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Difference between revisions of "Cystic nephroma, adult type"
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| Caption = Micrograph of cystic nephroma (adult type) showing the characteristic stroma and hobnailed epithelium. [[H&E stain]]. | | Caption = Micrograph of cystic nephroma (adult type) showing the characteristic stroma and hobnailed epithelium. [[H&E stain]]. | ||
| Synonyms = | | Synonyms = mixed epithelial and stromal tumour family (term recommended in WHO 2016 classification) | ||
| Micro = cysts lined by simple epithelium with hobnailing, stroma has an ovarian look (blue, spindle cells) | | Micro = cysts lined by simple epithelium with hobnailing, stroma has an ovarian look (blue, spindle cells) | ||
| Subtypes = | | Subtypes = on a spectrum with ''[[mixed epithelial stromal tumour]]'' | ||
| LMDDx = [[localized cystic disease of the kidney]] | |||
| Stains = | | Stains = | ||
| IHC = ER +ve, PR +ve, CD10 +ve | | IHC = ER +ve, PR +ve, CD10 +ve | ||
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| Endoscopy = | | Endoscopy = | ||
| Prognosis = benign | | Prognosis = benign | ||
| Other = | | Other = | ||
| ClinDDx = other cystic kidney tumours | | ClinDDx = other cystic kidney tumours | ||
| Tx = | | Tx = | ||
| Line 32: | Line 32: | ||
'''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, | 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> | In the 2016 World Health Organization classification of renal neoplasia, this tumour was lumped with ''[[mixed epithelial and stromal tumour]]'' and the recommended term '''''mixed epithelial and stromal tumour family'''''.<ref name=pmid26935559>{{Cite journal | last1 = Moch | first1 = H. | last2 = Cubilla | first2 = AL. | last3 = Humphrey | first3 = PA. | last4 = Reuter | first4 = VE. | last5 = Ulbright | first5 = TM. | title = The 2016 WHO Classification of Tumours of the Urinary System and Male Genital Organs-Part A: Renal, Penile, and Testicular Tumours. | journal = Eur Urol | volume = 70 | issue = 1 | pages = 93-105 | month = 07 | year = 2016 | doi = 10.1016/j.eururo.2016.02.029 | PMID = 26935559 }}</ref> | ||
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> came-up with a cheeky term for the the tumour family (''[[renal epithelial and stromal tumour]]'', abbreviated ''REST'') that didn't catch on. | |||
==General== | ==General== | ||
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Notes: | Notes: | ||
*Parenchymal elements (e.g. glomueruli, tubules) are not found in the septa between the cysts. | *Parenchymal elements (e.g. glomueruli, tubules) are not found in the septa between the cysts. | ||
*The WHO 2016 classification considers it to be on a spectrum with ''[[mixed epithelial stromal tumour]]''.<ref name=pmid26935559/> | |||
DDx: | DDx: | ||
*[[Localized cystic disease of the kidney]]. | *[[Localized cystic disease of the kidney]]. | ||
| Line 85: | Line 87: | ||
==IHC== | ==IHC== | ||
Features: | Features:<ref name=pmid16390243>{{Cite journal | last1 = Antic | first1 = T. | last2 = Perry | first2 = KT. | last3 = Harrison | first3 = K. | last4 = Zaytsev | first4 = P. | last5 = Pins | first5 = M. | last6 = Campbell | first6 = SC. | last7 = Picken | first7 = MM. | title = Mixed epithelial and stromal tumor of the kidney and cystic nephroma share overlapping features: reappraisal of 15 lesions. | journal = Arch Pathol Lab Med | volume = 130 | issue = 1 | pages = 80-5 | month = Jan | year = 2006 | doi = 10.1043/1543-2165(2006)130[80:MEASTO]2.0.CO;2 | PMID = 16390243 }}</ref> | ||
*ER +ve. | *Stromal cells: | ||
*PR +ve. | **ER +ve. | ||
*CD10 +ve. | **PR +ve. | ||
*Cyst lining cells: | |||
**CK7 +ve. | |||
**CD10 +ve. | |||
**HMWCK +ve. | |||
==Molecular== | ==Molecular== | ||
*Negative for DICER1 mutations. | *Negative for DICER1 mutations. | ||
**DICER1 mutations seen in [[cystic nephroma, pediatric type]]. | **DICER1 mutations seen in [[cystic nephroma, pediatric type]]. | ||
==Sign out== | |||
<pre> | |||
Cystic Lesion, Right Kidney, Nephrectomy: | |||
- Cystic nephroma/mixed epithelial stromal tumour, see comment. | |||
Comment: | |||
The stromal cells are POSITIVE for ER, PR, CD10, and alpha-SMA. The cyst lining cells are POSITIVE for CK7 and CK34betaE12. | |||
</pre> | |||
===Micro=== | |||
The tumour is predominantly cystic. The cysts are lined by hobnail cells. Ovarian-like stroma is present. | |||
==See also== | ==See also== | ||