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'''[[Kidney]] tumours''' - includes '''malignant kidney tumours''' and '''benign kidney tumours'''.  Medical renal diseases are dealt with in the [[medical renal diseases]] article.
[[Image:Renal oncocytoma.jpg|thumb|250px|A kidney tumour ([[renal oncocytoma]]). (WC/Emmanuelm)]]
'''[[Kidney]] tumours''' - includes '''malignant kidney tumours''' ('''kidney cancer''') and '''benign kidney tumours'''.  Medical renal diseases are dealt with in the [[medical renal diseases]] article.


Pediatric kidney tumours are dealt with in the ''[[pediatric kidney tumours]]'' article.
Pediatric kidney tumours are dealt with in the ''[[pediatric kidney tumours]]'' article.


=Renal specimens=
=Renal specimens=
*Renal biopsy - usually for renal onocytoma vs. renal cell carcinoma (RCC) ''or'' medical diseases - see ''[[medical kidney]]''.
*[[Renal biopsy]] - usually for [[renal oncocytoma]] vs. renal cell carcinoma (RCC) ''or'' medical diseases - see ''[[medical kidney]]''.
*Partial nephrectomy.
*[[Partial nephrectomy]].
*Nephrectomy.
*[[Nephrectomy]].
*Nephroureterectomy (includes ureter) - done for [[urothelial cell carcinoma]] (UCC) of the renal pelvis and ureteric UCC.
*[[Nephroureterectomy]] (includes [[ureter]]) - done for [[urothelial cell carcinoma]] (UCC) of the renal pelvis and ureteric UCC.
*Radical nephrectomy - includes Gerota's fascia.
*[[Radical nephrectomy]] - includes Gerota's fascia.
**May include the [[adrenal gland]].<ref name=Ref_WMSP|288>{{Ref WMSP|288}}</ref>
**May include the [[adrenal gland]].<ref name=Ref_WMSP|288>{{Ref WMSP|288}}</ref>


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==Anatomy==
==Anatomy==
The anatomy is important for properly staging renal neoplasms.
Layers (superficial to deep):
Layers (superficial to deep):
#Renal fascia (Gerota's fascia).
#Renal fascia (Gerota's fascia).
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#Renal capsule.  
#Renal capsule.  
#Renal parenchyma (cortex).
#Renal parenchyma (cortex).
===Sign out===
====Missed renal biopsy====
{{Main|Missed renal biopsy}}


=Tabular comparison (selected tumours)=  
=Tabular comparison (selected tumours)=  


