A 35-year-old man who presented with months of symptomatic nonproductive cough was found to have a large right-sided pleural effusion, diffuse pleural thickening, lymphadenopathy, and partial collapse of the right lung. He was diagnosed with a rare form of vascular tumor: pleural epithelioid hemangioendothelioma. He underwent a successful right extrapleural pneumonectomy with diaphragm and pericardial resection and reconstruction. Although often managed with firstline chemotherapy or radiation therapy, our case shows that upfront surgical resection is feasible for extensive pleural epithelioid hemangioendothelioma at a high-volume regional center. Whether aggressive resection improves survival is yet to be determined.
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