Renal Cell Carcinoma in Childhood and Adolescence: A Clinical and Pathological Study of 17 Cases

Abstract

From 1955 through 1984, 17 patients aged 17 yr or younger were treated or seen in consultation for renal cell carcinoma at the Children''s Hospital and Dana Farber Cancer Institute. There were 11 girls and 6 boys, with an average age at diagnosis of 11 yr (range 4-17 yr). Gross hematuria and abdominal or flank pain were the most common presenting complaints, and in 5 cases there was a history of antecedent trauma. The tumors ranged in size from 2-15 cm (average diameter 7.5 cm). There were 10 tumors in the left kidney and 7 in the right kidney. Childhood renal cell carcinoma displayed similar gross and microscopic features compared with counterparts in adults. The smallest renal cell carcinoma to metastasize measured 3 cm in diameter and the pathological features suggested an origin from distal collecting tubules (ducts of Bellini). There were 6 tumor-related deaths (35% overall mortality) occuring 2 mo. to 2 yr after diagnosis. Eleven children were well, with an average duration of followup of 12 yr (range 4-27 yr). The most important prognostic factor was clinical stage at the time of diagnosis. The presence of renal vascular invasion per se did not appear to be an adverse finding. The most optimal treatment for children with stage I renal cell carcinoma is radical nephrectomy with regional lymphadenectomy. While adjuvant radiation and/or chemotherapy may be used in patients with more advanced stage disease, their efficacy in achieving long-term survival free of relapse or cure is not well defined.