Epithelioid hemangioendothelioma of the liver: MR imaging findings
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.
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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
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Cited by (20)
Pediatric Hepatobiliary Neoplasms: An Overview and Update
2017, Radiologic Clinics of North AmericaCitation 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
Epithelioid hemangioendothelioma: an uncommon liver tumor
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2010, GastroenterologyHepatic Epithelioid Hemangioendothelioma in Taiwan: A Clinicopathologic Study of Six Cases in a Single Institution Over a 15-Year Period
2010, Journal of the Formosan Medical AssociationHepatic epithelioid hemangioendothelioma: review of 3 cases
2009, Journal de RadiologieMagnetic Resonance Imaging of Liver Lesions: Exceptions and Atypical Lesions
2008, Current Problems in Diagnostic RadiologyCitation 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