Cystic nephroma
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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]
Synonyms and keywords: Multilocular cystic nephroma, Mixed epithelial stromal tumor, Renal epithelial stromal tumor
Overview
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] 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. Cystic nephroma is caused by mutations in the DICER1 gene.[2] Cystic nephroma must be differentiated from Wilm’s tumor, renal cell carcinoma, cystic mesoblastic nephroma, and autosomal recessive polycystic kidney disease. 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] Cystic nephromas are often asymptomatic. Possible symptoms of cystic nephroma include hematuria, urinary frequency, and dysuria.[3] Biopsy is helpful in the diagnosis of cystic nephroma. Surgery is the mainstay of treatment for cystic nephroma. Common complications of cystic nephroma include infection, urinary tract obstruction, hemorrhage, and rupture.[4] Prognosis is generally excellent.
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.[5]
Complications and Prognosis
Common complications of cystic nephroma include infection, urinary tract obstruction, hemorrhage, and rupture.[4] Prognosis is generally excellent.
Diagnosis
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.
Laboratory Findings
There are no laboratory findings associated with cystic nephroma.
X Ray
Abdominal X-ray may be helpful in the diagnosis of cystic nephroma. Findings on X-ray suggestive of cystic nephroma is a large abdominal mass displacing and effacing adjacent bowel loops.[3]
CT
CT scan may be helpful in the diagnosis of cystic nephroma. Findings on CT scan suggestive of cystic nephroma include large multilocular encapsulated cystic mass, herniation into the renal pelvis, variable septal enhancement, and associated streakiness in perirenal fat.[3]
MRI
MRI may be helpful in the diagnosis of cystic nephroma.
Ultrasound
Ultrasound may be helpful in the diagnosis of cystic nephroma. Findings on ultrasound suggestive of cystic nephroma is a renal mass with multiple circumscribed sonolucent areas.[3]
Other Imaging Findings
There are no other imaging findings associated with cystic nephroma.
Other Diagnostic Studies
There are no other diagnostic study findings associated with cystic nephroma.
Biopsy
Biopsy is helpful in the diagnosis of cystic nephroma.
Treatment
Medical Therapy
The mainstay of therapy for cystic nephroma is surgery.
Surgery
Surgery is the mainstay of treatment for cystic nephroma.
Primary Prevention
There is no established method for prevention of cystic nephroma.
Secondary Prevention
There are no secondary preventive measures available for cystic nephroma.
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.
- ↑ 2.0 2.1 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 3.2 3.3 3.4 3.5 3.6 3.7 3.8 Cystic nephroma.Dr Matt A. Morgan and Dr Frank Gaillard et al. Radiopaedia.org 2015. http://radiopaedia.org/articles/multilocular-cystic-nephroma
- ↑ 4.0 4.1 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.
- ↑ renal oncocytoma. U.S. Preventive Service Task Force (USPSTF) 2015. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=cystic+nephroma Accessed on October, 31 2015
Historical Perspective
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References
Classification
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]
Overview
There is no classification system established for cystic nephroma.
Classification
There is no classification system established for cystic nephroma.
References
Pathophysiology
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]
Overview
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.
Gross Pathology
Characteristic findings of cystic nephroma on gross pathology include:
Microscopic Pathology
The characteristics of cystic nephromas include:
- Cysts lined by a simple epithelium with a hobnail morphology（i.e. the nuclei of the cyst lining epithelium bulges into the lumen of the cysts）
- Ovarian-like stroma
- Spindle cell morphology
- Basophilic cytoplasm
-
![Cystic nephroma[1]](https://www.wikidoc.org/images/b/b6/Cystic_nephroma_high_mag.jpg)
Cystic nephroma[1]
![Cystic nephroma[1]](https://www.wikidoc.org/index.php/File%3ACystic_nephroma_high_mag.jpg)
Genetics
- DICER1 is a human gene.[3] This gene encodes a protein possessing an RNA helicase motif containing a DEXH box in its amino terminus and an RNA motif in the carboxy terminus. The encoded protein functions as a ribonuclease and is required by the RNA interference and small temporal RNA (stRNA) pathways to produce the active small RNA component that represses gene expression. Two transcript variants encoding the same protein have been identified for this gene.[3]
References
- ↑ Cystic nephroma. Wikipedia 2015. https://en.wikipedia.org/wiki/Cystic_nephroma#/media/File:Cystic_nephroma_high_mag.jpg Accessed on October, 30 2015
- ↑ 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 “Entrez Gene: DICER1 Dicer1, Dcr-1 homolog (Drosophila)”.
Causes
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 caused by mutations in the DICER1 gene.[1]
Causes
Cystic nephroma is caused by mutations in the DICER1 gene.[1]
References
- ↑ 1.0 1.1 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.
Differentiating Cystic nephroma
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]
Overview
Cystic nephroma must be differentiated from Wilm’s tumor, renal cell carcinoma, cystic mesoblastic nephroma, and autosomal recessive polycystic kidney disease.
Differential diagnosis
Cystic nephroma must be differentiated from:[1]
- Wilm’s tumor
- Renal cell carcinoma
- Cystic mesoblastic nephroma
- Autosomal recessive polycystic kidney disease (ARPKD)
- Multicystic dysplastic kidney (MCDK)
- Mulitlocular cystic nephroma (MLCN)
- Nephronophthisis
References
- ↑ Cystic nephroma.Dr Matt A. Morgan and Dr Frank Gaillard et al. Radiopaedia.org 2015. http://radiopaedia.org/articles/multilocular-cystic-nephroma
Epidemiology and Demographics
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 rare disease that tends to affect the pediatric population and individuals in the 5th – 6th decades.[1] In the pediatric population, males are more commonly affected with cystic nephroma than females. In adulthood, females are more commonly affected with cystic nephroma than males.[1]
Age
- Cystic nephroma is a rare disease that tends to affect the pediatric population and individuals in the 5th – 6th decades.[1]
Gender
- In the pediatric population, males are more commonly affected with cystic nephroma than females.
- In later life, females are more commonly affected with cystic nephroma than males.[1]
References
- ↑ 1.0 1.1 1.2 1.3 Cystic nephroma.Dr Matt A. Morgan and Dr Frank Gaillard et al. Radiopaedia.org 2015. http://radiopaedia.org/articles/multilocular-cystic-nephroma
Risk Factors
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]
Overview
There are no established risk factors for cystic nephroma.
Risk Factors
There are no established risk factors for cystic nephroma.
References
Screening
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]
Overview
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening 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.[1]
References
- ↑ cystic nephroma. U.S. Preventive Service Task Force (USPSTF) 2015. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=cystic+nephroma Accessed on October, 31 2015
Natural History, Complications and Prognosis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]
Overview
Common complications of cystic nephroma include infection, urinary tract obstruction, hemorrhage, and rupture.[1] Prognosis is generally excellent.
Complications
Common complications of cystic nephroma include:[1]
Prognosis
- Prognosis of cystic nephroma is generally excellent.
References
- ↑ 1.0 1.1 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.
Diagnosis
Diagnosis
Staging | History and Symptoms | Physical Examination | Laboratory Findings | X Ray | CT | MRI Ultrasound | Other Imaging Findings | Other Diagnostic Studies | Biopsy
Treatment
Treatment
Medical therapy | Surgery | Primary prevention | Secondary prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies
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