RENAL CELL CARCINOMA: CHILDREN’S HOSPITAL BOSTON EXPERIENCE CARLOS R. ESTRADA, ANJALI M. SUTHAR, SAMUEL H. EATON, AND BARTLEY G. CILENTO, JR 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. UROLOGY 66: 1296–1300, 2005. © 2005 Elsevier Inc. R enal cell carcinoma (RCC) in children is rare, accounting for 5.9% of pediatric malignant re- nal tumors, 1 but it shares equal prevalence with Wilms’ tumor in the second decade of life. 2 In con- trast, RCC accounts for 90% to 95% of renal neo- plasms in adults and is the sixth leading cause of adult cancer death in the United States. 3,4 The fre- quency of RCC in adults has provided for large clinical series and intensive basic research. Because of the relative rarity of RCC in children, studies matching the complexity and power of adult stud- ies are impossible to conduct. Consequently, pub- lished pediatric RCC reports consist of case series and retrospective analyses of small numbers of patients, with approximately 350 reported cases. Complicat- ing the small number of reports is the inconsistency of their content, including substantial variation in the reported clinical and pathologic characteristics. Recent data have suggested that pediatric RCC may be a different entity from adult RCC, with different clinical presentation and behavior char- acteristics, 1,5–7 unique genetic abnormalities, 5,8 –10 an association with neuroblastoma, 11 and distinct pathologic characteristics. 5,6 Although these data are provocative, pediatric RCC remains under- characterized. We reviewed our experience with RCC from 1965 to 2003 to address this disease in our population. MATERIAL AND METHODS With institutional review board approval, a retrospective review of the hospital and pathology records from 1965 to 2003 was conducted. Admission and discharge records were reviewed for the diagnosis codes for renal tumor. Similarly, the hospital pathology records were examined for the diagno- sis of RCC. The record sets were then cross-referenced. After identification of all renal tumors, complete clinical and patho- logic data were gathered for patients with RCC. Only patients younger than 18 years old were included. The clinical data included age, sex, signs and symptoms, workup findings, sur- This study was presented at the 99th Annual Meeting of the American Urological Association, May 2004. From the Department of Urology, Children’s Hospital Boston and Harvard Medical School, Boston, Massachusetts Reprint requests: Carlos R. Estrada, M.D., Department of Urol- ogy, Children’s Hospital Boston, 300 Longwood Avenue, HU-390, Boston, MA 02115. E-mail: carlos.estrada@childrens.harvard.edu Submitted: March 29, 2005, accepted (with revisions): June 22, 2005 PEDIATRIC UROLOGY CME ARTICLE © 2005 ELSEVIER INC. 0090-4295/05/$30.00 1296 ALL RIGHTS RESERVED doi:10.1016/j.urology.2005.06.104