Pulmonary Epithelioid Hemangioendothelioma Presenting as Isolated Cervical Lymphadenopathy: A Diagnostic Dilemma

Epithelioid hemangioendothelioma (EHE) is a rare malignant vascular sarcomatous tumor which can arise from the soft tissue of diverse anatomical sites. Histologically, it shows a characteristic primitive vasoformation expressing typical endothelial markers with the classical WWTR1-CAMTA1 fusion gene. It follows an unpredictable clinical trajectory between the indolence of hemangiomas and the aggressiveness of angiosarcomas. It poses a diagnostic challenge due to its common symptoms masking its uncommon nature. We report the case of a 69 year old male who presented with hemoptysis and a painless neck swelling. Imaging showed multiple cavitating lung nodules and necrotic lymphadenopathy. Initial microbiological and autoimmune workups were inconclusive. A biopsy of the cervical lymph node revealed florid vascular proliferation with epithelioid cells, confirming EHE. Despite initiation of systemic chemotherapy with paclitaxel at 175 mg/m², Bevacizumab 15 mg/kg and Carboplatin Area Under Curve (AUC) 6 every 3 weeks for 6 cycles, the patient’s condition deteriorated progressively and the patient succumbed to the disease approximately four months after the initial diagnosis. This case underscores the diagnostic uncertainties of rare vascular tumors, how it can present as cervical lymph node and reinforces the importance of integrating multiple clinical insights.