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Nephroblastic Tumors

  • Living reference work entry
  • First Online:
Encyclopedia of Pathology

Part of the book series: Encyclopedia of Pathology ((EP))

  • 36 Accesses

Definition

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

Synonyms

Wilms tumor

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

  • Beckwith, J. B., Kiviat, N. B., & Bonadio, J. F. (1990). Nephrogenic rests, nephroblastomatosis, and the pathogenesis of Wilms’ tumor. Pediatric Pathology, 10, 1–36.

    Article  CAS  Google Scholar 

  • 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.

    Article  CAS  Google Scholar 

  • 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.

    Article  CAS  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Diagnostic and Public Health, Section of Pathology, University of Verona, Verona, Italy

    Anna Caliò & Guido Martignoni

  2. Department of Pathology, Pederzoli Hospital, Peschiera del Garda, Peschiera del Garda, Italy

    Diego Segala & Guido Martignoni

Authors
  1. Anna Caliò
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  2. Diego Segala
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  3. Guido Martignoni
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Corresponding author

Correspondence to Guido Martignoni .

Editor information

Editors and Affiliations

  1. NIJMEGEN, The Netherlands

    J.H.J.M. van Krieken

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Caliò, A., Segala, D., Martignoni, G. (2019). Nephroblastic Tumors. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_4861-1

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  • DOI: https://doi.org/10.1007/978-3-319-28845-1_4861-1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

  • eBook Packages: Springer Reference MedicineReference Module Medicine

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