We experienced a case of malignant rhabdoid tumor of the left kidney. The case was a 1-year and 2-month-old girl and had the left a 9 X11 cm sized mass at the left upper abdominal region and multiple metastases in the left lower lung field. Left nephrectomy and tumor extirpation was performed, but the metastatic tumor to paraaortic lymph nodes could not be resected. Histological examination showed malignant rhabdoid tumor. She had been treated after operation with radiotherapy and chemotherapy using actinomycin D, vincristine, adriamycin and cis-platinum. However, several bilateral pulmonary metastases and pleural effusion with numerous tumor cells appeared and the patient expired on the 114th day after operation. The prognosis of this tumor is poor and is considered to be a separate group from the usual nephroblastoma. Seven cases of this tumor have ever been reported in the Japanese literature. There is no effective therapy for this tumor and further study is desired particulary in chemotherapic agent.
