Young Male with Rare Tumor of Liver

Abstract

Purpose: A 38-year-old Asian male presented with 4 months' history of non-productive cough, severe back pain, loss of appetite, and weight loss. Physical examination revealed non-tender hepatomegaly and mild spinal tenderness at lower back. Laboratory studies included the following: normal complete blood count, aspartate aminotransferase 58 IU/L (normal 10-40), alanine aminotransferase 136 IU/L (normal 0-40), alkaline phosphatase 211 IU/L (normal 53-128), gamma-glutamyl transpeptidase 167 IU/L (normal 0-50), albumin 3.5 mg/dl, bilirubin 0.6mg/dl, normal alpha-fetoprotein, CA 19-9, and carcinoembrinogen antigen. Hepatitis A, B, and C serology was negative. Computerized tomography with contrast enhancement of the chest and abdomen revealed bilateral reticular infiltrates in the lung and 4.5-cm hypoechoic mass in the right lobe of the liver with several round hepatic calcifications. MRI of the spine showed abnormal signal from T8 to L2, suspicious for metastasis. CT-guided liver biopsy was done, which revealed presence of patchy areas of perivascular, large, atypical epitheloid and spindle cells in the pool of chondromyxoid stroma, with mild-to-moderate nuclear atypia and hyperchromasia. Immunohistochemistry displayed reactivity to cytokeratin's AE1/AE3, S-100, and vimentin, suggesting epithelioid hemangioendothelioma (EHE). Chemo-radiation therapy was contemplated, but due to complications secondary to chemotherapy, the patient expired. EHE is a rare, well-differentiated endothelial tumor representing 1% of all vascular tumors. Primary malignant EHE of the liver is a rare tumor with an incidence of less than 0.1 per 100,000, and arise in absence of any underlying chronic liver disease. It is more frequent in males (3:1) during the second and third decades of life. Liver involvement can present as right-upper quadrant pain or hepatomegaly, with laboratory data usually revealing elevated alkaline phosphatase. Computed tomography findings suggestive of EHE include presence of a large mass located mainly in periphery, with peripheral enhancement. The definitive diagnosis requires histopathological and immunohistochemical evidence of endothelial differentiation. Treatment is usually surgical resection or liver transplant. However, it can be associated with early hematogenous metastasis, as happened in our patient. In multicentric or metastatic lesions, adjuvant chemoradiation therapy can be given. Prognosis is variable; however, involvement of liver is a poor prognostic sign. Although EHE is classified in WHO classification as low-grade to borderline malignant tumor, it has variable malignant potential and can present with metastasis at initial presentation.