A Primary Malignant Rhabdoid Tumor in Adult Liver

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/ by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Malignant rhabdoid tumors (MRTs) are rare high-grade ma-lignancies in the renal or extrarenal organs. MRTs of the extra-renal organs mostly affect the liver, central nervous system, pelvis , soft tissue, 1 and intra-abdominal cavity. 2 Most MRTs occur in infants and young children, 3-8 although adult onset MRTs do occur. 9 Here, we report a case of primary MRT arising in the liver of a 50-year-old male. A 50-year-old male had admitted for further evaluation of large hepatic mass. The hepatic mass was found accidently in ultrasonography. His major symptom was a 7-kg weight loss in one month. No gastrointestinal symptoms were present. He had no history of hypertension or diabetes. The serologic markers for hepatitis were negative. His mother had died due to cholangiocarcinoma. On physical examination of the patient, a spider angioma was detected. In the laboratory tests, aspartate aminotransferase/alanine aminotransferase/alkaline phosphatase were increased at 148/54/1,215 U/L, alpha-fetoprotein (AFP) 166 ng/mL, and carcinoembryonic antigen (CEA) 8.19 ng/mL, but cancer antigen 19-9 was in the normal range at 26.6 U/mL. Computerized tomography (CT) showed a very large multinod-ular hypoattenuating mass with rim enhancement in the whole left lobe and anterior segment of the right lobe (Fig. 1). Also the common hepatic lymph nodes were increased in size. There was no abnormality in each right of left kidney. On positron emission tomography-CT, a large hypermetabolic mass was seen, encompassing the whole left lobe and the anterior segment of the right lobe (Fig. 2). For diagnostic confirmation, CT-guided needle biopsy was performed. The biopsy cores were composed of hypercellular areas alternating with necrosis and hemorrhage. The cellular le-sion showed loosely cohesive tumor cell clusters separated by intermediate fibrous septa. The large round tumor cells showed abundant eosinophilic cytoplasm and eccentric vesicular nuclei with prominent nucleoli (Fig. 3A). Abnormal mitoses were frequent. Mucicarmine staining was negative for abundant cyto-plasm. The immunohistochemical stains showed diffuse cyto-plasmic-positive staining for cytokeratin (CK) 19 and vimentin (Fig. 3B, C) and focal positivity for epithelial membrane anti-gen and CK7. The immunohistochemical stains for hepatocyte antigen, alpha-fetoprotein, CK20, CD34, CD117, actin, des-min, S100, and human melanoma black 45 (HMB45) showed no reaction in tumor cells. Also the tumor cells showed partial …