Metastatic Papillary Renal Cell Carcinoma in a 13-Year Old Girl: A Case Report and Review of the Literature Mainly Focusing on Treatment and Prognosis.

INTRODUCTION

Renal cell carcinoma (RCC) accounts for less than 0.3% of all tumours occurring in children and adolescents and it also affects 2.6% of all renal tumours for the pediatriac population. The aim of this report is to present the case of a 13-year old girl with metastatic papillary RCC and to review the literature mainly on treatment modalities and prognosis of children and adolescents with RCC.

PRESENTATION OF CASE

The case of a 13-year old girl is presented. The girl presented with a painless abdominal mass in the right side for three months. Abdominal ultrasound revealed a heterogeneous mass of 15 cm in diameter with metastasis to the liver. Also CT scan of the abdomen and lungs revealed metastasis to the liver and lungs. She underwent radical right nephrectomy.

DISCUSSION

Pediatric RCC is an aggressive malignancy and some series have reported a 50% incidence of metastasis at the point of initial diagnosis similar to our patient who had metastasis to both lungs and liver at the time of initial diagnosis. Over 50% of metastasis of RCC in the pediatric population occurs in the lungs and liver.

CONCLUSION

RCC in children is extremely rare with no known specific treatment regimen. Early diagnosis when the tumour is still confined to the kidney provides better clinical outcomes since radiotherapy, chemotherapy and immunotherapy have not been found to improve the prognosis.