Pulmonary epithelioid hemangioendothelioma: A case report and brief review of the literature

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Due to its rarity and nonspecific symptoms, pulmonary epithelioid hemangioendothelioma (PEH) is often prone to misdiagnosis or delayed diagnosis until it progresses to advanced stages.The present case report describes the case of a patient with symptomatic PEH identified by computed tomography (CT) angiography.A 31-year-old male worker presented with a 1-month history of left-sided chest pain.He had a history of 10 years of smoking.The patient initially sought evaluation by a cardiologist.A cardiac workup revealed normal coronary vessels.However, a contrast-enhanced chest CT scan demonstrated multiple well-defined bilateral pulmonary nodules.The patient underwent video-assisted thoracoscopic surgery for obtaining biopsy of bilateral lung nodules, pleura and mediastinal lymph nodes.A histopathological examination confirmed the diagnosis of PEH.Immunohistochemical staining supported the diagnosis with ERG positivity, and TTF1 and AE1/AE3 negativity in the tumor cells.In addition, a total of 12 cases of PEH were selected from the literature and these cases were reviewed.The ages of these patients ranged from 35 to 70 years, with a slightly equal sex distribution.Of note, one-third of the cases were incidental findings.The majority of the cases had bilateral lung lesions on imaging, some of which exhibited calcifications, necrosis, or pleural effusion.Metastases were observed in 4 cases, affecting bones, lymph nodes, liver, spleen and brain.Immunohistochemistry demonstrated positivity for vascular markers in all cases.A total of 6 patients succumbed due to disease progression and related complications.On the whole, the present case report demonstrates that PEH may present with non-specific symptoms and may often be diagnosed incidentally during general checkups.

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