Diffuse Pulmonary Lymphangiomatosis



Diffuse Pulmonary Lymphangiomatosis


Monica P. Revelo, MD, PhD

Brandon T. Larsen, MD, PhD









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




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



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


MICROSCOPIC PATHOLOGY


Histologic Features

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