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• W2912673354 abstract "An 86-year-old man presented with prolonged fever for 1 month. He had 13-kg weight loss over 1 year. On physical examination, body temperature was 38°C. Otherwise was unremarkable. Laboratory tests showed white blood cell count of 9.8x109/L (neutrophil 71%), hemoglobin 10.6 g/L, platelet count 470x109/L, alkaline phosphatase 394 U/L, aspartate aminotransferase 66 U/L, alanine aminotransferase 43 U/L, total bilirubin 0.48 mg/dl. Hemoculture, tests for hepatitis C and B viruses were negative. Computed tomography (CT) showed multiple ill-defined hypodense lesions varying in sizes up to 1.6x2x1.9-cm with delayed enhancement scattering throughout the liver, encasing the left hepatic and portal veins, bilateral intrahepatic bile duct dilation, moderate amount of ascites, bilateral pleural effusion and multiple bilateral adrenal nodules. No lymph node enlargement was seen (Figure 1). These findings mimic radiological characteristics of cholangiocarcinoma. Tumor markers, including CA 19-9, AFP and CEA were normal. Liver biopsy showed spindle-shaped tumor cells with hyperchromatic nuclei and eosinophilic cytoplasm arranged in storiform pattern (Figure 2A) with positive FLI1, CD31 and CD34 (Figure 2B-D), compatible with hepatic epithelioid hemangioendothelioma (HEH). Imaging characteristics of several hepatic tumors including intrabiliary metastases, intraductal growing hepatocellular carcinoma and primary biliary lymphoma, sometimes, resemble to the typical CT finding of cholangiocarcinoma. We report a rare case of HEH which the radiological features mimic cholangiocarcinoma. Malignant HEH commonly affects female and has variable disease spectrum. The incidence is < 1 per million population and presentations vary from asymptomatic disease to liver failure. Fever is uncommon presentation (4.4%). The imaging features mostly show hypodense mass lesions with contrast enhancement. Large lesions located in periphery of the liver with features of calcifications (13%), capsular retraction (11%) and compensatory hypertrophy (3%) may be the radiological clues for the diagnosis. Laboratory results are less helpful. Liver biopsy is needed for definite diagnosis of HEH. HEH is a slow progressive tumor and has good prognostic outcome. Liver transplantation is most common modality for HEH without extrahepatic involvement. Adjuvant chemotherapy and/or metastatectomy should be considered for extrahepatic disease. Our patient has expired from pneumonia before the treatment of HEH.Figure: Axial contrast-enhanced computed tomography: arterial phase (A), venous phase (B), and 15-minute delayed phase (C) show multiple ill-defined various-sized hypodense lesions (the largest at black arrow heads) with delayed enhancement and caused upstream left intrahepatic duct dilation (white arrow).Figure: Histopathology of the liver biopsy (X200), (A) Hematotoxin and eosin stain shows spindle-shaped tumor cells with hyperchromatic nuclei, eosinophilic cytoplasm, and lumina formation. Immunohistochemistry reveals positive FLI1 (2B), CD31 (2C), and CD34 (2D)." @default.
• W2912673354 created "2019-02-21" @default.
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• W2912673354 date "2017-10-01" @default.
• W2912673354 modified "2023-09-26" @default.
• W2912673354 title "Hepatic Epithelioid Hemangioendothelioma Mimicking Cholangiocarcinoma: A Case Report" @default.
• W2912673354 doi "https://doi.org/10.14309/00000434-201710001-02318" @default.
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