Hepatic Epithelioid Hemangioendothelioma: Difficult Differential Diagnosis from Angiosarcoma

Abstract

Hepatic epithelioid hemangioendothelioma (EHE) is extremely rare, and preoperative diagnosis is difficult because hepatic EHE has clinicopathological features that are similar to those of angiosarcoma. However, it is important to differentiate hepatic EHE from angiosarcoma because the latter is an aggressive tumor with poor prognosis. We herein report a case of hepatic EHE that was difficult to distinguish from angiosarcoma by tumor biopsy. A 30-year-old man with Crohn's disease presented with multiple liver tumors. The tumors were preoperatively diagnosed as angiosarcoma by tumor biopsy. The patient underwent extended left hemihepatectomy with biliary reconstruction and partial resection of segments 6 and 8. Immunohistochemical staining was positive for CD34, factor VIII, and calmodulin binding transcription activator 1 (CAMTA1), and the pathological diagnosis was EHE. Two years after surgery, a recurrent tumor was found in liver segment 6, for which laparoscopic partial hepatectomy was performed. Pathological examination revealed recurrence of EHE. The patient remained well with no evidence of tumor recurrence as of 9 months after the second resection. In conclusion, we

Multifocal Epithelioid Hemangioendothelioma – A Case Report

“…In this situation a possibility of other epithelioid vascular tumors like epithelioid angiosarcoma may be considered, but histologically epithelioid angiosarcoma exhibits a higher grade of cytological atypia and mitotic activity, which shows atypical mitotic figures. High grade anaplasia is not present in epithelioid hemangioendothelioma [13] .…”

“…Metastatic lesions involve liver and other viscera rarely. Such cases have very poor prognosis, and the biopsy usually shows an aggressive growth pattern, resembling angiosarcoma [4] . Mostly, the lesions are asymptomatic and rarely, presents with respiratory discomfort, painful masses and symptoms of spine compression [5] .…”

Hepatic epithelioid hemangioendothelioma simulating liver metastasis: A case report

“…Nearly all the patients reported in the large study of Makhlouf et al expressed factor VIII-related antigen; CD34 and CD31 were expressed in 94% and 86% of the cases respectively [ 6 ]. 75% of the cases are initially misdiagnosed as liver metastases or primary liver tumors [ 15 ]. Differential diagnosis includes hepatic hemangioma, hepatic angiosarcoma, hepatocellular carcinoma, cholangiocarcinoma, and metastatic tumors [ 5 , 6 , 15 ].…”

“…75% of the cases are initially misdiagnosed as liver metastases or primary liver tumors [ 15 ]. Differential diagnosis includes hepatic hemangioma, hepatic angiosarcoma, hepatocellular carcinoma, cholangiocarcinoma, and metastatic tumors [ 5 , 6 , 15 ]. The most challenging differential diagnosis remains the hepatic angiosarcoma [ 6 , 15 ].…”

“…Differential diagnosis includes hepatic hemangioma, hepatic angiosarcoma, hepatocellular carcinoma, cholangiocarcinoma, and metastatic tumors [ 5 , 6 , 15 ]. The most challenging differential diagnosis remains the hepatic angiosarcoma [ 6 , 15 ]. Both tumors show positivity for factor VIII-related antigen, CD34, and CD31 [ 6 , 15 ].…”