A Case of Hepatic Epithelioid Hemangioendothelioma Who Underwent Multidisciplinary Treatments with Difficulty in Preoperative Diagnosis

Abstract

Epithelioid hemangioendothelioma (EHE) of the liver is a very rare malignant tumor. We report a case of multidisciplinary treatment of EHE of the liver in which there was difficulty in preoperative diagnosis. A 62-year-old woman showed mild elevations of liver enzyme levels, and a computed tomography examination detected a ring-enhanced hepatic mass in S6. Fluorodeoxyglucose positron emission tomography (FDG-PET) showed an accumulation of FDG in the same area and in a cervical lymph node. We diagnosed the tumor as an advanced stage cholangiocarcinoma and combination chemotherapy of gemcitabine and S-1 was performed. After chemotherapy, right lobectomy was performed using hand-assisted laparoscopic surgery; the histological findings were consistent with an EHE. After the operation, FDG-PET demonstrated an accumulation of FDG in the liver and the abdominal para-aortic lymph node. Therefore same chemotherapy was performed again. Post chemotherapy the patient has had no recurrence and no metastasis has been observed. EHE is difficult to distinguish from metastatic liver cancer and cholangiocarcinoma, and we could not diagnose the hepatic EHE before the operation. This case suggests that gemcitabine and S-1 may be a new therapeutic option in cases where there is a recurrence of hepatic EHE and in cases of metastasis.