomography, X-Ray ComputedClinical characteristicsLymphangiomatosisPediatric population

Identifiers

PMID40615978
PMCPMC12231639

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.