Selected common tumours of the kidney:<ref>{{Ref GUP|281-304}}</ref><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>
Selected common tumours of the kidney:<ref name=Ref_GUP281>{{Ref GUP|281-304}}</ref><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>
{| class="wikitable"
{| class="wikitable"
|
|
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|-
|-
|IHC
|IHC
| CK7-, EMA+
| [[CK7]]-, EMA+
| AMACR+, EMA+, CK7+
| AMACR+, EMA+, CK7+
| AMACR+, E-cadherin+, CK7-
| AMACR+, E-cadherin+, CK7-
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|-
|-
|Image(s)
|Image(s)
| [http://commons.wikimedia.org/wiki/File:Renal_clear_cell_ca_%281%29_Nephrectomy.jpg CCRCC (WC)]
| [[Image:Renal_clear_cell_ca_%281%29_Nephrectomy.jpg|thumb|150px| CCRCC (WC)]]
| [http://commons.wikimedia.org/wiki/File:Papillary_renal_cell_carcinoma_intermed_mag.jpg PaRCC - intermed. (WC)], [http://commons.wikimedia.org/wiki/File:Papillary_renal_cell_carcinoma_very_high_mag.jpg PaRCC - high (WC)]
| [[Image:Papillary_renal_cell_carcinoma_intermed_mag.jpg|thumb|150px| PaRCC - intermed. (WC)]], [[Image:Papillary_renal_cell_carcinoma_very_high_mag.jpg | thumb|150px| PaRCC - high (WC)]]
|
|
|
|
| [http://commons.wikimedia.org/wiki/File:Oncocytic_chromophobe_rcc_high_mag.jpg ChRCC (WC)]
| [[Image:Oncocytic_chromophobe_rcc_high_mag.jpg|thumb|150px| ChRCC (WC)]]
| [http://commons.wikimedia.org/wiki/File:Renal_oncocytoma2.jpg Oncocytoma (WC)]
| [[Image:Renal_oncocytoma2.jpg|thumb|150px| Oncocytoma (WC)]]
|}
|}
Notes:
Notes:
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===Tabular comparison of oncocytoma and chromophobe RCC===
===Tabular comparison of oncocytoma and chromophobe RCC===
Histomorphologic features useful to distinguish chromophobe RCC (eosinophilic variant) and oncocytoma:<ref name=pmid9844591>{{Cite journal  | last1 = Tickoo | first1 = SK. | last2 = Amin | first2 = MB. | title = Discriminant nuclear features of renal oncocytoma and chromophobe renal cell carcinoma. Analysis of their potential utility in the differential diagnosis. | journal = Am J Clin Pathol | volume = 110 | issue = 6 | pages = 782-7 | month = Dec | year = 1998 | doi =  | PMID = 9844591 }}
Histomorphologic features useful to distinguish chromophobe RCC (eosinophilic variant) and oncocytoma:<ref name=pmid9844591>{{Cite journal  | last1 = Tickoo | first1 = SK. | last2 = Amin | first2 = MB. | title = Discriminant nuclear features of renal oncocytoma and chromophobe renal cell carcinoma. Analysis of their potential utility in the differential diagnosis. | journal = Am J Clin Pathol | volume = 110 | issue = 6 | pages = 782-7 | month = Dec | year = 1998 | doi =  | PMID = 9844591 }}</ref>
</ref>
{| class="wikitable sortable"
{| class="wikitable"
! Morphologic feature
|
! [[ChRCC]] (eosinophilic<br> variant)
| '''ChRCC (eosinophilic<br> variant)'''
! [[Renal oncocytoma]]
| '''Renal oncocytoma'''
|-
|-
| Nuclear morphology
| Nuclear morphology
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|-
|-
| Image
| Image
| [http://commons.wikimedia.org/wiki/File:Oncocytic_chromophobe_rcc_high_mag.jpg ChRCC (WC)]
| [[Image:Oncocytic_chromophobe_rcc_high_mag.jpg |thumb|150px|ChRCC (WC/Nephron)]]
| [http://commons.wikimedia.org/wiki/File:Renal_oncocytoma2.jpg Oncocytoma (WC)]
| [[Image:Renal_oncocytoma2.jpg |thumb|150px|Oncocytoma (WC/Nephron)]]
|}
|}


=WHO classification=
===Common DDx===
*Based on 2004 iteration - as per WMSP, slightly modified.<ref>{{Ref WMSP|291}}</ref>
====Spindle cell tumours====
==Renal cell tumours==
Malignant spindle cell tumours of the kidney:
*[[Renal cell carcinoma with sarcomatoid differentiation]].
*[[Renal mucinous tubular and spindle cell carcinoma]].
*[[Wilms tumour]].
*[[Renal cell carcinoma, unclassified]].
 
Benign spindle cell tumours of the kidney:
*[[Angiomyolipoma]].
*[[Cystic nephroma]].
 
====Renal tumours with eosinophilic cytoplasm====
{{Main|Renal tumours with eosinophilic cytoplasm}}
 
=WHO classification of renal neoplasia=
*Based on 2004 iteration - as per WMSP, slightly modified.<ref>{{Ref WMSP|291}}</ref> Online, the classification can [http://www.urosource.com/fileadmin/user_upload/european_urology/PIIS0302283805008316.pdf found here].
===Renal cell tumours===
Common:
Common:
*[[Clear cell renal cell carcinoma]].
*[[Clear cell renal cell carcinoma]].
*[[Multilocular clear cell renal cell carcinoma]].
*[[Papillary renal cell carcinoma]].
*[[Papillary renal cell carcinoma]].
*Papillary adenoma.
*[[Renal papillary adenoma|Papillary adenoma]].
*[[Chromophobe renal cell carinoma]].
*[[Chromophobe renal cell carcinoma]].
*[[Renal oncocytoma|Oncocytoma]].
*[[Renal oncocytoma|Oncocytoma]].


Less common:
Less common:
*Carcinoma of the collecting ducts of Bellini.
*[[Multilocular clear cell renal cell carcinoma]].
*Renal cell carcinoma, unclassified.
*[[Carcinoma of the collecting ducts of Bellini]].
*[[Renal cell carcinoma, unclassified]].
*[[Renal medullary carcinoma]].
*[[Renal medullary carcinoma]].
*[[Renal tumour with Xp11.2 translocation|Xp11 translocation carcinoma]].
*[[Renal tumour with Xp11.2 translocation|Xp11 translocation carcinoma]].
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*Carcinoma associated with neuroblastoma.
*Carcinoma associated with neuroblastoma.


