Renal cell carcinoma in childhood and adolescence: a clinical and pathological study of 17 cases.
From 1955 through 1984, 17 patients aged 17 years 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 years (range 4 to 17 years). 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 to 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 per cent over-all mortality) occurring 2 months to 2 years after diagnosis. Eleven children were well, with an average duration of followup of 12 years (range 4 to 27 years). 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.
D000293:Adolescent; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002292:Carcinoma, Renal Cell / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D002648:Child; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007668:Kidney / Q000000981:diagnostic imaging / Q000473:pathology; D007680:Kidney Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D008197:Lymph Node Excision; D008207:Lymphatic Metastasis; D008297:Male; D009367:Neoplasm Staging; D009392:Nephrectomy; D011859:Radiography; D011878:Radiotherapy; D012086:Reoperation
D000970:Antineoplastic Agents