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Encyclopedia of Pathology pp 1–2Cite as

Rhabdoid Tumor

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

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

  • 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.

    Article  Google Scholar 

  • 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.

    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, 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). Rhabdoid Tumor. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_3482-1

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  • DOI: https://doi.org/10.1007/978-3-319-28845-1_3482-1

  • Received:

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  • Published:

  • 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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