S. Avčin
Klinični oddelek za hematologijo in onkologijo, Pediatrična klinika, Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija

R. Kordič
Klinični oddelek za urologijo, Kirurška klinika, Univerzitetni Klinični center Ljubljana, Slovenija

J. Blazina
Oddelek za patologijo, Onkološki inštitut, Ljubljana, Slovenija

L. Zadravec Zaletel
Oddelek za radioterapijo, Onkološki inštitut, Ljubljana, Slovenija

M. Debeljak
Klinični oddelek za hematologijo in onkologijo, Pediatrična klinika, Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija

Abstract

Paediatric renal tumours are one of the most common solid tumours in childhood, representing around 6-7% of all paediatric malignancies. They mostly occur in children under 5 years of age. In nephroblastoma, also known as Wilms tumour, the most common renal tumour, 72% of the patients present with unilateral tumour, 10% with local or distant metastases and 7% of the patients present with bilateral disease. Other tumours, comprising rare entities such as congenital mesoblastic nephroma, clear cell sarcoma of the kidney, malignant rhabdoid kidney tumours and renal cell carcinoma, account for the remaining 11%. In our country, the diagnostic workup, treatment and follow-up is performed according to the recommendations of the international committee for paediatric renal tumours, called the SIOP Renal tumour study group. Treatment consists of chemotherapy, surgical excision of the tumour and metastasis and, if indicated, radiotherapy of the tumour bed, the whole abdomen and/or metastases. It is planned based on the patient’s age, disease stage and tumour histology. The overall survival of patients with localised disease is 90% and 75% for patients with metastatic disease. One of the main future directions for the improvement in treatment outcome is to tailor the treatment strategy with the inclusion of new insights into biological tumour characteristics, especially for the patients with unfavourable histology and/or relapse.

Key words: paediatric renal tumours, nephroblastoma, workup, treatment.