==Metanephric tumours==
===Metanephric tumours===
*[[Metanephric adenoma]].
*[[Metanephric adenoma]].
*[[Metanephric stromal tumour]].
*[[Metanephric stromal tumour]].
*[[Metanephric adenofibroma]].
*[[Metanephric adenofibroma]].


==Nephroblastic tumours==
===Nephroblastic tumours===
*Nephrogenic rests.
*Nephrogenic rests.
*[[Nephroblastoma]].
*[[Nephroblastoma]].


==Mesenchymal tumours==
===Mesenchymal tumours===
Childhood:
Childhood:
*[[Clear cell sarcoma of the kidney]].
*[[Clear cell sarcoma of the kidney]].
*[[Rhabdoid tumour]].
*[[Rhabdoid tumour]].
*[[Mesoblastic nephroma|Congenital mesoblastic nephroma]]
*[[Mesoblastic nephroma|Congenital mesoblastic nephroma]].


Adults:  
Adults:  
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**[[Renomedullary interstitial cell tumour]].
**[[Renomedullary interstitial cell tumour]].
Other:
Other:
**[[Angiomyolipoma]].
*[[Angiomyolipoma]].
**[[Epithelioid angiomyolipoma]].
*[[Epithelioid angiomyolipoma]].
**[[Leiomyosarcoma]].
*[[Leiomyosarcoma]].
**[[Angiosarcoma]].
*[[Angiosarcoma]].
**[[Pleomorphic undifferentiated sarcoma]].
*[[Pleomorphic undifferentiated sarcoma]].
**[[Hemangiopericytoma]].
*[[Hemangiopericytoma]].
**[[Solitary fibrous tumour]].
*[[Solitary fibrous tumour]].
**[[Osteosarcoma]].
*[[Osteosarcoma]].
**[[Schwannoma]].
*[[Schwannoma]].


==Mixed mesenchymal and epithelial tumours==
===Mixed mesenchymal and epithelial tumours===
*[[Cystic nephroma]].
*[[Cystic nephroma]].
*[[Mixed epithelial and stromal tumour]].
*[[Mixed epithelial and stromal tumour]].
*[[Synovial sarcoma]].
*[[Synovial sarcoma]].


==Others==
===Others===
*[[Neuroendocrine tumours]].
*[[Neuroendocrine tumours]].
*Hematologic tumours.
*Hematologic tumours.
*Germ cell tumours.
*[[Germ cell tumour]]s.
*Metastases.
*[[Metastases]].
 
==Vancouver modification of WHO classification==
In 2012/2013, several additions were made:<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>
*[[Tubulocystic renal cell carcinoma]].
*[[Acquired cystic disease associated renal cell carcinoma]].
*[[Clear cell papillary renal cell carcinoma]] (clear cell tubulopapillary renal cell carcinoma).
*[[Hereditary leiomyomatosis renal cell carcinoma syndrome associated renal cell carcinoma]].
*MiT family translocation renal cell carcinoma - includes:
**[[t(6;11) renal cell carcinoma]].
 
"Emerging" entities (as per Vancouver) are:
*[[Thyroid-like follicular renal cell carcinoma]].
*[[Succinate dehydrogenase-deficient renal cell carcinoma]].
*[[ALK translocation renal cell carcinoma]].
 
==Entities proposed after Vancouver==
*[[Eosinophilic, solid and cystic renal cell carcinoma]] - part of 2022 WHO Classification.
*[[Biphasic hyalinizing psammomatous renal cell carcinoma]].
*[[Papillary renal neoplasm with reverse polarity]].
*[[Low-grade oncocytic renal tumour]].


=Renal cell carcinoma=
=Renal cell carcinoma=
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*Male>female (~2:1).
*Male>female (~2:1).
*Hereditary - familial syndromes (see [[Hereditary RCC]]).
*Hereditary - familial syndromes (see [[Hereditary RCC]]).
*[[Obesity]].<ref name=pmid8770461>{{Cite journal  | last1 = Chow | first1 = WH. | last2 = McLaughlin | first2 = JK. | last3 = Mandel | first3 = JS. | last4 = Wacholder | first4 = S. | last5 = Niwa | first5 = S. | last6 = Fraumeni | first6 = JF. | title = Obesity and risk of renal cell cancer. | journal = Cancer Epidemiol Biomarkers Prev | volume = 5 | issue = 1 | pages = 17-21 | month = Jan | year = 1996 | doi =  | PMID = 8770461 }}</ref>


