Selle B, Furtwangler R, Graf N, et al. Population-based study of renal cell carcinoma in children in Germany, 1980-2005: more frequently localized tumors and underlying disorders compared with adult counterparts

Department of Pediatric Hematology and Oncology, St. Annastift Children's Hospital, Ludwigshafen, Germany.
Cancer (Impact Factor: 4.89). 01/2007; 107(12):2906-14. DOI: 10.1002/cncr.22346
Source: PubMed


Childhood renal cell carcinomas (RCCs) differ histologically and biologically from their adult counterparts. The characteristics of RCC-affected children and their tumors, the influence of treatment, and outcome have so far not been studied in a nonselected, population-based cohort.
A retrospective analysis was undertaken of RCC patients less than 16 years old reported to the German Childhood Cancer Registry and to the Kiel Paediatric Tumor Registry from 1980 to 2005.
Forty-nine RCC in children (24 boys, 25 girls) with a median age of 10.6 years were identified. In about every third child possibly RCC-related underlying disorders (tuberous sclerosis, neuroblastoma, teratoma with chemotherapy, Saethre-Chotzen syndrome, chronic renal failure) or related diseases in their family were found. The pathologic subtypes were papillary in 16 (33%), translocation type in 11 (22%), unclassified in 8 (16%), and rarely clear-cell (n = 3) or others. Thirty-four (69%) patients had localized RCC, 8 (16%) patients regional lymph node metastases, and 4 (8%) patients distant metastases. Event-free survival and overall survival rates at 5 years were 96% for localized RCC, 69% and 75% for regional lymph node-positive, 25% and 33% for distant metastatic RCC, respectively. Two of 4 patients with distant metastases received immunotherapy combined with chemotherapy and surgery, both are alive, 1 of them disease-free for 6.9 years.
Pediatric RCCs are predominantly localized diseases. Children with RCC frequently suffer underlying disorders. Survival rates in localized and regional lymph node-positive cases are favorable. Because of the rarity of RCC in childhood, an international study is necessary.

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Available from: Norbert Graf, Oct 04, 2015
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    • "It may be associated to the von Hippel–Lindau syndrome, in which the tumors are multiple and have an earlier occurrence (2). One population based study showed about 30% of cases associated to underlying disorders, like tuberous sclerosis, neuroblastoma, teratoma, Saethre–Chotzen syndrome, chronic renal failure, or related diseases in their family (30). "
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    ABSTRACT: Tumors of the kidney, bladder, prostate, testis, and adrenal represent a large part of the adult urologic practice, but are relatively infrequent in children. The natural history and management of these tumors in the pediatric age is different from that of the adults. As result of the successful work of several clinical trial groups in recent decades, there has been a significant improvement in their cure rates. The aim of this article is to review their most significant clinical aspects, as well as to present an update in their management.
    Frontiers in Pediatrics 12/2013; 1:48. DOI:10.3389/fped.2013.00048
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    • "Study T1 T2 T3 T4 Nþ Mþ Selle et al. [1] "
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    ABSTRACT: Renal cell carcinoma (RCC) comprises about 5% of pediatric renal neoplasms. It has been recognized as a second malignancy in multiple reports. It is generally symptomatic at diagnosis, and most children with RCC present with more locally advanced disease than do adults. Contemporary investigation of pediatric RCC has demonstrated that a large percentage of these tumors bear cytogenetic translocations involving the MiT family of transcription factors. Surgical therapy for these children resembles operative intervention for adult RCC, though debate continues about the precise role of lymph node dissection. There are no adequately powered studies to support conclusions about adjuvant or neoadjuvant chemotherapy for children with RCC. This may be ameliorated by a multi-institutional protocol which is enrolling patients.
    Journal of pediatric urology 06/2009; 5(4):308-14. DOI:10.1016/j.jpurol.2009.04.007 · 0.90 Impact Factor
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    • "Study T1 T2 T3 T4 Nþ Mþ Selle et al. [1] "
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    ABSTRACT: Renal cell carcinoma in children and adolescents is rare and comprises only about 1% of renal tumors in this age group. Since the last WHO classification in 1997, new entities of renal tumors in young patients have been described and have been included into the new 2004 WHO renal cell carcinoma classification. Renal cell carcinoma associated with neuroblastoma comprises 2.5% of renal cell carcinoma in young patients. It occurs several years after neuroblastoma. A large proportion of these tumors show allelic imbalance of chromosomes 20q13, 2p31-32.2, 13q22 and 14q31. TFE3-translocation carcinomas correspond to approximately 20% of renal cell carcinomas in the pediatric and adolescent age group. Both translocations t(X;17)(p11.2;q25) and t(X;1)(p11.2;q21.2) result in immunohistochemically detectable TFE3 protein overexpression. By conventional morphology, TFE3-translocation carcinomas typically show prominent "voluminous" clear cytoplasm and partially papillary architecture. Even according to the revised 2004 WHO classification, in children and adolescents, far more renal cell carcinomas than in the adult age group are currently not classifiable but constitute a phenotypically heterogeneous group with ample potential for future renal cell carcinoma subtypes.
    Der Pathologe 08/2004; 25(4):324-7. · 0.39 Impact Factor
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