Malignant rhabdoid tumor: Difference between revisions
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==Overview== | ==Overview== | ||
*Malignant rhabdoid tumor is one of the aggressive tumors in the pediatric population. It was originally described as a rhabdomyosarcomatoid variant of [[Wilms' tumor]], which is primarily a | *Malignant rhabdoid tumor is one of the aggressive tumors in the pediatric population. It was originally described as a rhabdomyosarcomatoid variant of [[Wilms' tumor]], which is primarily a renal tumor.<ref name=intromalignnat1>Malignant rhabdoid tumour. Wikipedia 2015. https://en.wikipedia.org/wiki/Malignant_rhabdoid_tumour. Accessed on December 22, 2015</ref> | ||
*Malignant rhabdoid tumor was first described as a variant of [[Wilms' tumor]] of the kidney, in 1978. | *Malignant rhabdoid tumor was first described as a variant of [[Wilms' tumor]] of the kidney, in 1978. | ||
*Malignant rhabdoid tumor is comprised of rhabdoid tumor cells and varying amounts of small undifferentiated primitive neuroectodermal tumor (PNET)-like, mesenchymally, and/or epithelially differentiated tumor cells. | *Malignant rhabdoid tumor is comprised of rhabdoid tumor cells and varying amounts of small undifferentiated primitive neuroectodermal tumor (PNET)-like, mesenchymally, and/or epithelially differentiated tumor cells. | ||
Revision as of 14:57, 23 December 2015
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
Synonyms and keywords: Malignant rhabdoid tumor; Malignant rhabdoid neoplasm; Atypical teratoid rhabdoid tumor
Overview
- Malignant rhabdoid tumor is one of the aggressive tumors in the pediatric population. It was originally described as a rhabdomyosarcomatoid variant of Wilms' tumor, which is primarily a renal tumor.[1]
- Malignant rhabdoid tumor was first described as a variant of Wilms' tumor of the kidney, in 1978.
- Malignant rhabdoid tumor is comprised of rhabdoid tumor cells and varying amounts of small undifferentiated primitive neuroectodermal tumor (PNET)-like, mesenchymally, and/or epithelially differentiated tumor cells.
- Gene involved in the pathogenesis of malignant rhabdoid tumor include SMARCB1 (hSNF5/INI-1), a tumor suppressor gene.
- Malignant rhabdoid tumor is characterized by loss of the long arm of chromosome 22, which results in loss of the hSNF5/INI-1 gene.
- INI1, a member of the SWI/SNF chromatin remodeling complex, is important in maintenance of the mitotic spindle and cell cycle control.
- Malignant rhabdoid tumor may be associated with rhabdoid predisposition syndrome.[2]
- Common locations associated with malignant rhabdoid tumor include:[3]
- Kidney: malignant rhabdoid tumor of the kidney (most common)
- Intracranial: atypical teratoid rhabdoid tumor
- Soft tissues: breast, skin, orbit, liver, etc
- Almost all reported cases of malignant rhabdoid tumor, regardless of site of origin, occur in young children between birth and 2 years of age.[4]
- In approximately 10% of cases of renal malignant rhabdoid tumor, the patients subsequently develop intracranial atypical teratoid rhabdoid tumor.
References
- ↑ Malignant rhabdoid tumour. Wikipedia 2015. https://en.wikipedia.org/wiki/Malignant_rhabdoid_tumour. Accessed on December 22, 2015
- ↑ Genetics of malignant rhabdoid tumour. Wikipedia 2015. https://en.wikipedia.org/wiki/Malignant_rhabdoid_tumour. Accessed on December 22, 2015
- ↑ Rhabdoid tumours. Dr Ayush Goel and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/rhabdoid-tumours. Accessed on December 22, 2015
- ↑ Epidemiology of rhabdoid tumours. Dr Ayush Goel and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/rhabdoid-tumours. Accessed on December 22, 2015