Epithelioid Hemangioendothelioma at the Junction of the Internal Jugular and Subclavian Vein: A Case Report.

Abstract

Epithelioid hemangioendothelioma (EHE) is an exceptionally rare vascular tumor with an incidence of <1/million people/year. This case report describes a patient who initially presented with progressive left-sided neck swelling and dysphagia and was found to have EHE at the confluence of the left subclavian and internal jugular veins. Diagnosis was confirmed with a core needle biopsy after an initial inconclusive fine-needle aspiration. Surgical management included direct laryngoscopy, left selective neck dissection (levels 2-4), left internal jugular vein sacrifice, and vascular reconstruction with a saphenous vein graft, followed by adjuvant radiation therapy due to positive margins. Early recognition of EHE in complex anatomical locations is essential and requires a multidisciplinary approach for optimal diagnosis and treatment.