Epithelioid hemangioendothelioma (EHE) is an extremely rare sarcoma of vascular origin. Primary pleural involvement is extremely under-reported and tends to have a more aggressive course. We report a case of pleural EHE in a Caucasian female in her 50s with a two-month history of dyspnea and chest pain. Investigations, including video-assisted thoracoscopy, revealed extensive pleural scarring and inflammation. Management with trametinib and pazopanib led to a stable disease course, reduction in the frequency of pleural effusion recurrence, and improvement in cancer-related pain.
Keywords: pleural effusion; pleural epithelioid hemangioendothelioma; recurrent epithelioid hemangioendothelioma; trapped lung; video-assisted thoracoscopic surgery (vats).
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