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Case Reports in Transplantation
Volume 2018, Article ID 3948784, 3 pages
https://doi.org/10.1155/2018/3948784
Case Report

Hepatic Epithelioid Hemangioendothelioma Presenting as an Enlarging Vascular Lesion within the Spleen

1Department of Surgery, University of Southern California, Los Angeles, CA, USA
2Department of Anatomic Pathology, University of Alberta, Edmonton, AB, Canada
3Department of Surgery, University of Alberta, Edmonton, AB, Canada

Correspondence should be addressed to Juliet A. Emamaullee; ude.csu.dem@eelluamame.teiluj

Received 10 November 2017; Revised 21 February 2018; Accepted 5 March 2018; Published 12 April 2018

Academic Editor: Federica Meloni

Copyright © 2018 Juliet A. Emamaullee et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Epithelioid hemangioendothelioma (EHE) is a rare vascular neoplasm with variable malignant potential that most often presents within the liver. Many patients present with bilobar or extrahepatic disease, and the current treatment paradigm involves liver transplantation, with favorable long term results. Up to 25% of patients are diagnosed incidentally following imaging for other indications, and confirmation of diagnosis requires histologic analysis, as there are no classical imaging features to distinguish hepatic EHE (HEHE) from other solid hepatic lesions. Here we describe a case of microscopic HEHE that was diagnosed following splenectomy for an enlarging vascular tumor within the spleen. Due to the unexpected diagnosis of EHE within the spleen and coexisting but stable appearing liver hemangiomata, a left hepatic lobectomy was performed. Explant histology revealed benign hemangiomata and diffuse, microscopic HEHE. The patient ultimately underwent liver transplantation. HEHE can be a challenging diagnosis, and this case emphasizes that any enlarging vascular lesion, even within the spleen, should prompt a high index of suspicion for HEHE in the setting of known hemangiomata.