PLEURAL AND CHEST WALL LYMPHANGIOMATOSIS

Abstract

SESSION TITLE: Disorders of the Mediastinum 2 SESSION TYPE: Fellow Case Reports PRESENTED ON: 10/10/2018 10:45 am - 11:45 am INTRODUCTION: Recurrent pleural effusions of unclear etiology can be a challenging clinical scenario. We present a rare case of pleural Lymphangiomatosis with no lung parenchymal involvement, presenting atypically late in adult life. CASE PRESENTATION: A 66 year old lady presented to pulmonary clinic with subacute on chronic shortness of breath. She had a history of recurrent exudative right sided pleural effusions for the last 5 years. The effusions were consistently culture and cytology negative. She had no other cardiopulmonary symptoms, past history, exposures or family history. Pertinent exam findings included absent breath sounds in the right lower lung field. Chest X-ray revealed a moderate right-sided pleural effusion with associated right lower lobe atelectasis (Image 1). CT chest (Image 2) showed a large mediastinal cystic mass, cysts along the internal mammary wall and a small right pleural effusion with areas of pleural thickening. She underwent a diagnostic and therapeutic Video-assisted Thoracoscopy/Thoracotomy. Serosanguinous pleural fluid was drained and a pleural peel excision/pleurodesis/cyst excisions were performed. Histopathology revealed lymphangiomatosis with lymphangiectasia. She was symptom free post-procedure but presented a few years later with recurrent disease confirmed on CT chest (Image 3). She declined surgery and is being managed with therapeutic thoracenteses, propranolol, anti-Vascular Endothelial Growth Factor (VEGF) therapies and Sirolimus, with good response over the last few years. DISCUSSION: Lymphangiomatosis is a rare disorder characterized by lymphatic overgrowth along normal systemic lymphatic channels(1). While it has been reported to present over diverse age groups from birth to 80 years, with no clear gender predilection, it rarely presents after 20 years of age. In the chest, it can present as Diffuse Pulmonary Lymphangiomatosis, involving only pulmonary parenchyma and is clinically/histopathologically unique from Lymphangioleiomyomatosis. It may also present purely with mediastinal, pleural and chest wall involvement. CT chest usually shows interlobular septal thickening with mediastinal involvement (2). Factor 8 related antigen and CD31 are usually positive on immunohistochemistry. Chylous effusions, chyloptysis, chylopericardium and chylous ascites are common and complications include protein wasting enteropathy, lymphedema and Disseminated Intravascular Coagulation. Surgical interventions are complex given difficulty separating pathologic from normal lymphatics /other anatomic structures. Lymphoscintigraphy can often help to guide therapy. Anti-VEGF therapies and Sirolimus(3) are being increasingly used as therapeutic and maintenance measures. CONCLUSIONS: While a rare cause of pleural effusions in the adult population, Lymphangiomatosis can be a challenging condition to manage. Reference #1: Tazelaar, H.D., et al., Diffuse pulmonary lymphangiomatosis. Human Pathology. 24(12): p. 1313-1322. Reference #2: Faul, J.L., et al., Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med, 2000. 161(3 Pt 1): p. 1037-46. Reference #3: Reinglas, J., R. Ramphal, and M. Bromwich, The successful management of diffuse lymphangiomatosis using sirolimus: a case report. Laryngoscope, 2011. 121(9): p. 1851-4. DISCLOSURES: No relevant relationships by Augustine Lee, source=Admin input No relevant relationships by Divya Padmanabhan Menon, source=Web Response