Difference between revisions of "Tubulocystic carcinoma of the kidney"

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'''Tubulocystic carcinoma''' is a very rare [[kidney tumour]].
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Tubulocystic renal cell carcinoma -- low mag.jpg
| Width      =
| Caption    = Tubulocystic renal cell carcinoma. [[H&E stain]].
| Synonyms  = low-grade collecting duct carcinoma, tubulocystic renal cell carcinoma
| Micro      = 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
| Subtypes  =
| LMDDx      = [[papillary renal cell carcinoma]] - especially oncocytic variant, [[renal oncocytoma]], [[cystic nephroma]]/[[mixed epithelial and stromal tumour]], [[multilocular cystic renal cell carcinoma]]
| Stains    =
| IHC        = CD10 +ve, RCC +ve, vimentin +ve, AMACR +ve
| EM        =
| Molecular  =
| IF        =
| Gross      = multicystic lesion (bubble wrap-like appearance)
| Grossing  = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]]
| Staging    = [[kidney cancer staging]]
| Site      = [[kidney]]
| Assdx      =
| Syndromes  =
| Clinicalhx = typically male
| Signs      =
| Symptoms  =
| Prevalence = very rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = good
| Other      =
| ClinDDx    =
| Tx        =
}}
'''Tubulocystic carcinoma of the kidney''', abbreviated '''TCCK''', is a very rare [[kidney tumour]].
 
In the [[Vancouver classification|Vancouver modification]] of the WHO classification of renal neoplasia, it is refered to as '''tubulocystic renal cell carcinoma''' (abbreviated '''TC-RCC''').<ref name=pmid24025519>{{Cite journal  | last1 = Srigley | first1 = JR. | last2 = Delahunt | first2 = B. | last3 = Eble | first3 = JN. | last4 = Egevad | first4 = L. | last5 = Epstein | first5 = JI. | last6 = Grignon | first6 = D. | last7 = Hes | first7 = O. | last8 = Moch | first8 = H. | last9 = Montironi | first9 = R. | title = The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia. | journal = Am J Surg Pathol | volume = 37 | issue = 10 | pages = 1469-89 | month = Oct | year = 2013 | doi = 10.1097/PAS.0b013e318299f2d1 | PMID = 24025519 }}</ref>


It is also known as '''low-grade collecting duct carcinoma'''.<ref name=pmid19011562 >{{Cite journal  | last1 = Amin | first1 = MB. | last2 = MacLennan | first2 = GT. | last3 = Gupta | first3 = R. | last4 = Grignon | first4 = D. | last5 = Paraf | first5 = F. | last6 = Vieillefond | first6 = A. | last7 = Paner | first7 = GP. | last8 = Stovsky | first8 = M. | last9 = Young | first9 = AN. | title = Tubulocystic carcinoma of the kidney: clinicopathologic analysis of 31 cases of a distinctive rare subtype of renal cell carcinoma. | journal = Am J Surg Pathol | volume = 33 | issue = 3 | pages = 384-92 | month = Mar | year = 2009 | doi = 10.1097/PAS.0b013e3181872d3f | PMID = 19011562 }}</ref>
It is also known as '''low-grade collecting duct carcinoma'''.<ref name=pmid19011562 >{{Cite journal  | last1 = Amin | first1 = MB. | last2 = MacLennan | first2 = GT. | last3 = Gupta | first3 = R. | last4 = Grignon | first4 = D. | last5 = Paraf | first5 = F. | last6 = Vieillefond | first6 = A. | last7 = Paner | first7 = GP. | last8 = Stovsky | first8 = M. | last9 = Young | first9 = AN. | title = Tubulocystic carcinoma of the kidney: clinicopathologic analysis of 31 cases of a distinctive rare subtype of renal cell carcinoma. | journal = Am J Surg Pathol | volume = 33 | issue = 3 | pages = 384-92 | month = Mar | year = 2009 | doi = 10.1097/PAS.0b013e3181872d3f | PMID = 19011562 }}</ref>
It should '''not''' be confused with ''[[collecting duct carcinoma]]'', a very aggressive [[renal tumours|renal tumour]].


