Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken

Rhabdoid Tumor

  • Anna Caliò
  • Diego Segala
  • Guido MartignoniEmail author
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_3482-1

Definition

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.

Macroscopy

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

Microscopy

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).

Immunophenotype

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.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

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