Synonyms
Wilms tumor
Definition
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
Beckwith, J. B., Kiviat, N. B., & Bonadio, J. F. (1990). Nephrogenic rests, nephroblastomatosis, and the pathogenesis of Wilms’ tumor. Pediatric Pathology, 10, 1–36.
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.
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.
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Caliò, A., Segala, D., Martignoni, G. (2020). Nephroblastic Tumors. In: Raspollini, M.R., Lopez-Beltran, A. (eds) Uropathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-41894-6_4861
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DOI: https://doi.org/10.1007/978-3-030-41894-6_4861
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