==General==
==General==
*Not in the WHO classification of renal tumours - as of 2013.<ref name=pmid23525677>{{Cite journal  | last1 = Alexiev | first1 = BA. | last2 = Drachenberg | first2 = CB. | title = Tubulocystic carcinoma of the kidney: a histologic, immunohistochemical, and ultrastructural study. | journal = Virchows Arch | volume = 462 | issue = 5 | pages = 575-81 | month = May | year = 2013 | doi = 10.1007/s00428-013-1398-0 | PMID = 23525677 }}</ref>
 
*Rare <1% - in a series of 615 cases.
*Rare <1% - in a series of 615 cases.
*Males:Females ~ 7:1.<ref name=pmid19011562/>
*Males:Females ~ 7:1.<ref name=pmid19011562/>
*Prognosis - probably favourable.<ref name=pmid19011562/>
*Prognosis - probably favourable.<ref name=pmid19011562/>
*Thought to be related to [[papillary renal cell carcinoma]].<ref name=pmid19898225>{{Cite journal  | last1 = Zhou | first1 = M. | last2 = Yang | first2 = XJ. | last3 = Lopez | first3 = JI. | last4 = Shah | first4 = RB. | last5 = Hes | first5 = O. | last6 = Shen | first6 = SS. | last7 = Li | first7 = R. | last8 = Yang | first8 = Y. | last9 = Lin | first9 = F. | title = Renal tubulocystic carcinoma is closely related to papillary renal cell carcinoma: implications for pathologic classification. | journal = Am J Surg Pathol | volume = 33 | issue = 12 | pages = 1840-9 | month = Dec | year = 2009 | doi = 10.1097/PAS.0b013e3181be22d1 | PMID = 19898225 }}</ref>


==Gross==
==Gross==
*Bubble wrap-like appearance - due to the cystic nature.<ref name=pmid19011562/>
*Bubble wrap-like appearance - due to the cystic nature.<ref name=pmid19011562/><ref name=pmid17786473>{{Cite journal  | last1 = Azoulay | first1 = S. | last2 = Vieillefond | first2 = A. | last3 = Paraf | first3 = F. | last4 = Pasquier | first4 = D. | last5 = Cussenot | first5 = O. | last6 = Callard | first6 = P. | last7 = Sibony | first7 = M. | title = Tubulocystic carcinoma of the kidney: a new entity among renal tumors. | journal = Virchows Arch | volume = 451 | issue = 5 | pages = 905-9 | month = Nov | year = 2007 | doi = 10.1007/s00428-007-0483-7 | PMID = 17786473 }}</ref>


==Microscopic==
==Microscopic==
Features:.<ref name=pmid19011562/>
Features:.<ref name=pmid19011562/>
*Cysts of variable size lined by a single layer of epithelium that has a:
*Cysts of variable size lined by a single layer of epithelium that has a:
**Cuboidal, flat, or hobnail morphology.
**Cuboidal, flat, or [[hobnail morphology]].
**Eosinophilic cytoplasm.
**Eosinophilic cytoplasm.
**Large nuclei with prominent nucleoli.  
**Large nuclei with prominent nucleoli.  
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DDx:
DDx:
*[[Papillary renal cell carcinoma]] (oncocytic variant).
*[[Hereditary leiomyomatosis renal cell carcinoma syndrome associated renal cell carcinoma]].
*[[Renal oncocytoma]].  
*[[Renal oncocytoma]].  
*[[Multilocular cystic renal cell carcinoma]].
*[[Cystic nephroma]]/[[Mixed epithelial and stromal tumour]].
*[[Cystic nephroma]]/[[Mixed epithelial and stromal tumour]].
*[[Multilocular cystic renal neoplasm of low malignant potential]] (multilocular cystic renal cell carcinoma).
===Images===
====Case 1====
<gallery>
Image: Tubulocystic renal cell carcinoma -- very low mag.jpg | TC-RCC - very low mag.
Image: Tubulocystic renal cell carcinoma -- low mag.jpg | TC-RCC - low mag.
Image: Tubulocystic renal cell carcinoma -- intermed mag.jpg | TC-RCC - intermed. mag.
Image: Tubulocystic renal cell carcinoma -- high mag.jpg | TC-RCC - high mag.
Image: Tubulocystic renal cell carcinoma -- very high mag.jpg | TC-RCC - very high mag.
Image: Tubulocystic renal cell carcinoma - alt -- low mag.jpg | TC-RCC - low mag.
Image: Tubulocystic renal cell carcinoma - alt -- intermed mag.jpg | TC-RCC - intermed. mag.
Image: Tubulocystic renal cell carcinoma - alt -- high mag.jpg | TC-RCC - high mag.
Image: Tubulocystic renal cell carcinoma - alt -- very high mag.jpg | TC-RCC - very high mag.
</gallery>
====Case 2====
<gallery>
Image: Tubulocystic carcinoma of the kidney -- low mag.jpg | TC - low mag.
Image: Tubulocystic carcinoma of the kidney -- intermed mag.jpg | TC - intermed. mag.
Image: Tubulocystic carcinoma of the kidney -- high mag.jpg | TC - high mag.
Image: Tubulocystic carcinoma of the kidney -- very high mag.jpg | TC - very high mag.
</gallery>
====www====
*[http://www.nature.com/modpathol/journal/v22/n2s/fig_tab/modpathol200970f14.html#figure-title TCCK - low mag. (nature.com)].<ref name=pmid19494850>{{Cite journal  | last1 = Srigley | first1 = JR. | last2 = Delahunt | first2 = B. | title = Uncommon and recently described renal carcinomas. | journal = Mod Pathol | volume = 22 Suppl 2 | issue =  | pages = S2-S23 | month = Jun | year = 2009 | doi = 10.1038/modpathol.2009.70 | PMID = 19494850 }}</ref>
*[http://www.nature.com/modpathol/journal/v22/n2s/fig_tab/modpathol200970f15.html#figure-title TCCK - intermed. mag. (nature.com)].
*[http://www.nature.com/modpathol/journal/v22/n2s/fig_tab/modpathol200970f16.html#figure-title TCCK - high mag. (nature.com)].


