Difference between revisions of "Cystic nephroma, adult type"

From Libre Pathology
Jump to navigation Jump to search
Line 97: Line 97:
*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==

Revision as of 17:36, 24 April 2017

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

Gross

  • Bubble wrap-like appearance.

Image

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

Case 2

IHC

Features:[4]

  • 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. 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. 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. 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.
  4. 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.