===Subtypes of RCC===
===Subtypes of RCC===
RCC (renal cell carcinoma) comes in different subtypes:<ref name=Ref_PBoD1016>{{Ref PBoD|1016}}</ref>
RCC (renal cell carcinoma) comes in different subtypes:<ref name=Ref_PBoD1016>{{Ref PBoD|1016}}</ref>
*Clear cell carcinoma (70-80% of RCC) -- abbrev. CCRCC,
*Clear cell carcinoma (70-80% of RCC) -- abbrev. CCRCC.
*Papillary renal cell carcinoma (10-15% of RCC) -- abbrev. PRCC,
*Papillary renal cell carcinoma (10-15% of RCC) -- abbrev. PRCC.
*Chromophobe renal carcinoma (5% of RCC) -- abbrev. ChRCC,
*Chromophobe renal carcinoma (5% of RCC) -- abbrev. ChRCC.
*Collecting duct (Bellini duct) carcinoma (1% of RCC).
*Collecting duct (Bellini duct) carcinoma (1% of RCC).


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*Subtype is an independent predictor of mortality - but adds very little to multivariate models with staging information.<ref name=pmid19076149>{{Cite journal  | last1 = Capitanio | first1 = U. | last2 = Cloutier | first2 = V. | last3 = Zini | first3 = L. | last4 = Isbarn | first4 = H. | last5 = Jeldres | first5 = C. | last6 = Shariat | first6 = SF. | last7 = Perrotte | first7 = P. | last8 = Antebi | first8 = E. | last9 = Patard | first9 = JJ. | title = A critical assessment of the prognostic value of clear cell, papillary and chromophobe histological subtypes in renal cell carcinoma: a population-based study. | journal = BJU Int | volume = 103 | issue = 11 | pages = 1496-500 | month = Jun | year = 2009 | doi = 10.1111/j.1464-410X.2008.08259.x | PMID = 19076149 }}</ref>
*Subtype is an independent predictor of mortality - but adds very little to multivariate models with staging information.<ref name=pmid19076149>{{Cite journal  | last1 = Capitanio | first1 = U. | last2 = Cloutier | first2 = V. | last3 = Zini | first3 = L. | last4 = Isbarn | first4 = H. | last5 = Jeldres | first5 = C. | last6 = Shariat | first6 = SF. | last7 = Perrotte | first7 = P. | last8 = Antebi | first8 = E. | last9 = Patard | first9 = JJ. | title = A critical assessment of the prognostic value of clear cell, papillary and chromophobe histological subtypes in renal cell carcinoma: a population-based study. | journal = BJU Int | volume = 103 | issue = 11 | pages = 1496-500 | month = Jun | year = 2009 | doi = 10.1111/j.1464-410X.2008.08259.x | PMID = 19076149 }}</ref>
*CCRCC tends to be worse than ChRCC and PRCC, probably due to higher incidence of mets.<ref name=pmid17886093>{{Cite journal  | last1 = Delahunt | first1 = B. | last2 = Bethwaite | first2 = PB. | last3 = Nacey | first3 = JN. | title = Outcome prediction for renal cell carcinoma: evaluation of prognostic factors for tumours divided according to histological subtype. | journal = Pathology | volume = 39 | issue = 5 | pages = 459-65 | month = Oct | year = 2007 | doi = 10.1080/00313020701570061 | PMID = 17886093 }}</ref>
*CCRCC tends to be worse than ChRCC and PRCC, probably due to higher incidence of mets.<ref name=pmid17886093>{{Cite journal  | last1 = Delahunt | first1 = B. | last2 = Bethwaite | first2 = PB. | last3 = Nacey | first3 = JN. | title = Outcome prediction for renal cell carcinoma: evaluation of prognostic factors for tumours divided according to histological subtype. | journal = Pathology | volume = 39 | issue = 5 | pages = 459-65 | month = Oct | year = 2007 | doi = 10.1080/00313020701570061 | PMID = 17886093 }}</ref>
*The exam answer (worst to best): clear cell RCC, papillary RCC, chromophobe RCC.
===IHC - is it RCC?===
===IHC - is it RCC?===
*RCC Ma (+), CD10 (+) -- specific for RCC<ref>{{cite journal |author=Zhou M, Roma A, Magi-Galluzzi C |title=The usefulness of immunohistochemical markers in the differential diagnosis of renal neoplasms |journal=Clin. Lab. Med. |volume=25 |issue=2 |pages=247?7 |year=2005 |month=June |pmid=15848735 |doi=10.1016/j.cll.2005.01.004 |url=}}</ref>
*RCC Ma (+), CD10 (+) -- specific for RCC<ref>{{cite journal |author=Zhou M, Roma A, Magi-Galluzzi C |title=The usefulness of immunohistochemical markers in the differential diagnosis of renal neoplasms |journal=Clin. Lab. Med. |volume=25 |issue=2 |pages=247?7 |year=2005 |month=June |pmid=15848735 |doi=10.1016/j.cll.2005.01.004 |url=}}</ref>
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*Papillary RCC type 1 vs. papillary RCC type 2:
*Papillary RCC type 1 vs. papillary RCC type 2:
** E-cadherin +ve in PRCC type 2.<ref name=pmid14657952>{{cite journal |author=Langner C, Ratschek M, Rehak P, Schips L, Zigeuner R |title=Expression of MUC1 (EMA) and E-cadherin in renal cell carcinoma: a systematic immunohistochemical analysis of 188 cases |journal=Mod. Pathol. |volume=17 |issue=2 |pages=180? |year=2004 |month=February |pmid=14657952 |doi=10.1038/modpathol.3800032 |url=}}</ref>
** E-cadherin +ve in PRCC type 2.<ref name=pmid14657952>{{cite journal |author=Langner C, Ratschek M, Rehak P, Schips L, Zigeuner R |title=Expression of MUC1 (EMA) and E-cadherin in renal cell carcinoma: a systematic immunohistochemical analysis of 188 cases |journal=Mod. Pathol. |volume=17 |issue=2 |pages=180? |year=2004 |month=February |pmid=14657952 |doi=10.1038/modpathol.3800032 |url=}}</ref>
**EMA (MUC1) +ve in PRCC type 1.<ref name=pmid14657952/>
**[[EMA]] (MUC1) +ve in PRCC type 1.<ref name=pmid14657952/>


