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Case Reports
. 2023 Mar 7;25(4):175.
doi: 10.3892/etm.2023.11874. eCollection 2023 Apr.

Diagnosis and treatment of diffuse pulmonary lymphangioma in children: A case report

Xiaopu Sun  1 , Chengyu Lu  1 , Zhanhang Huang  1 , Jiaxing Xu  1 , Huiyi Zhu  1 , Sen Yang  1 , Deihui Chen  1
Affiliations
Case Reports

Diagnosis and treatment of diffuse pulmonary lymphangioma in children: A case report

Xiaopu Sun et al. Exp Ther Med. .

Abstract

The present study aimed to investigate the clinical characteristics of diffuse pulmonary lymphangioma (DPL) in children to improve the diagnosis and treatment of this disease. A case of pediatric DPL was observed for its clinical symptoms, imaging features, lung biopsy pathological characteristics and immunohistochemical phenotypes, and relevant literature was also reviewed. The main clinical manifestations of this pediatric patient were a cough, shortness of breath, hemoptysis, bloody chylothorax and pericardial effusion. Chest computed tomography showed a grid-like shadow and markedly thickened interlobular septa. Pathological examination revealed lymphatic vessel hyperplasia and expansion. Immunohistochemistry showed positive staining of lymphatic endothelial cells CD31 and D2-40. The patient's condition improved after combined treatment with methylprednisone, propranolol, sirolimus and somatostatin, whose bloody chylothorax also achieved good therapeutic effect after conservative treatment. Overall, the clinical and imaging appearances of DPL are lack of characterization, and its clinical manifestations include cough, shortness of breath and chylothorax. Computed tomography may show mesh-like shadows of both lungs and thickened interlobular septa. The definite diagnosis of DPL depends on biopsy pathology. In addition to this case, B-ultrasound-guided puncture biopsy is effective and safe, and propranolol-sirolimus treatment has a certain effect, but the clinical effect may be different. Conservative treatment of pleural effusion can result in better curative effect.

Keywords: children; diagnosis; diffuse pulmonary lymphangiomatosis; oncology; sirolimus.

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Conflict of interest statement

The authors declare they have no competing interests.

Figures

Figure 1
Figure 1
Multiple thickening of interlobular septa in the right lung.
Figure 2
Figure 2
Multiple lymph nodes in bilateral cervical roots and mediastinum, which were fused into clusters, and the trachea and mediastinal great vessels were surrounded.
Figure 3
Figure 3
Pleural effusion of the child was chylous.
Figure 4
Figure 4
Biopsy of mediastinal mass revealed patchy small lymphocytes with a few thymus corpuscles, which were fissured and had a sparse reticular structure.
Figure 5
Figure 5
Bronchoscopy showed obvious congestion, erosion and bleeding of the left bronchial mucosa.
See this image and copyright information in PMC

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