S1498 Epitheliod Hemangioendothelioma a Silent Killer

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Introduction: Epitheloid Hemangioendothelioma (EHE) is a rare vascular tumor which accounts for less than 1% of all vascular tumors. EHE originates from vascular endothelial or pre-endothelial cells. Due to their rarity and heterogeneous presentation, which may often lead to late diagnosis, EHE tumors typically carry a poor prognosis. We present a case of a 76 year old male who presented with metastatic epithelioid hemangioendothelioma. Case Description/Methods: A 76 yo male with a past medical history of AAA, COPD, CAD with CABG, and pulmonary nodule positive for EHE localized 1 month prior, presented to the emergency department with shortness of breath and generalized fatigue for the past 3 weeks. Physical exam findings were pertinent for pallor of the skin, tachycardia and hypotension. Laboratory findings revealed a hemogram of 6.8 and a positive guiac test for which the patient was transfused with 2 units of packed red blood cells. Esophagogastroduodenoscopy was performed which revealed a small, flat square shaped mass at the distal esophagus. A duodenal ulcer with a flat, pigmented spot was visualized as well and treated with Argon plasma coagulation. Patient was taken for Endoscopic Ultrasound, for which the esophageal mass was completely resected and treated with hot biopsy forceps. Histopathological analysis showed highly atypical epithelioid vascular proliferation arranged in solid sheets and rare small vascular channels likely from metastasis. The lesion was associated with abundant hemorrhagic stroma and lympho-vascular involvement. Immunohistochemistry analysis revealed positive staining for CD31, FLI1 and ERG leading to EHE as the diagnosis. Patient also had a lung nodule that was positive for CD 31 and FLI1. Since the patient had metastatic disease palliative measures were offered. Discussion: EHE tumors are typically asymptomatic which may lead to delayed diagnosis. Given the paucity of medical literature due to their rarity, there is currently no defined standard of treatment. EHE is typically found is the Liver, lung, bone or liver and lung together. The mean survival is approximately 4 years ranging from 6 months to 24 years, with mortality reaching 65% if the lung is involved. Current treatment modalities include chemotherapy and surgical resection for localized tumors.Figure 1.: Endoscopic view of tumor at GE junction.

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