Cystic nephroma overview

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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]

Complications and Prognosis

Common complications of cystic nephroma include infection, urinary tract obstruction, hemorrhage, and rupture.[5] Prognosis is generally excellent.

Staging

There is no established system for the staging of cystic nephroma.

History and Symptoms

Cystic nephromas are often asymptomatic. Possible symptoms of cystic nephroma include hematuria, urinary frequency, and dysuria.[3]

Physical Examination

Physical examination of patients with cystic nephroma is usually remarkable for a palpable abdominal mass.


References

  1. 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.
  2. 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. 3.0 3.1 3.2 Cystic nephroma.Dr Matt A. Morgan and Dr Frank Gaillard et al. Radiopaedia.org 2015. http://radiopaedia.org/articles/multilocular-cystic-nephroma
  4. renal oncocytoma. U.S. Preventive Service Task Force (USPSTF) 2015. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=cystic+nephroma Accessed on October, 31 2015
  5. Eknoyan G (2009). "A clinical view of simple and complex renal cysts". Journal of the American Society of Nephrology : JASN. 20 (9): 1874–6. doi:10.1681/ASN.2008040441. PMID 18776117.

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