Tubulocystic carcinoma of the kidney

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Tubulocystic carcinoma of the kidney
Diagnosis in short

Tubulocystic renal cell carcinoma. H&E stain.

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 papillary renal cell carcinoma - especially oncocytic variant, 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)
Grossing notes total nephrectomy for tumour grossing, partial nephrectomy grossing
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 modification of the WHO classification of renal neoplasia, it is refered to as tubulocystic renal cell carcinoma (abbreviated TC-RCC).[2]

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

General

Gross

  • Bubble wrap-like appearance - due to the cystic nature.[1][4]

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:[6]

  • CD10 +ve.
  • RCC +ve.
  • Vimentin +ve.
  • AMACR +ve.
  • CK7 -ve/+ve (focally in 2/3 tumours[7]).

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. Zhou, M.; Yang, XJ.; Lopez, JI.; Shah, RB.; Hes, O.; Shen, SS.; Li, R.; Yang, Y. et al. (Dec 2009). "Renal tubulocystic carcinoma is closely related to papillary renal cell carcinoma: implications for pathologic classification.". Am J Surg Pathol 33 (12): 1840-9. doi:10.1097/PAS.0b013e3181be22d1. PMID 19898225.
  4. Azoulay, S.; Vieillefond, A.; Paraf, F.; Pasquier, D.; Cussenot, O.; Callard, P.; Sibony, M. (Nov 2007). "Tubulocystic carcinoma of the kidney: a new entity among renal tumors.". Virchows Arch 451 (5): 905-9. doi:10.1007/s00428-007-0483-7. PMID 17786473.
  5. 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.
  6. 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.
  7. 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.