Renal cell carcinoma: Children's Hospital Boston experience

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.