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Metastatic Angiosarcoma: A Rare Cause of Massive Spontaneous Hemothorax FREE TO VIEW

Muhammad Khawar, MD; Yasir Usman, MD; Syed Naqvi, MD; Shoaib Ahmad, MD
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Internal Medicine, Oklahoma University Health Sciences Center, Oklahoma City, OK

Chest. 2014;146(4_MeetingAbstracts):655A. doi:10.1378/chest.1992231
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SESSION TITLE: Cancer Student/Resident Case Report Posters II

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Angiosarcoma is a rare malignant tumor of endothelial derivation that exhibits a strong tendency to metastasize to the lungs. We describe an unusual case of a young patient with spontaneous massive hemothorax on presentation, who later was found to have metastatic angiosarcoma involving the lungs.

CASE PRESENTATION: A previously healthy 39-year-old male, who was an ultra-marathon runner, presented with the complaint of shortness of breath and generalized fatigue for one day. Physical examination was notable for dullness to percussion and decreased breath sounds over left hemithorax. He was anemic on presentation with hemoglobin (Hb) and hematocrit (Hct) of 7 g/dL and 22% respectively. Chest X-ray (CXR) revealed large left sided pleural effusion with multiple areas of ground glass opacification (GGO). Nearly 1900 mL of uniformly bloody fluid was evacuated during thoracentesis. Laboratory analysis identified the fluid as true hemothorax (Pleural fluid Hct 16% with peripheral blood Hct 22%). Cytological and microbiological examinations produced normal results. Chest computed tomography (CT) showed multiple large pleural-based heterogeneous lesions with prominent GGO within the lung parenchyma. Further, a positron emission tomographic scan (PET-CT) revealed a large hypermetabolic lesion at left paraspinal musculature (20.1 SUV) and multiple hypermetabolic pulmonary lesions (8.4 SUV). CT-guided biopsy of left paraspinal mass was done and pathology was significant for undifferentiated angiosarcoma with CD31, Vimentin, BAF47 and CD138 strongly and diffusely positive on immunohistochemistry. The patient was transferred to Oncology for palliative chemotherapy of his underlying metastatic sarcoma.

DISCUSSION: Angiosarcomas are soft-tissue tumors that are highly prone to distant metastasis and account for <2% of all sarcomas. The most common presentation of pulmonary metastasis in angiosarcoma includes hemoptysis, pneumothorax and atelectasis. However, spontaneous hemothorax as the initial clinical manifestation of metastatic disease is rare. Surgical resection, radiation and chemotherapy have all been attempted as therapeutic options, but none of them have been shown to be dramatically effective. The prognosis of angiosarcoma is especially poor; with most patients dying within 1 year after the diagnosis, whatever the mode of therapy was used.

CONCLUSIONS: Primary or metastatic pulmonary angiosarcomas should be considered a cause of spontaneous massive hemothorax. They are aggressive tumors that need to be recognized and treated promptly in order to decrease the risk of distant metastatic disease.

Reference #1: Rafal Krenke, Joanna Klimiuk, Piotr Korczynski et al. Hemoptysis and Spontaneous Hempothorax in a Patient With Multifocal Nodular Lung Lesions. CHEST 2011; 140(1): 245 - 251

DISCLOSURE: The following authors have nothing to disclose: Muhammad Khawar, Yasir Usman, Syed Naqvi, Shoaib Ahmad

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