Malignant rhabdoid tumor is a distinct renal tumor in pediatric age group and extremely rare in adults. It was originally described as a rhabdomyosarcomatoid variant of Wilms' tumor. But subsequent studies failed to confirm myogenous differentiation, so the rhabdoid tumor is now considered to be a distinct and unique disease type of highly malignant renal tumor, histogenetically unrelated to Wilms' tumor. However the histogenesis have not been clearly defined until now. We report a case of malignant rhabdoid tumor of the kidney in a 34-year-old man who represented with a left abdominal mass. Grossly, a large mass occupying most of the left kidney except for a part of upper pole was invading beyond renal capsule and the perirenal soft tissue.
It measured 18x14 cm in dimension and was soft, lobulated and yellowish gray with large areas of hemorrhage and necroses. Microscopically, the tumor mass was composed of sheets of round or polygonal neoplastic cells growing in a solid pattern. These tumor cells were medium to large in size with ample cytoplasm containing recognizable eosinophilic inclusion and had an eccentrically located, large nucleus with one or a few prominent nucleoli. Mitotic figures were frequently observed. Ultrastructurally, the tumor cells contained whorled filamentous inclusions corresponding to vimentin, epithelial membrane antigen and cytokeratin in immunostaining.
Malignant rhabdoid tumor of the kidney(MRTK), an uncommon renal tumor found in children, is one of the most lethal neoplasms of early life. It was first recognized during a review of the first National Wilms' Tumor Study(NWTS) as an extremely aggressive neoplasm whose appearance often mimicks those of skeletal muscle tumors, but without histological, immunohistochemical, and ultrastructural markers of rhabdomyogenesis. Herein we present two cases of malignant rhabdoid tumor of the kidney, one occurring in a 6-month-old male baby, and the other in a 123-month-old girl. They presented a huge tender mass on the left upper quadrant of the abdomen. Microscopically, each case was very cellular and composed of sheets of round or polygonal cells with ample cytoplasm often containing eosinophilic filamentous inclusions and round vesicular nuclei with prominent nucleoli. Case 1 showed lymphomatoid pattern, but case 2 showed foci of several variant patterns associated with classic appearances. The tumor cells showed a strong reactivity for vimentin in both cases. Unusually, case 2 showed focal reactivity for epithelial membrane antigen(EMA), muscle specific actin, and smooth muscle actin. Ultrastructural study confirmed the presence of whorled bundles of intermediate filaments in paranuclear position, and a prominent nucleolus.
Eosinophilic cytoplasmic globules may be seen in a variety of neoplastic and nonneoplastic conditions and are most often associated with alpha-1-antitrypsin deficiency, several pathologic liver conditions and yolk sac tumors. A few cases of eosinophilic cytoplasmic globules in renal cell carcinoma have been reported but there has only been one report of papillary type. We report another case of papillary renal cell carcinoma with eosinophilic cytoplasmic globules, which is similar to a Mallory body but with different properties.