*ChRCC vs. oncocytoma (ONC):
*ChRCC vs. oncocytoma (ONC):
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*Pathologically, this is not very difficult.   
*Pathologically, this is not very difficult.   
*On gross specimens, it is almost always obvious what one is dealing with:
*On gross specimens, it is almost always obvious what one is dealing with:
**UCC = ''nephroureterectomy''.
**[[UCC]] = ''[[nephroureterectomy]]''.
**RCC = ''partial nephrectomy'', ''nephrectomy'' or ''radical nephrectomy''.
**[[RCC]] = ''[[partial nephrectomy]]'', ''nephrectomy'' or ''[[radical nephrectomy]]''.


==Hereditary renal cell carcinoma==
===Renal cell carcinoma with sarcomatoid differentiation===
The classics - which are ''all'' autosomal dominant:<ref name=Ref_PBoD1016>{{Ref PBoD|1016}}</ref>
*[[AKA]] ''sarcomatoid renal cell carcinoma''.
# [[Von Hippel-Lindau syndrome]].
{{Main|Renal cell carcinoma with sarcomatoid differentiation}}
#* VHL gene mutation.
#* Clear cell RCC.
# Hereditary clear cell carcinoma.
#* VHL gene mutation. 
# Hereditary papillary carcinoma
#* MET proto-oncogene mutation.
#* PaRCC type 1.<ref name=Ref_WMSP290>{{Ref WMSP|290}}</ref>
# Hereditary leiomyomatosis and renal cell cancer:<ref name=Ref_WMSP290>{{Ref WMSP|290}}</ref>
#* FH (fumarate hydratase) gene mutation.<ref name=omim136850>{{OMIM|136850}}</ref>
#* PaRCC type 2.
#* Benign leiomyomas skin/uterus.
#* Uterine [[leiomyosarcoma]].
# Birt–Hogg–Dubé syndrome:<ref name=Ref_WMSP290>{{Ref WMSP|290}}</ref>
#* FLCN (folliculin) gene mutation.<ref name=omim135150>{{OMIM|135150}}</ref>
#* Skin lesions (fibrofolliculoma, trichodiscoma, [[acrochordon]]).
#* ChRCC most common, other types seen (e.g. [[renal oncocytoma|oncocytoma]]).
#* Variable penetrance (autosomal dominant).


