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.
Written by:
Geller JI, Ehrlich PF, Cost NG, Khanna G, Mullen EA, Gratias EJ, Naranjo A, Dome JS, Perlman EJ. Are you the author?
Division of Pediatric Oncology, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio; Section of Pediatric Surgery, Department of Surgery, CS Mott Children's Hospital, University of Michigan School of Medicine, Ann Arbor, Michigan; Division of Urology, Department of Surgery, University of Colorado School of Medicine and the Children's Hospital Colorado, Aurora, Colorado; Mallinckrodt Institute of Radiology, Washington University School of Medicine, St. Louis, Missouri; Department of Pediatric Oncology, Children's Hospital Boston/Dana-Farber Cancer Institute, Boston, Massachusetts; Division of Pediatric Hematology/Oncology, T.C. Thompson Children's Hospital, University of Tennessee College of Medicine-Chattanooga, Chattanooga, Tennessee; Department of Biostatistics, University of Florida, Children's Oncology Group Statistics and Data Center, Gainesville, Florida; Division of Pediatric Oncology, Children's National Medical Center, Washington, DC; Department of Pathology, Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
Reference: Cancer. 2015 Apr 6. Epub ahead of print.
doi: 10.1002/cncr.29368
PubMed Abstract
PMID: 25845370