Malignant hepatic epithelioid hemangioendothelioma is a rare, vascular tumor of the liver with peak incidence in the middle age with a female preponderance and of unknown etiology. Majority of the tumors are asymptomatic. The gold standard for diagnosis of the tumor is liver histology showing presence of epithelioid tumor cells on a background of a hyaline stroma. Immunohistochemical positivity for CD31, CD34, Factor VIII, and Podoplanin (D2-40) is confirmatory. The treatment of choice is liver transplantation. ( J CLIN EXP HEPATOL 2017;7:68-70) A 26-year-old woman was referred to our institution with a histopathological diagnosis of focal nodular hyperplasia for a space occupying lesion in the liver. The patient had a history of abdominal discomfort localized to the right upper quadrant with significant anorexia and weight loss of 8 kg over a period of 3 months. On examination of the abdomen, at admission, the liver was enlarged (span of 20 cm) and firm in consistency with a smooth surface and regular margin. Spleen was not palpable and there was no free fluid. Other system examinations were normal.The following laboratory investigations were investigated: Hemoglobin-11.9 g/dL, white blood cell count-8100 cells/mm 3 (neutrophils 72%, lymphocytes 17%, monocytes 07%, and eosinophil 04%), ESR-36 mm per h, and platelet count-259 Â 10 5 /mm 3 . Blood sugar, and serum creatinine were normal. The following liver function tests were conducted: serum total bilirubin 1.23 mg/dL, direct-0.35 mg/dL, serum asparate amino transferase-37 U/L, serum alanine amino transferase-37 U/L, serum alkaline phosphatase-430 U/L, and serum gamma glutamyl transpeptidase-123 U/L. International Normalized Ratio was 1.21.Ultrasound of the abdomen done elsewhere showed normal liver echotexture, with regular margins and multiple hypoechoic lesions in the right lobe, suggestive of hepatocellular carcinoma. Portal, hepatic veins, and suprahepatic inferior vena cava were normal. There was no free fluid. Ultrasound-guided fine needle aspiration of the lesion revealed focal nodular hyperplasia.Contrast-enhanced computed tomography (CECT) done at our institution showed multiple hypoattenutating lesions in both lobes of the liver with conglomeration of lesions in the right lobe. Differentials included multicentric hepatocellular carcinoma and malignant epithelioid hemangioendothelioma (Figure 1). A repeat ultrasound-guided biopsy was done, which showed spindle to epithelioid tumor cells embedded in myxoid background and cytoplasmic lumina (vacuole inside their cytoplasm corresponding to a vascular lumen) (Figure 2). Immunohistochemistry showed a diffuse positivity for CD 34 (Figure 3) and focal positivity for CD 31 (Figure 3) suggestive of malignant hepatic epithelioid hemangioendothelioma (HEH).Liver transplant was recommended, which she denied. She reported 7 months later with hoarseness of voice, dysphagia, and nasal regurgitation. Neurological examination revealed left IX, X, XI, XII cranial nerve palsy, and ipsilateral Horner's syndrome. Magne...
