Difference between revisions of "Cystic nephroma, adult type"
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*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 | |
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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
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
- Negative for DICER1 mutations.
- DICER1 mutations seen in cystic nephroma, pediatric type.
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
- ↑ 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.
- ↑ 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.