Others:
===Renal cell carcinoma with rhabdoid morphology===
* Hereditary papillary carcinoma (TFE3 related translocations).<ref name=omim314310>{{OMIM|314310}}</ref>
*[[AKA]] ''renal cell carcinoma with rhabdoid change''.
{{Main|Renal cell carcinoma with rhabdoid morphology}}


Notes:<br>
==Hereditary renal cell carcinoma==
*A total of ten hereditary renal cancer syndromes have been described.  In eight of the ten the gene is known.<ref name=pmid20817385>{{Cite journal  | last1 = Verine | first1 = J. | last2 = Pluvinage | first2 = A. | last3 = Bousquet | first3 = G. | last4 = Lehmann-Che | first4 = J. | last5 = de Bazelaire | first5 = C. | last6 = Soufir | first6 = N. | last7 = Mongiat-Artus | first7 = P. | title = Hereditary renal cancer syndromes: an update of a systematic review. | journal = Eur Urol | volume = 58 | issue = 5 | pages = 701-10 | month = Nov | year = 2010 | doi = 10.1016/j.eururo.2010.08.031 | PMID = 20817385 }}</ref>
{{Main|Hereditary renal cell carcinoma}}
 
==Grading RCC==
===General===
Some subtypes are graded based on the '''Fuhrman system''' which considers:<ref name=Ref_GUP282>{{Ref GUP|282}}</ref>
*Nuclear pleomorphism (size, shape).
*Chromatin pattern.
*Nucleoli prominence.


Notes:
==Renal cell carcinoma grading==
*The system was validated for clear cell RCC. 
{{Main|Renal cell carcinoma grading}}
*Fuhrman nuclear grade is '''not''' prognostic in ''chromophobe RCC'' and should not be used in that context.<ref name=pmid17527087>{{Cite journal  | last1 = Delahunt | first1 = B. | last2 = Sika-Paotonu | first2 = D. | last3 = Bethwaite | first3 = PB. | last4 = McCredie | first4 = MR. | last5 = Martignoni | first5 = G. | last6 = Eble | first6 = JN. | last7 = Jordan | first7 = TW. | title = Fuhrman grading is not appropriate for chromophobe renal cell carcinoma. | journal = Am J Surg Pathol | volume = 31 | issue = 6 | pages = 957-60 | month = Jun | year = 2007 | doi = 10.1097/01.pas.0000249446.28713.53 | PMID = 17527087 }}</ref>


===Criteria & grades===
==Renal cell carcinoma staging==
*Grade 1: no nucleoli, near 'normal' appearance.
{{Main|Kidney cancer staging}}
*Grade 2: finely granular chromatin (key feature), no nuclei visible with 10x objective lens.
*Grade 3: [[nucleoli]] seen easily (key feature).
*Grade 4: prominent pleomorphism (key feature), hyperchromasia, [[macronucleoli]].
Note: Most tumours are grade 2 & 3.


===Fuhrman grading in short===
===Renal sinus invasion===
*1 vs. 2: grade 2 has granular chromatin, grade 2 has nucleoli visible @ 20x objective.<ref>AE. June 2010.</ref>
{{Main|Kidney cancer staging}}
*2 vs. 3: grade 3 has nucleoli @ 10x objective.
*3 vs. 4: grade 4 has pleomorphism/hyperchromasia.


==Clear cell renal cell carcinoma==
==Clear cell renal cell carcinoma==
*Often abbreviated ''CRCC''.
{{Main|Clear cell renal cell carcinoma}}
 
===General===
*Most common subtype of RCC.
*May be associated with [[Von Hippel-Lindau syndrome]].
 
===Gross===
*Gold/yellow.
*+/-Haemorrhage (common).
*+/-Necrosis (common in large tumours).
*+/-Calcification.
*+/-Cysts.
 
===Microscopic===
Features:<ref name=Ref_PBoD1017-8>{{Ref PBoD|1017-8}}</ref>
*Solid or [[trabecular]] pattern.
*Polygonal cells.
*Clear cytoplasm.
*Central nucleus.
*Delicate branching vasculature.
**Often called "chicken wire-like" vasculature.
 
Notes:
*Cytoplasm may be eosinophilic.<ref name=Ref_WMSP291>{{Ref WMSP|291}}</ref>
**This change is typically focal - other areas have a classic appearance.
**Chicken wire-like vasculature present - helps distinguish from other tumours.
*[[Hyaline bodies]] common.<ref>AFIP Renal Tumours Book.</ref>
**Not common in papillary RCC.
 
