Lung tumor
Benign tumors
Diffuse pulmonary lymphangiomatosis

Reviewer: Roseann Wu, M.D. (see Reviewers page)

Revised: 24 August 2015, last major update August 2015

Copyright: (c) 2003-2015, PathologyOutlines.com, Inc.

PubMed Search: Diffuse pulmonary lymphangiomatosis [title]
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 focal
  • Epidemiology
  • Rare condition, typically presents in children and young adults
  • No sex predilection
  • Sites
  • Localized to lung/chest or can also involve multiple organs, including bone, spleen, soft tissue, liver
  • Pathophysiology / Etiology
  • Progressive disease with recurrent effusions and respiratory failure secondary to infections and chylous accumulation
  • Congenital, possibly from abnormal lymphatic development
  • Clinical Features
  • Generally presents with dyspnea, wheezing, cough and may be confused with asthma
  • Occasionally with chyloptysis or hemoptysis
  • Diagnosis
  • 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
  • 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 densities
  • Radiology
    Missing Image

    Lower lung heterogeneous opacities

    Missing Image

    Thickening with effusions

    Prognostic Factors
  • Children have a worse prognosis than older patients; pleural and lung involvement is adverse prognostic factor (J Pediatr Hematol Oncol 2004;26:136)
  • Case Reports
  • 4 year old girl with recurrent hemorrhagic pericardial effusion due to diffuse lymphangiohemangiomatosis (J Med Case Rep 2010;4:62)
  • 35 year old man with diffuse pulmonary lymphangiomatosis (Basic Appl Pathol 2012;5:63)
  • Diffuse pulmonary lymphatic disease presenting as interstitial lung disease in adulthood (Am J Surg Pathol 2012;36:1548)
  • Kaposiform lymphangiomatosis (Pediatr Blood Cancer 2015;62:901)
  • Treatment
  • Supportive, including observation and medical management
  • Surgical resection for localized disease; pleurocentesis, pleurodesis, pleurodectomy, ligation of thoracic duct (J Thorac Cardiovasc Surg 2007;133:1664)
  • Successful lung transplantation has been reported (Am J Transplant 2008;8:1946)
  • Clinical Images
    Missing Image

    Subpleural lymphatic vessels

    Gross Description
  • Subpleural and septal thickening
  • Micro 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, hemosiderin
  • Micro Images
    Missing Image

    D2-40

    Missing Image

    Native lung

    Missing Image

    D2–40, Masson trichrome

    Missing Image

    Multiple proliferating vascular spaces

    Positive Stains
  • CD31, D2-40, Factor VIII in endothelial cells
  • Vimentin, actin, desmin in spindle cells
  • Negative Stains
  • HMB45, keratin (AE1/3 staining of alveoli contrasts with negative lymphatics), HHV8
  • Differential Diagnosis
  • Kaposiform hemangioendothelioma
  • Kaposi sarcoma: HHV8 positive
  • Lymphangiectasis: dilation of existing lymphatics in normal distribution
  • Lymphangioleiomyomatosis: HMB45 positive, more smooth muscle cells
  • Pulmonary capillary hemangiomatosis