Cystic nephroma

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Cystic nephroma is a rare, benign kidney tumour.

Cystic nephroma
Diagnosis in short

Micrograph of cystic nephroma 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

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:

  • ER +ve.
  • PR +ve.
  • CD10 +ve.

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.