Renal cell carcinoma in children under 14 years old: long-term survival.

Abstract

To assess the treatment and long-term survival of children under 14 years old operated upon for renal cell carcinoma (RCC). The records of five patients (aged 2, 4, 6, 9 and 13 years) who had been treated for RCC between 1982 and 1993 were reviewed. All had undergone nephrectomy with (two) or without (three) regional lymphadenectomy. After surgery four patients received chemotherapy (vincristine with actinomycin-D, three, vinblastine, one), and two with advanced-stage disease received other agents (interferon alpha, interleukin-2). They were followed for 5 to 15 years. Three patients with stage I-II RCC were well at 13, 15 and 15 years after treatment. Of the two patients with regional lymph node involvement (stage III), one was well 5 years after surgery while the other developed hepatic, lung and bone metastases, and died 2 years after presentation. The prognosis and long-term survival of children under 14 years old with RCC is very good for those with low-stage tumours. Nephrectomy with complete tumour extirpation is the main mode of treatment. For advanced-stage tumours, the role of adjuvant chemotherapy and/or biological agents must be defined more clearly.