In this study, Selle and colleagues out of Germany report on their 25 year experience with renal cell carcinoma (RCC) in the pediatric population.

The incidence of RCC in children is 2.2 per million, and these tumors represent only 1.9-6.0% of pediatric malignancies. Here, the authors report on 49 patients less than 16 years of age that were treated for RCC at multiple institutions throughout Germany. Mean patient age was 10.6 years (range 1.2-15.9). One third of patients had underlying disorders that have been associated with RCC in previous studies. A significant number of patients presented with symptoms related to their RCC: 53% had pain, 30% had hematuria, 12.5% had a palpable mass, 5% had dysuria, 2.5% had urinary retention, and 42.5% had generalized symptoms (malaise, fever, weight loss, etc.). Only 15% presented with no symptoms were their tumors were found on imaging studies obtained for other reasons. Regarding histology, 33% had papillary, 22% had translocation carcinomas, 16% had unclassified, only 6% had clear cell, 4.1% had chromophobe, and 2% had pure sarcomatoid. There were also a number of very rare variants. In examining stage, 69% were localized, 16% had nodal metastases, and 8% had distant metastases. At 5 years, the median progression free survival and overall survival was 96% and 96%, respectively, for localized, 69% and 75%, respectively, for node positive, and 25% and 33%, respectively, for patients with distant metastases (p=0.004). The progression free and overall survival for the entire population was 84.8%. Only tumor stage correlated with outcome.

RCC is a rare tumor in the pediatric population that displays histology and outcomes that are divergent from that seen in older patients. Male patients older than 10 are more likely to have a papillary tumor, whereas females less than 10 are more likely to harbor a translocation tumor. Clear cell tumors, common in adults, are quite rare in children. Overall, outcomes are good, even in patients with locally advanced disease and nodal metastases, with aggressive treatment approaches that include surgical resection.

Barbara Selle, Rhoikos Furtwängler, Norbert Graf, Peter Kaatsch, Elisabeth Bruder, Ivo Leuschner

Cancer : 107(12): 2906-2914.

Written by Christopher G. Wood, MD, a Contributing Editor with UroToday.

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