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

Rhabdoid Tumor

  • Anna Caliò
  • Diego Segala
  • Guido MartignoniEmail author
Reference work entry
DOI: https://doi.org/10.1007/978-3-030-41894-6_3482


Rhabdoid tumor of the kidney is a highly invasive and highly lethal neoplasm of young children with genetic abnormalities of SMARCB1/INI1 tumor suppressor gene.

Clinical Features

  • Incidence

    It comprises 2% of pediatric renal neoplasms.

  • Age

    Ninety percent of the cases occurs in patients before 3 years of age.

  • Sex

    ts male-to-female ratio is 1.5:1.

  • Site

    There is no site predilection.

  • Treatment

    There is no standard treatment option for rhabdoid tumor of the kidney.

  • Outcome

    The patients frequently die within 1 year of diagnosis. Hematuria and abdominal mass are the most common presentations.


Tumors are typically large, hemorrhagic, and necrotic, with ill-defined borders (Weeks et al. 1989).


It is an infiltrative, necrotic hemorrhagic tumor composed of sheets of large discohesive polygonal cells with large vesicular nuclei showing prominent central nucleoli and hyaline pink cytoplasmic inclusions (Weeks et al. 1989).


Loss of INI1 protein by...

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References and Further Reading

  1. Eaton, K. W., Tooke, L. S., Wainwright, L. M., et al. (2011). Spectrum of SMARCB1/INI1 mutations in familial and sporadic rhabdoid tumors. Pediatric Blood & Cancer, 56, 7–15.CrossRefGoogle Scholar
  2. Weeks, D. A., Beckwith, J. B., Mierau, G. W., & Luckey, D. W. (1989). Rhabdoid tumor of kidney. A report of 111 cases from the National Wilms’ Tumor Study Pathology Center. The American Journal of Surgical Pathology, 13, 439–458.PubMedCrossRefGoogle 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