Summary:Although the prognosis of clear cell sarcoma of the kidney (CCSK) has improved, when metastases occur the probability of cure is very low. We have treated two pediatric patients with relapsed CCSK, one with multiple bone metastases and another with brain metastases. After one or two courses of re-induction chemotherapy and radiation therapy to the sites of metastasis, they received double high-dose chemotherapy with autologous bone marrow rescue. Conditioning regimens were ifosphamide plus melphalan for the first autograft and busulfan plus thiotepa for the second. Hematological recovery was prompt, and no severe complications were observed. They are doing well without evidence of recurrence at 19 and 49 months after the second autograft, respectively. Keywords: clear cell sarcoma of the kidney (CCSK); bone marrow transplantation; childhood Clear cell sarcoma of the kidney (CCSK) was first described by Kidds 1 in 1970, and its histologic characteristics were defined thereafter. 2,3 It was also called bonemetastasizing renal tumor of childhood by Marsden et al 2 due to its unusual pattern of spread.Investigation of the efficacy of therapies for CCSK in the National Wilms' Tumor Study (NWTS)-1, NWTS-2 and NWTS-3 revealed that addition of doxorubicin to the combination of vincristine plus dactinomycin improved the 6-year relapse-free survival rate of this tumor. 3 However, about one-third of children with CCSK died due to treatment failure. The prognosis for relapsed cases is very poor and no convincing treatment strategy has been thus far established. 4 We present two patients with CCSK relapsing during the treatment of NWTS-3 who received intensive treatments including double autografts. They have remained in second remission longer than first remission.
Case reports
Case 1A 22-month-old boy presented in April 1992 with a history of vomiting and abdominal distention. A CT scan showed a large mass in the left renal fossa. Bone marrow aspiration, CT scan of the lung and brain, and bone scintigrams showed no metastases. A left radical nephrectomy was carried out and the resected kidney contained a large tumor which weighed 710 g and measured 11.2 × 11 × 8.9 cm. Microscopic examination showed nests or cords of cells separated by a network of small capillaries. The nuclei of the tumor cells were finely granular, imparting an 'empty' appearance compatible with CCSK.