==IHC==
==IHC==
Features:<ref name=pmid23525677/>
Features:<ref name=pmid23525677>{{Cite journal  | last1 = Alexiev | first1 = BA. | last2 = Drachenberg | first2 = CB. | title = Tubulocystic carcinoma of the kidney: a histologic, immunohistochemical, and ultrastructural study. | journal = Virchows Arch | volume = 462 | issue = 5 | pages = 575-81 | month = May | year = 2013 | doi = 10.1007/s00428-013-1398-0 | PMID = 23525677 }}</ref>
*CD10 +ve.
*CD10 +ve.
*RCC +ve.
*RCC +ve.
*Vimentin +ve.
*Vimentin +ve.
*AMACR +ve.
*[[AMACR]] +ve.
*[[CK7]] -ve/+ve (focally in 2/3 tumours<ref name=pmid23157747>{{Cite journal  | last1 = Zhang | first1 = SH. | last2 = Shi | first2 = SF. | last3 = Huang | first3 = SF. | last4 = Zhang | first4 = YN. | last5 = Zheng | first5 = YY. | last6 = Chen | first6 = GY. | title = [Clinical and pathologic features of tubulocystic carcinoma of kidney]. | journal = Zhonghua Bing Li Xue Za Zhi | volume = 41 | issue = 8 | pages = 543-6 | month = Aug | year = 2012 | doi =  | PMID = 23157747 }}</ref>).
 
Notes:
*IHC profile essentially that of [[PaRCC]].{{fact}}


==See also==
==See also==
*[[Clear cell papillary renal cell carcinoma]].
*[[Clear cell papillary renal cell carcinoma]].
*[[Collecting duct carcinoma]].
*[[Kidney tumours]].
*[[Kidney tumours]].


Latest revision as of 18:14, 28 February 2017

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
Staging kidney cancer staging
Site kidney
Clinical history typically male
Prevalence very rare
Prognosis good

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

In the Vancouver modification of the WHO classification of renal neoplasia, it is refered to as tubulocystic renal cell carcinoma (abbreviated TC-RCC).[1]

It is also known as low-grade collecting duct carcinoma.[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.[2][4]

Microscopic

Features:.[2]

  • 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

Case 1

  • TC-RCC - very low mag.

  • TC-RCC - low mag.

  • TC-RCC - intermed. mag.

  • TC-RCC - high mag.

  • TC-RCC - very high mag.

  • TC-RCC - low mag.

  • TC-RCC - intermed. mag.

  • TC-RCC - high mag.

  • TC-RCC - very high mag.

Case 2

  • TC - low mag.

  • TC - intermed. mag.

  • TC - high mag.

  • TC - very high mag.

www

IHC

Features:[6]

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

Notes:

See also

References

  1. ↑ 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.
  2. ↑ 2.0 2.1 2.2 2.3 2.4 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.
  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.
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