2020 Edition
| Editors: Maria Rosaria Raspollini, Antonio Lopez-Beltran

Nephroblastic Tumors

  • Anna Caliò
  • Diego Segala
  • Guido MartignoniEmail author
Reference work entry
DOI: https://doi.org/10.1007/978-3-030-41894-6_4861


Wilms tumor


Nephroblastic tumors include nephroblastoma, the most common, and cystic partially differentiated nephroblastoma.

Clinical Features

  • Incidence

    Nephroblastoma is a malignant tumor which comprises more than 85% of pediatric renal tumors and 5% of childhood cancers.

  • Age

    It is usually found in children 2–4 years old, it is relatively uncommon in the first 6 months of life and in children older than 6 years, whereas it is rare in the neonatal period. Nephroblastoma rarely occurs in adults.

  • Sex

    There is no gender predilection.

  • Site

    Five percent of the tumors are multicentric and bilateral.

  • Treatment

    Tumor resection followed by chemotherapy or adjuvant chemotherapy and surgical treatment are the two different protocols of therapy.

  • Outcome

    Despite the different managements, the overall survival currently is more than 90%.

The associations with cryptorchidism, hypospadias, other genital anomalies, hemihypertrophy, and aniridia are well-recognized and described in...

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References and Further Reading

  1. Beckwith, J. B., Kiviat, N. B., & Bonadio, J. F. (1990). Nephrogenic rests, nephroblastomatosis, and the pathogenesis of Wilms’ tumor. Pediatric Pathology, 10, 1–36.CrossRefGoogle Scholar
  2. Faria, P., Beckwith, J. B., Mishra, K., et al. (1996). Focal versus diffuse anaplasia in Wilms tumor – new definitions with prognostic significance: A report from the National Wilms Tumor Study Group. The American Journal of Surgical Pathology, 20, 909–920.CrossRefGoogle Scholar
  3. Huang, C. C., Gadd, S., Breslow, N., et al. (2009). Predicting relapse in favorable histology Wilms tumor using gene expression analysis: A report from the Renal Tumor Committee of the Children’s Oncology Group. Clinical Cancer Research, 15, 1770–1778.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of Diagnostic and Public Health, Section of PathologyUniversity of VeronaVeronaItaly
  2. 2.Department of PathologyPederzoli HospitalPeschiera del GardaItaly