Tubulocystic carcinoma of the kidney

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Tubulocystic carcinoma of the kidney
Diagnosis in short
Synonyms low-grade collecting duct carcinoma, tubulocystic renal cell carcinoma
LM cysts of variable size lined by a single layer of epithelium that has a cuboidal, flat, or hobnail morphology, eosinophilic cytoplasm, large nuclei with prominent nucleoli, +/-fibrotic stroma
LM DDx renal oncocytoma, cystic nephroma/mixed epithelial and stromal tumour, multilocular cystic renal cell carcinoma
IHC CD10 +ve, RCC +ve, vimentin +ve, AMACR +ve
Gross multicystic lesion (bubble wrap-like appearance)
Site kidney
Clinical history typically male
Prevalence very rare
Prognosis good

Tubulocystic carcinoma of the kidney, abbreviated TCCK, is a very rare kidney tumour.

It is also known as low-grade collecting duct carcinoma.[1]

In the Vancouver classification it is refered to as tubulocystic renal cell carcinoma.[2]


It should not be confused with collecting duct carcinoma, a very aggressive renal tumour.

General

  • Not in the WHO classification of renal tumours - as of 2013.[3]
  • Rare <1% - in a series of 615 cases.
  • Males:Females ~ 7:1.[1]
  • Prognosis - probably favourable.[1]

Gross

  • Bubble wrap-like appearance - due to the cystic nature.[1]

Microscopic

Features:.[1]

  • Cysts of variable size lined by a single layer of epithelium that has a:
    • Cuboidal, flat, or hobnail morphology.
    • Eosinophilic cytoplasm.
    • Large nuclei with prominent nucleoli.
  • +/-Fibrotic stroma.

DDx:

Images

IHC

Features:[3]

  • CD10 +ve.
  • RCC +ve.
  • Vimentin +ve.
  • AMACR +ve.
  • CK7 -ve/+ve (focally in 2/3 tumours[5]).

See also

References

  1. ↑ 1.0 1.1 1.2 1.3 1.4 Amin, MB.; MacLennan, GT.; Gupta, R.; Grignon, D.; Paraf, F.; Vieillefond, A.; Paner, GP.; Stovsky, M. et al. (Mar 2009). "Tubulocystic carcinoma of the kidney: clinicopathologic analysis of 31 cases of a distinctive rare subtype of renal cell carcinoma.". Am J Surg Pathol 33 (3): 384-92. doi:10.1097/PAS.0b013e3181872d3f. PMID 19011562.
  2. ↑ Srigley, JR.; Delahunt, B.; Eble, JN.; Egevad, L.; Epstein, JI.; Grignon, D.; Hes, O.; Moch, H. et al. (Oct 2013). "The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia.". Am J Surg Pathol 37 (10): 1469-89. doi:10.1097/PAS.0b013e318299f2d1. PMID 24025519.
  3. ↑ 3.0 3.1 Alexiev, BA.; Drachenberg, CB. (May 2013). "Tubulocystic carcinoma of the kidney: a histologic, immunohistochemical, and ultrastructural study.". Virchows Arch 462 (5): 575-81. doi:10.1007/s00428-013-1398-0. PMID 23525677.
  4. ↑ Srigley, JR.; Delahunt, B. (Jun 2009). "Uncommon and recently described renal carcinomas.". Mod Pathol 22 Suppl 2: S2-S23. doi:10.1038/modpathol.2009.70. PMID 19494850.
  5. ↑ Zhang, SH.; Shi, SF.; Huang, SF.; Zhang, YN.; Zheng, YY.; Chen, GY. (Aug 2012). "[Clinical and pathologic features of tubulocystic carcinoma of kidney].". Zhonghua Bing Li Xue Za Zhi 41 (8): 543-6. PMID 23157747.
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