Pulmonary Epithelioid Haemangioendothelioma Involving Pleura: A Case Report

Abstract

Introduction/Objective Pulmonary epithelioid haemangioendothelioma is a rare, low to intermediate grade tumor of endothelial origin. It is more common in middle aged women. In lung, more than 60 % of the cases present as bilateral multiple perivascular nodules on imaging raising the differential of metastasis or inflammatory cause. WWTR1- CAMTA1 fusion gene is characteristic for this entity. Evidence of pleural involvement carries a poor prognosis. Methods Here, we present a case of epithelioid haemangioendothelioma involving pleura in a 52-year-old woman with a long standing history of rheumatoid arthritis. Patient presented with recurrent pleural effusions and shortness of breath. Results A diagnosis of epithelioid haemangioendothelioma was made on her pleural decortication specimen that showed atypical vascular proliferation and confirmed by immunohistochemical staining. Subsequent right lung wedge resection performed later confirmed the presence of epithelioid haemangioendothelioma in lung parenchyma. Conclusion Pathologists should be aware of this rare condition that can clinically present in a very deceptive manner of recurrent effusion.