Pediatric Extra Renal Reno cellular Carcinoma with Xp11 Translocation Responding to Sunitinib, Everolimus and Metronomic Chemotherapy

István Szegedi, Gábor Méhes, Imre Gáspár, Tamás Csonka, István Csízy and Csongor Kiss

Department of Pediatrics, University of Debrecen, Hungary

^*Correspondance to: Istv�n Szegedi 

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Abstract:

Background: Fewer than 2% of renal cell carcinomas (RCC) develop in childhood, making up less than 0.1-0.3% of all pediatric tumors and 2.6% of renal neoplasms. The 2004 WHO classification defined RCC with Xp11.2 aberration as a separate entity accumulating characteristically in childhood. Ectopic development of pediatric renal neoplasms is rare and there are no data in the literature about the extra renal origin of RCC.Case
Presentation: We describe the case of a 16-year old boy with an extra renal RCC showing Xp11.2 translocation demonstrated by FISH. The tumor exhibited a favourable response to a combination of targeted treatment with sunitinib and everolimus in association with metronomic chemotherapy.Conclusion: The novelty of the reported case, in addition to the extra renal origin of the tumor, is the favourable response to a combination of sunitinib, everolimus and metronomic chemotherapy resulted in a major partial control of advanced RCC accompanied by a 15 months survival from diagnosis without major toxicities

Keywords:

Renal cell carcinoma; pediatric; Extra renal; Xp11 translocation; Targeted therapy; Metronomic treatment

Cite the Article:

Szegedi I, Méhes G, Gáspár I, Csonka T, Csízy I, Kiss C. Pediatric Extra Renal Reno cellular Carcinoma with Xp11 Translocation Responding to Sunitinib, Everolimus and Metronomic Chemotherapy. Clin Oncol. 2018; 3: 1458.