Renal cell carcinoma: Children's Hospital Boston experience

Abstract

Objectives: To review our experience to better define pediatric renal cell carcinoma (RCC). Pediatric RCC is rare, and recent data suggest it may be a unique disease.

Methods: A retrospective review was conducted of hospital and pathology records from 1965 to 2003. Patients with RCC were identified, and the clinical and pathologic data were extracted.

Results: Since 1965, 11 patients with RCC were treated, accounting for 3% of all renal tumors. In the first 20 years, 191 patients with renal tumor were treated, of whom 3 had RCC. In the most recent 15 years, 172 patients with renal tumor were treated, of whom 8 had RCC. The mean age at presentation was 14.7 years (range 9 to 17 years), with a female predominance (2.7:1). The clinical signs and symptoms included hematuria in 36%, flank pain in 27%, and an abdominal mass in 9%; 36% were discovered incidentally. Of the 11 RCC tumors, 45% were papillary and 55% were clear cell carcinoma. Papillary tumors presented at a worse stage and displayed more aggressive clinical behavior. Of 10 patients with available follow-up data, 6 had no evidence of RCC, 1 had died of other causes, 2 had died of metastatic RCC, and 1 was alive with recurrent RCC at a mean follow-up of 4.9 years.

Conclusions: The clinical presentation, pathologic characteristics, and clinical behavior of pediatric RCC are different than those for adult RCC. A possible increasing incidence of RCC in children would parallel an increased incidence in adults. Our findings warrant additional and coordinated efforts to better characterize RCC in children.