Difference between revisions of "Cystic nephroma, adult type"
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'''Cystic nephroma, adult type''' is a rare, benign [[kidney tumours|kidney tumour]]. | '''Cystic nephroma, adult type''' is a rare, benign [[kidney tumours|kidney tumour]]. | ||
Pathologically, '''cystic nephroma, adult type''' is separated from [[cystic nephroma, pediatric type]] by DICER1 mutations. | |||
Turbiner ''et al.''<ref name=pmid17414095>{{Cite journal | last1 = Turbiner | first1 = J. | last2 = Amin | first2 = MB. | last3 = Humphrey | first3 = PA. | last4 = Srigley | first4 = JR. | last5 = De Leval | first5 = L. | last6 = Radhakrishnan | first6 = A. | last7 = Oliva | first7 = E. | title = 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. | journal = Am J Surg Pathol | volume = 31 | issue = 4 | pages = 489-500 | month = Apr | year = 2007 | doi = 10.1097/PAS.0b013e31802bdd56 | PMID = 17414095 }}</ref> have suggested that ''cystic nephroma'' and ''[[mixed epithelial and stromal tumour]] (MEST)'' are one tumour; the lumping term is ''[[renal epithelial and stromal tumour]]'' (abbreviated ''REST''). | Turbiner ''et al.''<ref name=pmid17414095>{{Cite journal | last1 = Turbiner | first1 = J. | last2 = Amin | first2 = MB. | last3 = Humphrey | first3 = PA. | last4 = Srigley | first4 = JR. | last5 = De Leval | first5 = L. | last6 = Radhakrishnan | first6 = A. | last7 = Oliva | first7 = E. | title = 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. | journal = Am J Surg Pathol | volume = 31 | issue = 4 | pages = 489-500 | month = Apr | year = 2007 | doi = 10.1097/PAS.0b013e31802bdd56 | PMID = 17414095 }}</ref> have suggested that ''cystic nephroma'' and ''[[mixed epithelial and stromal tumour]] (MEST)'' are one tumour; the lumping term is ''[[renal epithelial and stromal tumour]]'' (abbreviated ''REST''). | ||
Revision as of 18:40, 13 March 2016
| Cystic nephroma, adult type | |
|---|---|
| Diagnosis in short | |
 Micrograph of cystic nephroma (adult type) showing the characteristic stroma and hobnailed epithelium. H&E stain. | |
|
| |
| LM | cysts lined by simple epithelium with hobnailing, stroma has an ovarian look (blue, spindle cells) |
| LM DDx | mixed epithelial stromal tumour, localized cystic disease of the kidney |
| IHC | ER +ve, PR +ve, CD10 +ve |
| Gross | bubble wrap-like appearance |
| Site | kidney - see kidney tumours |
|
| |
| Prevalence | rare |
| Prognosis | benign |
| Clin. DDx | other cystic kidney tumours |
Cystic nephroma, adult type is a rare, benign kidney tumour.
Pathologically, cystic nephroma, adult type is separated from cystic nephroma, pediatric type by DICER1 mutations.
Turbiner et al.[1] have suggested that cystic nephroma and mixed epithelial and stromal tumour (MEST) are one tumour; the lumping term is renal epithelial and stromal tumour (abbreviated REST).
General
- Rare.[2]
- Good prognosis/benign.[2][3]
- Considered to be on a spectrum with cystic partially differentiated nephroblastoma and Wilms tumour.[3]
Gross
- Bubble wrap-like appearance.
Image
Bubble wrap. (WC)
www:
Microscopic
Features:
- Cysts lined by simple epithelium with hobnailing - key feature.
- Stroma has an ovarian look:
- Blue (basophilic).
- Spindle cells.
Notes:
- Parenchymal elements (e.g. glomueruli, tubules) are not found in the septa between the cysts.
DDx:
Images
Case 1
Cystic nephroma - low mag. (WC/Nephron)
Cystic nephroma - intermed. mag. (WC/Nephron)
Case 2
CN - low mag. (WC/Nephron)
CN - intermed. mag. (WC/Nephron)
CN - high mag. (WC/Nephron)
CN - high mag. (WC/Nephron)
CN - very high mag. (WC/Nephron)
CN - low mag. (WC/Nephron)
CN - low mag. (WC/Nephron)
CN - intermed. mag. (WC/Nephron)
CN - high mag. (WC/Nephron)
IHC
Features:
- ER +ve.
- PR +ve.
- CD10 +ve.
Molecular
- Negative for DICER1 mutations.
- DICER1 mutations seen in cystic nephroma, pediatric type.
See also
References
- ↑ Turbiner, J.; Amin, MB.; Humphrey, PA.; Srigley, JR.; De Leval, L.; Radhakrishnan, A.; Oliva, E. (Apr 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 2.2 Mohanty, D.; Jain, BK.; Agrawal, V.; Gupta, A. (May 2010). "Cystic nephroma: a diagnostic dilemma.". Saudi J Kidney Dis Transpl 21 (3): 518-20. PMID 20427881.
- ↑ 3.0 3.1 van den Hoek, J.; de Krijger, R.; van de Ven, K.; Lequin, M.; van den Heuvel-Eibrink, MM. (2009). "Cystic nephroma, cystic partially differentiated nephroblastoma and cystic Wilms' tumor in children: a spectrum with therapeutic dilemmas.". Urol Int 82 (1): 65-70. doi:10.1159/000176028. PMID 19172100.