Definition / general
Diffuse proliferation of abnormal, complex lymphatic channels (lymphangiomas) that may involve lung or other organs (Lymphat Res Biol 2011;9:191)
Pulmonary involvement often accompanied by chylous pleural effusions
May be progressive and fatal from respiratory failure Terminology
Lymphangioma if proliferation is focalEpidemiology
Rare condition, typically presents in children and young adults
No sex predilectionSites
Localized to lung/chest or can also involve multiple organs, including bone, spleen, soft tissue, liverPathophysiology / etiology
Progressive disease with recurrent effusions and respiratory failure secondary to infections and chylous accumulation
Congenital, possibly from abnormal lymphatic developmentClinical features
Generally presents with dyspnea, wheezing, cough and may be confused with asthma
Occasionally with chyloptysis or hemoptysisDiagnosis
Clinical presentation and imaging findings may suggest diagnosis, particularly if bone involvement is noted
Most cases diagnosed with open lung biopsy
Transbronchial biopsy may confirm disease if vesicles seen on bronchoscopy (Clin Radiol 2005;60:921) Laboratory
Thoracocentesis may reveal chylous effusion (elevated triglycerides)
Radiology images
Xray: nonspecific findings, including pleural effusion, diffuse pulmonary opacities, interstitial thickening
CT: bilateral ground-glass opacities, smooth thickening of interlobular septa, bronchovascular bundles, pleura; may show infiltration of the mediastinal soft tissue by cystic fluid densitiesRadiology images
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Lower lung heterogeneous opacities
Thickening with effusions
Clinical images
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Subpleural lymphatic vessels
Gross description
Subpleural and septal thickening
Microscopic (histologic) description
Diffuse proliferation of complex, anastomotic lymphatic channels without significant dilatation, lined by benign appearing, flattened endothelial cells
Prominence of lymphatic channels in visceral pleura, interlobular septa, and bronchovascular bundles with surrounding bundles of spindle cells, interspersed collagen, vessels
Lymphatic channels may contain acellular, eosinophilic, proteinaceous material
May show hemorrhagic "kaposiform" component, with compressed vascular channels, plump spindle cells, hemosiderinMicroscopic (histologic) images
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D2-40
Native lung
Multiple proliferating vascular spaces