Epithelioid hemangioendothelioma of the liver: MR imaging findings

https://doi.org/10.1016/S0730-725X(02)00604-5Get rights and content

Abstract

We report the MR appearance of epithelioid hemangioendothelioma (EHE) in a 51 year old man, who presented with a 14-month history of fatigue and anemia to his physician. The tumor showed massive replacement of the liver and had an unusual appearance of multiple high fluid content focal lesions with diminished vascularity.

Introduction

Epithelioid hemangioendothelioma (EHE) is a tumor of vascular origin and is a very rare clinical entity [1]. The tumor has variable malignant potential, ranging between benign hemangioma and malignant hemangioendotheliosarcoma [1]. The tumor generally occurs in adults, with a female predominance. Clinical signs and symptoms are often non-specific and, additionally, the tumor can be difficult to diagnose on the basis of biopsy results. EHE arises predominantly in soft tissue and bone and rarely in the liver [2]. Two types of hepatic EHE have been reported: nodular-type and diffuse-type [3]. The etiology remains unknown. Most patients survive 5–10 years after diagnosis, reflecting its moderate malignant potential. Few reports have described the MR appearance of EHE with the prior reports describing lesions which resemble HCC [4]. We report a case of EHE with massive involvement of the liver showing an unusual MR appearance in a 51-year old male patient.

Section snippets

Case report

The patient, a 51-year-old white man, presented 14 months prior to our MR examination with fatigue to his physician. He was found to have severe anemia (HGB 6.5 g/dl). The patient underwent bone marrow biopsy which was unremarkable. CT examination revealed an enlarged spleen and the patient underwent splenectomy. Six months following splenectomy he improved symptomatically and his blood counts returned to normal. After another eight months his feelings of fatigue returned, and the patient again

Discussion

Histologically, EHE is composed of variable proportions of epithelioid, intermediate and dendritic cells in a myxoid stroma [1], [5]. Neoplastic cells obliterate sinusoids, terminal hepatic and portal veins. Progressive sclerosis and calcification may be seen in up to 30% of patients [3]. Pleomorphism of tumor cells, together with the varied cell population and the various parenchymal and stromal reactions, may render establishing the pathologic diagnosis difficult. In our patient, multiple

References (9)

  • H. Makhlouf et al.

    Epithelioid hemangioendothelioma of the livera clinicopathologic study of 137 cases

    Cancer

    (1999)
  • J.M. Lauffer et al.

    Epithelioid hemangioendothelioma of the liver. A rare hepatic tumor

    Cancer

    (1996)
  • W.J. Miller et al.

    Epithelioid hemangioendothelioma of the liverimaging findings with pathologic correlation

    AJR

    (1992)
  • K. Uchimura et al.

    Hepatic epithelioid hemangioendothelioma

    J Clin Gastroenterol

    (2001)
There are more references available in the full text version of this article.

Cited by (20)

  • Pediatric Hepatobiliary Neoplasms: An Overview and Update

    2017, Radiologic Clinics of North America
    Citation Excerpt :

    The central myxoid and hyalinized stroma may appear more hypointense on T1-weighted MR images and more hyperintense on T2-weighted MR images. The targetoid enhancement pattern is similar to that of CT.64 Another characteristic finding of epithelioid hemangioendothelioma is the so-called lollipop sign, with the hepatic or portal vein tapering toward the masses avascular core (Fig. 17).65 Numerous malignancies may metastasize to the liver in children including neuroblastoma, Wilms tumor, rhabdomyosarcoma, germ cell tumors, neuroendocrine pancreatic tumors, pancreatoblastoma, gastrointestinal stromal tumor, and desmoplastic small round cell tumor.4

  • Magnetic Resonance Imaging of Liver Lesions: Exceptions and Atypical Lesions

    2008, Current Problems in Diagnostic Radiology
    Citation Excerpt :

    Epithelioid hemangioendothelioma (EH) of the liver is a rare vascular tumor and typically has a low- to intermediate-grade malignant behavior.23,24 EH occurs most frequently in adults, with higher incidence in female patients.24,25 Two types of liver EH have been described, which include the nodular and diffuse-type EH.25

View all citing articles on Scopus
View full text