Pulmonary Epithelioid Hemangioendothelioma: Case report and a brief review of the literature

Pulmonary Epithelioid Hemangioendothelioma (PEHE) is an uncommon endothelial cell-derived vascular tumor of the lung. It is a form of Epithelioid Hemangioendothelioma (EHE), a low to intermediate-grade malignant vascular sarcoma which may be aggressive and can metastasize. It presents with a wide range of clinical and radiological findings, including bilateral lung nodules and pleural effusion. Diagnosis of PEHE is challenging due to its rarity and similarity to other benign and malignant vascular lesions. It is essential to distinguish PEHE from other vascular lesions of an epithelioid nature, particularly epithelioid angiosarcoma, which has a distinct prognosis and treatment options. Molecular analysis has now conclusively demonstrated this difference by detecting typical gene fusions, such as that of WWTR1-CAMTA1, which is not identified in angiosarcoma. However, even though molecular pathogenesis has been more clearly understood, PEHE lacks a universal treatment regimen, and management is often personalized, depending on therapies such as mTOR inhibitors. The present case illustrates the challenges in diagnosis, variable clinical progression, and the ongoing need for greater awareness among medical professionals regarding PEHE to facilitate earlier diagnosis, improve therapeutic strategies, and enhance patient outcomes for this rare tumor.