Wilms tumour (Nephroblastoma) and other kidney tumours

Wilms tumour (Nephroblastoma) is by far the most frequent kidney tumour in children and adolescents. Other kidney tumours, benign or malignant, are very rare. This text particularly provides information on Wilms tumour.

Author:  Maria Yiallouros, Reviewer:  Prof. Dr. med. Norbert Graf, English Translation:  Dr. med. habil. Gesche Tallen, Last modification: 2022/09/23 https://kinderkrebsinfo.de/doi/e77090

About 100 to 130 children and adolescents are newly diagnosed with a kidney tumour in Germany per year. These tumours account for 5.6 % or 4.6 % of all childhood cancers, depending on the age group that is considered (patients from 0-14 years or patients from 0-17 years, respectively). Children at the age of two and three years are most frequently affected.

Nephroblastoma, also known as Wilms tumour, accounts for 85 % of childhood kidney tumours, thereby representing the most frequent type. Much less frequent, with an incidence of 2-5 % each, are clear cell sarcomas of the kidney and mesoblastic nephromas. For patients with clear cell sarcoma, the mean age at diagnosis is about four years; patients with mesoblastic nephroma are in average less than four weeks old at diagnosis. Rhabdoid tumour of the kidney occurs mostly during the first year of life and may present with lung metastases already at diagnosis. Even more rare in childhood are kidney carcinomas, which mostly affect children older than 10 years. Other rare kidney tumours are angiomyolipoma, primary synovial sarcoma of the kidney, renal soft tissue sarcoma and renal Ewing sarcoma.

The following information is on the most frequent kidney tumour, the nephroblastoma or Wilms tumour.

Literature

  1. Erdmann F, Kaatsch P, Grabow D, Spix C: German Childhood Cancer Registry - Annual Report 2019 (1980-2018). Institute of Medical Biostatistics, Epidemiology and Informatics (IMBEI) at the University Medical Center of the Johannes Gutenberg University Mainz 2020 [URI: https://www.kinderkrebsregister.de/ typo3temp/ secure_downloads/ 42507/ 0/ 1c5976c2ab8af5b6b388149df7182582a4cd6a39/ Buch_DKKR_Jahresbericht_2019_komplett.pdf] ERD2020
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  3. Graf N: Nephroblastom (Wilms-Tumor). S1-Leitlinie 025/004 AWMF online 2016 [URI: https://www.awmf.org/ uploads/ tx_szleitlinien/ 025-004l_S1_Nephroblastom_Wilms-Tumor_2016-06-abgelaufen.pdf] GRA2016