Characteristics and Outcomes of Paediatric Renal Cell Carcinoma in Hong Kong

Abstract

Purpose/Objective We reviewed the characteristics and outcomes of renal cell carcinoma (RCC) in children in Hong Kong.

Materials and Methods All paediatric cancers diagnosed including RCC in Hong Kong have been prospectively collected by the Hong Kong Paediatric Hematology and Oncology Study Group. This is a review of the clinical data on all children with RCC treated in Hong Kong from 2004 to 2012.

Results Altogether 5 children with RCC were registered. Median age at diagnosis was 13 years (range, 1-17 years). Four patients were boys. They presented with gross hematuria (n=4), abdominal or flank pain (n=2), and palpable abdominal mass (n=2). Two patients had stage I disease, while 1 patient each had stage II, III and IV disease (distant lymph node metastases). Total nephrectomy was performed in all 5 patients. Histology were Xp11.2 translocation RCC (n=2), clear cell RCC (n=2), or papillary RCC (n=1). All 4 patients with stage I-III RCC were disease-free after operation upon follow-up for 0.6-8.9 years. The patient with stage IV translocation RCC received sunitinib after nephrectomy but the metastatic diseases progressed soon and sunitinib was switched to everolimus with stable disease for 4 months. However, he progressed again at 12 months after diagnosis and everolimus was switched to axitinib recently. He is expected to have poor prognosis.

Conclusions RCC is rare in children and hematuria is the commonest presentation. Xp11.2 translocation RCC appears to be more common in children than adults. Prognosis is good for locoregional disease even with surgical resection alone. Metastatic RCC has extremely poor prognosis. Targeted therapy with vascular endothelial growth factor receptor tyrosine kinase inhibitors or mammalian target of rapamycin inhibitors may prolong survival.