Clear cell sarcoma kidney: Clinical features and outcome

Clear cell sarcoma of kidney (CCSK) is a rare, highly malignant tumor. The clinical features and treatment outcome of 12 patients with CCSK are report ed. From 1982 through December 1996, 12 cases of CCSK were seen at the Regi onal Cancer Centre, Trivandrum, India. Patients were staged according to NW TS III recommendation. They were treated with chemotherapy containing vincr istine, actinomycin, and Adriamycin and radiotherapy. The survival curve wa s calculated by the Kaplan-Meier method. Mass and pain in the abdomen were Me presenting symptoms. Male/female ratio was 3:1. Six had stage 1, 4 had s tage II; and 2 had stage III disease. Of the 12, 10 were evaluable, 6 are a live, and 3 recurred in 9 evaluable. Six patients are alive free of disease 10 to 108 months after diagnosis. The overall survival and disease-free su rvival of the 10 patients are 64 and 56%. It would appear that combined mod ality treatment can cure two thirds of children with CCSK. Effective treatm ent needs to be developed for children who Sail after first line treatment.