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Renal Cell Carcinoma in the Pediatric Population: A 12-year experience
ELIAS WEHBI, MD, NIKI KANAROGLOU, MD, MARTIN KOYLE, MD, DARIUS BAGLI, MD, WALID FARHAT, MD, JOAO LUIZ PIPPI SALLE, MD, RONALD GRANT, MD, ARMANDO LORENZO, MD.
Hospital for Sick Children - Toronto, Toronto, ON, Canada.
The most common renal malignancy in the second decade of life is renal cell carcinoma (RCC). Although overall incidence is rare in children, with approximately 2-5% of RCCs occurring in the pediatric population, it is a poorly understood entity with considerably different pathobiology than its adult counterpart. There is a higher proportion of papillary RCC and recent data has shown that many of these tumours have a predisposition for translocation involving transcription factor E3 (TFE3) gene at Xp11.2. Here we describe our experience with RCC over a 12 year period.
We retrospectively reviewed all renal tumours at our institution from January 2000 to April 2012 and assessed patient characteristics, stage at presentation and overall survival.
154 patients with renal masses were reviewed, of whom 21 (13.6%) were diagnosed with RCC. Of these, 19 patients with complete charts were included.
Male to Female ratio was 1:1.1, with a median age at presentation of 12.7 years and a median follow up of 29 months. The most common presentation was abdominal mass with or without pain followed by hematuria, which is known to occur more commonly with RCC than with Wilm’s tumour. Only 1 patient had an associated syndrome, Von Hippel-Lindau. Of those patients with papillary RCC (11/19, 58%), 5 had tumours with associated translocation Xp11.2. There were only 3 patients with clear cell type. Below is a table of stage at presentation. Three patients died from their disease (3/19, 16%), all of whom had Stage IV disease at presentation. One of these patients had a tumour thrombus on both pre-operative imaging and during intra-operative dissection.
RCC is a relatively rare entity in children with a significant to predisposition to papillary tumour biology. Mortality can be significant with reduced efficacy of radio and chemotherapeutic agents, although a role for tyrosine kinase inhibitors is emerging this groups, Future large, multi-centred data collection is required to better understand this potentially fatal disease in the pediatric population.
Stage at Presentation
|Stage||# of Patients|
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