Difference between revisions of "Cystic nephroma, adult type"

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| Width      =
| Width      =
| 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      = [[mixed epithelial stromal tumour]], [[localized cystic disease of the kidney]]
| 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        =
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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.
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'').
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:
*[[Mixed epithelial stromal tumour]].
*[[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==

Latest revision as of 03:57, 6 March 2019

Cystic nephroma, adult type
Diagnosis in short

Micrograph of cystic nephroma (adult type) showing the characteristic stroma and hobnailed epithelium. H&E stain.
Synonyms mixed epithelial and stromal tumour family (term recommended in WHO 2016 classification)
LM cysts lined by simple epithelium with hobnailing, stroma has an ovarian look (blue, spindle cells)
Subtypes on a spectrum with mixed epithelial stromal tumour
LM DDx 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.

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.[1]

Turbiner et al.[2] came-up with a cheeky term for the the tumour family (renal epithelial and stromal tumour, abbreviated REST) that didn't catch on.

General

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.
  • The WHO 2016 classification considers it to be on a spectrum with mixed epithelial stromal tumour.[1]

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:[5]

  • Stromal cells:
    • ER +ve.
    • PR +ve.
  • Cyst lining cells:
    • CK7 +ve.
    • CD10 +ve.
    • HMWCK +ve.

Molecular

Sign out

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.

Micro

The tumour is predominantly cystic. The cysts are lined by hobnail cells. Ovarian-like stroma is present.

See also

References

  1. ↑ 1.0 1.1 Moch, H.; Cubilla, AL.; Humphrey, PA.; Reuter, VE.; Ulbright, TM. (07 2016). "The 2016 WHO Classification of Tumours of the Urinary System and Male Genital Organs-Part A: Renal, Penile, and Testicular Tumours.". Eur Urol 70 (1): 93-105. doi:10.1016/j.eururo.2016.02.029. PMID 26935559.
  2. ↑ 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.
  3. ↑ 3.0 3.1 3.2 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.
  4. ↑ 4.0 4.1 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.
  5. ↑ Antic, T.; Perry, KT.; Harrison, K.; Zaytsev, P.; Pins, M.; Campbell, SC.; Picken, MM. (Jan 2006). "Mixed epithelial and stromal tumor of the kidney and cystic nephroma share overlapping features: reappraisal of 15 lesions.". Arch Pathol Lab Med 130 (1): 80-5. doi:10.1043/1543-2165(2006)130[80:MEASTO]2.0.CO;2. PMID 16390243.
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