Metastatic rhabdoid tumor of the kidney (RTK) is a highly lethal malignancy; only one survivor with stage 4 disease has been reported. The authors reviewed the cases of two patients with metastatic RTK who had excellent responses to therapy. Both patients were treated with radiation therapy and alternating courses of ifosfamide, carboplatin, and etoposide (ICE) and vincristine, doxorubicin, and cyclophosphamide (VDC). The patients are without evidence of disease at 24 months and 12 months from the detection of metastasis. Alternating courses of ICE and VDC have activity against metastatic RTK. This combination of agents warrants prospective investigation in clinical trials.

Malignant rhabdoid tumor is a rare childhood tumor that commonly starts in the kidneys but also can occur in other soft tissues or in the brain, where it is referred to as atypical teratoid rhabdoid tumor (ATRT).

Malignant rhabdoid tumor occurs most commonly in infants and toddlers; the average age of diagnosis is 15 months old. There are about 20 to 25 new cases of malignant rhabdoid tumor diagnosed each year in the United States. Cells from malignant rhabdoid tumors in children can spread (metastasize) to other areas of the body.