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.
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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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
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