Rhabdoid Tumor

Caliò, Anna; Segala, Diego; Martignoni, Guido

doi:10.1007/978-3-030-41894-6_3482

Anna Caliò⁴,
Diego Segala⁵ &
Guido Martignoni^4,5

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

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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.
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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.
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Authors and Affiliations

Department of Diagnostic and Public Health, Section of Pathology, University of Verona, Verona, Italy
Anna Caliò & Guido Martignoni
Department of Pathology, Pederzoli Hospital, Peschiera del Garda, Italy
Diego Segala & Guido Martignoni

Authors

Anna Caliò
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Diego Segala
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Guido Martignoni
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Corresponding author

Correspondence to Guido Martignoni .

Editor information

Editors and Affiliations

Histopathology and Molecular Diagnostics, University Hospital Careggi, Florence, Italy
Maria Rosaria Raspollini
Pathology Service, Champalimaud Clinical Center, Lisbon, Portugal
Antonio Lopez-Beltran
Unit of Anatomic Pathology, Department of Surgery, Cordoba University Medical School, Cordoba, Spain
Antonio Lopez-Beltran

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Caliò, A., Segala, D., Martignoni, G. (2020). Rhabdoid Tumor. In: Raspollini, M.R., Lopez-Beltran, A. (eds) Uropathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-41894-6_3482

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DOI: https://doi.org/10.1007/978-3-030-41894-6_3482
Published: 19 June 2020
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-41893-9
Online ISBN: 978-3-030-41894-6
eBook Packages: MedicineReference Module Medicine

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