Diffuse Pulmonary Lymphangiomatosis



Diffuse Pulmonary Lymphangiomatosis


Monica P. Revelo, MD, PhD

Brandon T. Larsen, MD, PhD





Key Facts


Terminology



  • Rare disease characterized by proliferation of lymphatic vessels and smooth muscle cells along lymphatic routes in visceral pleura, bronchovascular bundles, and interlobular septa


Etiology/Pathogenesis



  • Congenital disease due to abnormal lymphatic system development


Clinical Issues



  • Mean age at presentation: 8.5 years


  • No gender predilection


  • Localized to lung or chest


  • Widespread distribution involving spleen, bones (e.g., ribs, vertebrae, humerus, femur), liver, and skin


Microscopic Pathology



  • Anastomosing lymphatic channels in pleura, interlobular septa, bronchovascular bundles


  • Thickening of pleura, interlobular septa, and bronchovascular bundles


  • Lymphatic channels filled with chyle


  • Smooth muscle fascicles in periphery of lymphatic channels


Ancillary Tests



  • CD31 and D2-40 stains highlight endothelial lining of lymphatic channels


Top Differential Diagnoses



  • Diffuse pulmonary lymphangiectasia


  • Lymphangioma


  • Lymphangioleiomyomatosis






Numerous irregular lymphatic channels image expand the pleura but preserve the underlying alveolated lung parenchyma image. In some cases, hemorrhage image into the lymphatic spaces may be present.






Irregular, thin-walled, anastomosing lymphatic channels dissect between pleural collagen bundles. On higher power, the bland endothelial cell lining image of the lymphatic spaces is apparent.


TERMINOLOGY



Abbreviations



  • Diffuse pulmonary lymphangiomatosis (DPL)



Definitions



  • Rare disease characterized by proliferation of lymphatic vessels and smooth muscle fascicles along preexisting lymphatic routes in visceral pleura, bronchovascular bundles, and interlobular septa


ETIOLOGY/PATHOGENESIS


Developmental Anomaly



  • Congenital disease due to abnormal lymphatic system development


CLINICAL ISSUES


Epidemiology



  • Age



    • Mean at presentation: 8.5 years


    • May manifest at birth and rarely in adults


  • Gender



    • No predilection


Site



  • Localized to lung or chest


  • Widespread distribution involving spleen, bones (e.g., ribs, vertebrae, humerus, femur), liver, and skin


Presentation



  • Dyspnea


  • Cough


  • Hemoptysis


  • Chest pain


  • Milky sputum


  • Pleural effusions


  • Mediastinal enlargement


Laboratory Tests



  • Pulmonary functional tests demonstrate



    • Mixed pattern obstructive/restrictive


    • Restrictive pattern only


Natural History



  • Progressive condition leading to end-stage respiratory failure



Treatment



  • Options, risks, complications



    • Definitive treatment is not available; palliative measurements and adjuvant therapies are used


  • Surgical approaches



    • Pleurocentesis


    • Pleurodesis


    • Pleurectomy


    • Ligation of thoracic duct


    • Transplantation


  • Drugs



    • Interferon alpha


    • Glucocorticoids


    • Propranolol


  • Radiation



    • For localized disease


Prognosis



  • Poor; causes of death include respiratory failure, infections, and chylus effusions


IMAGE FINDINGS


Radiographic Findings



  • Chest x-ray shows mediastinal enlargement


  • Peribronchial expansion


  • Increased interstitial markings


  • Chylous pleural/pericardial effusions


  • Multifocal cystic bone lesions with porous soap or honeycomb texture



CT Findings



  • Thickening of interlobular septa and bronchovascular bundles


  • Ground-glass opacities


  • Pleural thickening and effusions


  • Diffuse fluid infiltration of mediastinal soft tissue


  • Increased mediastinal/hilar nodes

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Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Diffuse Pulmonary Lymphangiomatosis

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