Clear cell vs. [[adrenocortical carcinoma]]:
*ACC: EMA- (epithelial membrane antigen), cytokeratin mostly neg., inhibin+ (neg. in RCC).<ref name=Ref_GUP285>{{Ref GUP|285}}</ref>
 
Images:
*[http://commons.wikimedia.org/wiki/File:Clear_cell_renal_cell_carcinoma_intermed_mag.jpg CRCC - intermed. mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Clear_cell_renal_cell_carcinoma_high_mag.jpg CRCC - high mag. (WC)].
 
===IHC===
*CK7 -ve.
*CK20 -ve.
*Hale's colloidal iron -ve.
**+ve in chromophobe RCC.
 
Note: ''[[Hale's colloidal iron]]'' does not stain iron... it stains hemosiderin.<ref>Latta. 27 January 2009.</ref>
Clear cell vs. chromophobe:
*Chromophobe: "translucent" (NOT quite clear), reticulated, Hale's colloidal iron stain+, CK7+ (cell membrane).


==Multilocular cystic renal cell carcinoma==
==Multilocular cystic renal cell carcinoma==
===General===
{{Main|Multilocular cystic renal cell carcinoma}}
*No recurrences or metastasis in the literature.<ref name=Ref_WMSP292>{{Ref WMSP|292}}</ref>
**This makes one wonder... is it really cancer.
*Case report rare.<ref name=pmid21366448>{{Cite journal  | last1 = Agarwal | first1 = S. | last2 = Agrawal | first2 = U. | last3 = Mohanty | first3 = NK. | last4 = Saxena | first4 = S. | title = Multilocular cystic renal cell carcinoma: a case report of a rare entity. | journal = Arch Pathol Lab Med | volume = 135 | issue = 3 | pages = 290-2 | month = Mar | year = 2011 | doi = 10.1043/2010-0243-LE.1 | PMID = 21366448 }}</ref>
 
===Gross===
Features:<ref name=Ref_WMSP292>{{Ref WMSP|292}}</ref>
*Cystic with thin septa.
*Well circumscribed.
 
Note:
*This tumour, radiologically, can often be separated from other cystic tumours.<ref name=pmid21722289>{{Cite journal  | last1 = You | first1 = D. | last2 = Shim | first2 = M. | last3 = Jeong | first3 = IG. | last4 = Song | first4 = C. | last5 = Kim | first5 = JK. | last6 = Ro | first6 = JY. | last7 = Hong | first7 = JH. | last8 = Ahn | first8 = H. | last9 = Kim | first9 = CS. | title = Multilocular cystic renal cell carcinoma: clinicopathological features and preoperative prediction using multiphase computed tomography. | journal = BJU Int | volume =  | issue =  | pages =  | month = Jul | year = 2011 | doi = 10.1111/j.1464-410X.2011.10247.x | PMID = 21722289 }}</ref>
===Microscopic===
Features:<ref name=Ref_WMSP292>{{Ref WMSP|292}}</ref>
*Polygonal cells within the septa.
*Clear cytoplasm.
*+/-Calcification (common).
 
DDx:
*Cystic renal disease with macrophages in the septa.
*Cystic clear cell renal cell carcinoma.
===IHC===
*EMA +ve.
*Keratins +ve.
*CD68 -ve.


==Papillary renal cell carcinoma==
==Papillary renal cell carcinoma==
*Abbreviated ''PRCC'', ''PaRCC'' and ''papillary RCC''.
{{Main|Papillary renal cell carcinoma}}
===General===
*Often subclassified<ref name=Ref_GUP289>{{Ref GUP|289}}</ref> into ''type 1'' and ''type 2'' -- see ''microscopic''.
**Type 1 and Type 2 are different on a cytogenetic and molecular basis.<ref>{{Cite journal  | last1 = Klatte | first1 = T. | last2 = Pantuck | first2 = AJ. | last3 = Said | first3 = JW. | last4 = Seligson | first4 = DB. | last5 = Rao | first5 = NP. | last6 = LaRochelle | first6 = JC. | last7 = Shuch | first7 = B. | last8 = Zisman | first8 = A. | last9 = Kabbinavar | first9 = FF. | title = Cytogenetic and molecular tumor profiling for type 1 and type 2 papillary renal cell carcinoma. | journal = Clin Cancer Res | volume = 15 | issue = 4 | pages = 1162-9 | month = Feb | year = 2009 | doi = 10.1158/1078-0432.CCR-08-1229 | PMID = 19228721 }}</ref>
 
====Epidemiology====
*Associated with ''[[acquired renal cystic disease]]''.<ref>{{Ref DARP|438}}</ref>
*May be familial.
 
