title: Characterization of adolescent and pediatric renal cell carcinoma: A report from the Children's Oncology Group study AREN03B2.
abstract: BACKGROUND
  The current study was conducted to characterize the epidemiology, histology, and radiographic features of as well as the surgical approach to pediatric and adolescent renal cell carcinoma (pRCC).
  
  
  METHODS
  pRCC cases prospectively enrolled on the Children's Oncology Group study AREN03B2 underwent central pathology, radiology, surgery, and oncology review.
  
  
  RESULTS
  As of June 2012, 120 of a total of 3250 patients enrolled on AREN03B2 (3.7%) were found to have unilateral RCC (median age, 12.9 years [range, 1.9-22.1 years]; 52.5% were female). Central review classified these as translocation morphology (56 patients), papillary (20 patients), renal medullary carcinoma (13 patients), chromophobe (4 patients), oncocytoma (1 patient), conventional clear cell (1 patient), and RCC not otherwise specified (25 patients). Lymph node (LN) involvement (N+) was found in 35 of 73 cases (47.9%) for which LNs were sampled, including 19 of 40 cases with primary tumors measuring <7 cm (47.5%). Using a size cutoff of 1 cm, imaging detection of LN involvement had a sensitivity of 57.14% (20 of 35 cases; 95% CI, 39.35%-73.68%) and a specificity of 94.59% (35 of 37 cases; 95% CI, 81.81%-99.34%). Distant metastases were present in 23 cases (19.2%). Initial surgery was radical nephrectomy in 88 patients (73.3%), nephron-sparing surgery in 18 patients (15.0%), and biopsy in 14 patients (11.7%). Compared with patients undergoing radical nephrectomy, those treated with nephron-sparing surgery were less likely to have LNs sampled (6 of 18 patients [33.3%] vs 65 of 88 patients [73.9%]; P = .002).
  
  
  CONCLUSIONS
  Translocation RCC is the most common form of pediatric and adolescent RCC. Lymph node disease is common and observed among patients with small primary tumors. Imaging has a high specificity but relatively low sensitivity for the detection of such lymph node disease. Failure to sample LNs results in incomplete staging and potentially inadequate disease control for younger patients with RCC.
authors.affiliation: Division of Pediatric Oncology, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio.
authors.forename: James I
authors.initials: JI
authors.lastname: Geller
authors.affiliation: Section of Pediatric Surgery, Department of Surgery, CS Mott Children's Hospital, University of Michigan School of Medicine, Ann Arbor, Michigan.
authors.forename: Peter F
authors.initials: PF
authors.lastname: Ehrlich
authors.affiliation: Division of Urology, Department of Surgery, University of Colorado School of Medicine and the Children's Hospital Colorado, Aurora, Colorado.
authors.forename: Nicholas G
authors.initials: NG
authors.lastname: Cost
authors.affiliation: Mallinckrodt Institute of Radiology, Washington University School of Medicine, St. Louis, Missouri.
authors.forename: Geetika
authors.initials: G
authors.lastname: Khanna
authors.affiliation: Department of Pediatric Oncology, Children's Hospital Boston/Dana-Farber Cancer Institute, Boston, Massachusetts.
authors.forename: Elizabeth A
authors.initials: EA
authors.lastname: Mullen
authors.affiliation: Division of Pediatric Hematology/Oncology, T.C. Thompson Children's Hospital, University of Tennessee College of Medicine-Chattanooga, Chattanooga, Tennessee.
authors.forename: Eric J
authors.initials: EJ
authors.lastname: Gratias
authors.affiliation: Department of Biostatistics, University of Florida, Children's Oncology Group Statistics and Data Center, Gainesville, Florida.
authors.forename: Arlene
authors.initials: A
authors.lastname: Naranjo
authors.affiliation: Division of Pediatric Oncology, Children's National Medical Center, Washington, DC.
authors.forename: Jeffrey S
authors.initials: JS
authors.lastname: Dome
authors.affiliation: Department of Pathology, Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors.forename: Elizabeth J
authors.initials: EJ
authors.lastname: Perlman
mesh_terms: D000293:Adolescent; D002292:Carcinoma, Renal Cell / Q000473:pathology* / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007680:Kidney Neoplasms / Q000473:pathology* / Q000601:surgery*; D008207:Lymphatic Metastasis; D008297:Male; D009367:Neoplasm Staging; D009392:Nephrectomy; D012680:Sensitivity and Specificity; D016896:Treatment Outcome; D055815:Young Adult
keywords: adolescent; lymph node; pediatric; translocation renal cell carcinoma
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