Difference between revisions of "Tubulocystic carcinoma of the kidney"

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{{ Infobox diagnosis
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Name      = {{PAGENAME}}
| Image      =  
| Image      = Tubulocystic renal cell carcinoma -- low mag.jpg
| Width      =
| Width      =
| Caption    =  
| Caption    = Tubulocystic renal cell carcinoma. [[H&E stain]].
| Synonyms  = low-grade collecting duct carcinoma
| 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
| 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  =
| Subtypes  =
| LMDDx      = [[renal oncocytoma]], [[cystic nephroma]]/[[mixed epithelial and stromal tumour]], [[multilocular cystic renal cell carcinoma]]
| LMDDx      = [[papillary renal cell carcinoma]] - especially oncocytic variant, [[renal oncocytoma]], [[cystic nephroma]]/[[mixed epithelial and stromal tumour]], [[multilocular cystic renal cell carcinoma]]
| Stains    =
| Stains    =
| IHC        =
| IHC        = CD10 +ve, RCC +ve, vimentin +ve, AMACR +ve
| EM        =
| EM        =
| Molecular  =
| Molecular  =
| IF        =
| IF        =
| Gross      =
| Gross      = multicystic lesion (bubble wrap-like appearance)
| Grossing  =
| Grossing  = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]]
| Staging    = [[kidney cancer staging]]
| Site      = [[kidney]]
| Site      = [[kidney]]
| Assdx      =
| Assdx      =
| Syndromes  =
| Syndromes  =
| Clinicalhx =
| Clinicalhx = typically male
| Signs      =
| Signs      =
| Symptoms  =
| Symptoms  =
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| Rads      =
| Rads      =
| Endoscopy  =
| Endoscopy  =
| Prognosis  =
| Prognosis  = good
| Other      =
| Other      =
| ClinDDx    =
| ClinDDx    =
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}}
}}
'''Tubulocystic carcinoma of the kidney''', abbreviated '''TCCK''', is a very rare [[kidney tumour]].
'''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]].  
*[[Cystic nephroma]]/[[Mixed epithelial and stromal tumour]].
*[[Cystic nephroma]]/[[Mixed epithelial and stromal tumour]].
*[[Multilocular cystic renal cell carcinoma]].
*[[Multilocular cystic renal neoplasm of low malignant potential]] (multilocular cystic renal cell carcinoma).


===Images===
===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/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/modpathol200970f15.html#figure-title TCCK - intermed. mag. (nature.com)].
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==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==
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