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Urologic Tumors in Childhood: Nephroblastoma and Wilms Tumor

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Urologic Oncology

Abstract

Around 6–7% of all childhood cancers are renal tumors with almost 90% of them are nephroblastoma – the so called Wilms tumor. Females are slightly more effected than males and 75% of the patients are diagnosed before the age of 5 years, 15% are younger than one year and up to 2% of WT develop in adult, whereas bilateral WT are found in up to 10% and up to 15% are metastasized at the time of diagnosis. In around 10% WT is associated with congenital malformations or syndromes such as the Denis-Drash Syndrom, WAGR-syndrome or the Beckwith-Wiedemann syndrome.

The ultrasound is the first imaging modality. Magnet Resonance Imaging (MRI) is the first choice after ultrasound, as it avoids ionizing radiation and gives an excellent soft tissue contrast an a computer tomography should be only performed If an MRI could not be performed within an acceptable timeframe.

In the current SIOP RTSG UMBRELLA protocol All patients between the age of 7 months and 16 years at the time of diagnosis and with the radiological suspicion of a WT receive chemotherapy before surgery. In the first 2 months of life the prevalence of congenital mesoblastic nephroma (CMN) is higher compared to WT. However, thereafter the percentage of WT increases rapidly and bilateral tumors below the age of 6 months are usually WT. The best therapeutic approach in infants should be discussed within a multidisciplinary team to weight out the risk of intraoperative tumor rupture during primary surgery (e.g. in WT) versus the risk of unnecessary chemotherapy (e.g. in CMN). In adult patients with a WT using the paediatric protocols improved the survival rate of up to 90%. Surgery should be performed an experienced surgeon and should includ lymph node sampling of at least seven or more lymph nodes to guarantee a precise postoperative staging. Due to the excellent imaging modalities today, exploration of the contralateral kidney is no longer necessary. Nephron sparing surgery should be performed in bilateral WT and unilateral syndromic and patients with smaller tumors (e.g. < 300 ml at diagnosis) may benefit from NSS.

Patients receiving an adequate stage and risk group-oriented treatment are cured in 90%, Those with metastasis have a 80% survival rate.

The WT is a great example, how multidisciplinary treatment mad e a former lethal tumor to a curable tumor.

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Authors and Affiliations

  1. Department of Paediatric Adolescent and Reconstructive Urology, University Medical Centre Mannheim, Mannheim, Germany

    Raimund Stein

  2. Department of Paediatric Oncology and Haematology, Saarland University, Homburg, Germany

    Norbert Graf

Authors
  1. Raimund Stein
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  2. Norbert Graf
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Correspondence to Raimund Stein .

Editor information

Editors and Affiliations

  1. Urologie, Univ Lübeck, Lübeck, Schleswig-Holstein, Germany

    Axel Merseburger

  2. Dept. of Urology, University of Regensburg St. Josef Medical Centre, Regensburg, Germany

    Maximilian Burger

Section Editor information

  1. Universitätsklinikum Rostock, Urologische Klinik und Poliklinik, Rostock, Germany

    Chris Protzel

  2. Division of Oncological Urology, Department of Urology, University of Köln, Köln, Germany

    Axel Heidenreich

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Stein, R., Graf, N. (2019). Urologic Tumors in Childhood: Nephroblastoma and Wilms Tumor. In: Merseburger, A., Burger, M. (eds) Urologic Oncology. Springer, Cham. https://doi.org/10.1007/978-3-319-42603-7_43-1

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  • DOI: https://doi.org/10.1007/978-3-319-42603-7_43-1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-42603-7

  • Online ISBN: 978-3-319-42603-7

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