Cystic nephroma overview: Difference between revisions
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Cystic nephroma is caused by mutations in the [[DICER1]] gene.<ref name="pmid24481001">{{cite journal |vauthors=Doros LA, Rossi CT, Yang J, Field A, Williams GM, Messinger Y, Cajaiba MM, Perlman EJ, A Schultz K, Cathro HP, Legallo RD, LaFortune KA, Chikwava KR, Faria P, Geller JI, Dome JS, Mullen EA, Gratias EJ, Dehner LP, Hill DA |title=DICER1 mutations in childhood cystic nephroma and its relationship to DICER1-renal sarcoma |journal=[[Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc]] |volume=27 |issue=9 |pages=1267–80 |year=2014 |pmid=24481001 |pmc=4117822 |doi=10.1038/modpathol.2013.242 |url=http://dx.doi.org/10.1038/modpathol.2013.242}}</ref> | Cystic nephroma is caused by mutations in the [[DICER1]] gene.<ref name="pmid24481001">{{cite journal |vauthors=Doros LA, Rossi CT, Yang J, Field A, Williams GM, Messinger Y, Cajaiba MM, Perlman EJ, A Schultz K, Cathro HP, Legallo RD, LaFortune KA, Chikwava KR, Faria P, Geller JI, Dome JS, Mullen EA, Gratias EJ, Dehner LP, Hill DA |title=DICER1 mutations in childhood cystic nephroma and its relationship to DICER1-renal sarcoma |journal=[[Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc]] |volume=27 |issue=9 |pages=1267–80 |year=2014 |pmid=24481001 |pmc=4117822 |doi=10.1038/modpathol.2013.242 |url=http://dx.doi.org/10.1038/modpathol.2013.242}}</ref> | ||
==Differential diagnosis== | |||
Cystic nephroma must be differentiated from [[Wilm's tumor]], [[renal cell carcinoma]], cystic [[mesoblastic nephroma]], and [[autosomal recessive polycystic kidney disease]]. | |||
==Epidemiology and Demographics== | |||
Cystic nephroma is a rare disease that tends to affect paediatric population and individuals in the 5th - 6th decades.<ref name=radio> Cystic nephroma.Dr Matt A. Morgan and Dr Frank Gaillard et al. Radiopaedia.org 2015. http://radiopaedia.org/articles/multilocular-cystic-nephroma </ref> In paediatric population, males are more commonly affected with cystic nephroma than females. In later life, females are more commonly affected with cystic nephroma than males.<ref name=radio> Cystic nephroma.Dr Matt A. Morgan and Dr Frank Gaillard et al. Radiopaedia.org 2015. http://radiopaedia.org/articles/multilocular-cystic-nephroma </ref> | |||
==Risk Factors== | |||
There are no established risk factors for cystic nephroma. | |||
==Screening== | |||
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine [[screening]] for cystic nephroma.<ref>renal oncocytoma. U.S. Preventive Service Task Force (USPSTF) 2015. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=cystic+nephroma Accessed on October, 31 2015 </ref> | |||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]
Overview
Cystic nephroma is a type of rare benign kidney tumor.[1]
Classification
There is no classification system established for cystic nephroma.
Pathophysiology
On gross pathology, large, unilateral, multiloculated, and well-encapsulated cystic tumor is the characteristic finding of cystic nephroma. On microscopic histopathological analysis, cysts lined by a simple epithelium with a hobnail morphology and ovarian-like stroma are characteristic findings of cystic nephroma.
Causes
Cystic nephroma is caused by mutations in the DICER1 gene.[2]
Differential diagnosis
Cystic nephroma must be differentiated from Wilm's tumor, renal cell carcinoma, cystic mesoblastic nephroma, and autosomal recessive polycystic kidney disease.
Epidemiology and Demographics
Cystic nephroma is a rare disease that tends to affect paediatric population and individuals in the 5th - 6th decades.[3] In paediatric population, males are more commonly affected with cystic nephroma than females. In later life, females are more commonly affected with cystic nephroma than males.[3]
Risk Factors
There are no established risk factors for cystic nephroma.
Screening
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for cystic nephroma.[4]
References
- ↑ Turbiner J, Amin MB, Humphrey PA, et al. (April 2007). "Cystic nephroma and mixed epithelial and stromal tumor of kidney: a detailed clinicopathologic analysis of 34 cases and proposal for renal epithelial and stromal tumor (REST) as a unifying term". Am. J. Surg. Pathol. 31 (4): 489–500. doi:10.1097/PAS.0b013e31802bdd56. PMID 17414095.
- ↑ Doros LA, Rossi CT, Yang J, Field A, Williams GM, Messinger Y, Cajaiba MM, Perlman EJ, A Schultz K, Cathro HP, Legallo RD, LaFortune KA, Chikwava KR, Faria P, Geller JI, Dome JS, Mullen EA, Gratias EJ, Dehner LP, Hill DA (2014). "DICER1 mutations in childhood cystic nephroma and its relationship to DICER1-renal sarcoma". Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc. 27 (9): 1267–80. doi:10.1038/modpathol.2013.242. PMC 4117822. PMID 24481001.
- ↑ 3.0 3.1 Cystic nephroma.Dr Matt A. Morgan and Dr Frank Gaillard et al. Radiopaedia.org 2015. http://radiopaedia.org/articles/multilocular-cystic-nephroma
- ↑ renal oncocytoma. U.S. Preventive Service Task Force (USPSTF) 2015. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=cystic+nephroma Accessed on October, 31 2015