===Microsopic===
Features:<ref name=Ref_PBoD1017-8>{{Ref PBoD|1017-8}}</ref>
*Cuboidal or low columnar cell in papillae.
*Interstitial foam cells in vascular cores - '''key feature'''.<ref>ALS Feb 9, 2009.</ref>
**Most sensitive and specific feature of PRCC.<ref>{{cite journal |author=Granter SR, Perez-Atayde AR, Renshaw AA |title=Cytologic analysis of papillary renal cell carcinoma |journal=Cancer |volume=84 |issue=5 |pages=303?8 |year=1998 |month=October |pmid=9801205 |doi= |url=http://dx.doi.org/10.1002/(SICI)1097-0142(19981025)84:5<303::AID-CNCR6>3.0.CO;2-7}}</ref>
*Highly vascular.
 
Size criterion:
*Papillary lesions '''''must''''' be >0.5 cm to be called ''carcinoma''; smaller lesions (<=0.5 cm) are called ''[[papillary adenoma]]s''.<ref name=Ref_GUP288>{{Ref GUP|288}}</ref>
 
Mnemonic ''HIP'':  '''h'''ighly vascular, '''i'''nterstitial foam cells, '''p'''apillae.
 
DDx:
*Clear cell RCC.
**Papillary: +histiocytes, +intracellular hemosiderin, [[CK7]]+.
*[[Clear cell papillary renal cell carcinoma]].
*[[Metanephric adenoma]] - esp. solid PRCC type 1.
*[[Collecting duct carcinoma]] - esp. PRCC type 2.
 
====Histological subtyping====
Subtypes:<ref name=Ref_GUP289>{{Ref GUP|289}}</ref>
*''Type 1'' - single layer of cells on basement membrane.
** usually low grade nuclear features, i.e. low Fuhrman grade.
*''Type 2'' - pseudostratification of cells.
** Usually high grade nuclear features, i.e. high Fuhrman grade.
 
===IHC===
Features:<ref name=Ref_GUP289>{{Ref GUP|289}}</ref>
*AMACR +ve.<ref>ALS Feb 9, 2009.</ref>
*HMWCK (34betaE12) +ve.
*Panker (AE1/AE3) +ve.
*CK7 +ve ~90% of type 1, 20% of type 2.
 
More reading:
*[http://www.e-immunohistochemistry.info/web/Papillary_renal_cell_carcinoma.htm e-immunohistochemistry.info]
 
===Molecular===
Features:<ref name=Ref_PBoD1016>{{Ref PBoD|1016}}</ref>
*Sporadic: trisomies 7, 16, 17.
*Familial: trisomy 7.
**Chromosome 7 = location of MET gene.
 
Note:
*Not used for diagnosis.<ref>{{Ref WMSP|292}}</ref>


==Chromophobe renal cell carcinoma==
==Chromophobe renal cell carcinoma==
*Abbreviated ''ChRCC''.
{{Main|Chromophobe renal cell carcinoma}}
===General===
*Least common of the common types of RCC.
*''Fuhrman grading'' for this entity is controversial, as it does not appear to have any predictive value.<ref name=pmid17527087>{{Cite journal  | last1 = Delahunt | first1 = B. | last2 = Sika-Paotonu | first2 = D. | last3 = Bethwaite | first3 = PB. | last4 = McCredie | first4 = MR. | last5 = Martignoni | first5 = G. | last6 = Eble | first6 = JN. | last7 = Jordan | first7 = TW. | title = Fuhrman grading is not appropriate for chromophobe renal cell carcinoma. | journal = Am J Surg Pathol | volume = 31 | issue = 6 | pages = 957-60 | month = Jun | year = 2007 | doi = 10.1097/01.pas.0000249446.28713.53 | PMID = 17527087 }}</ref>


There are two subtypes:<ref name=Ref_GUP293>{{Ref GUP|293}}</ref>
==Clear cell papillary renal cell tumour==
*Classic.
In the past, it was known as ''clear cell papillary renal cell carcinoma''.
*Eosinophilic variant.
{{Main|Clear cell papillary renal cell tumour}}


===Gross===
==Unclassified renal cell carcinoma==
*Tan, light-brown.
*Abbreviated ''URCC''.
*Solitary.
{{Main|Unclassified renal cell carcinoma}}
*Well-circumscribed.
 
===Microscopic===
====Classic====
Features - classic type (3 P's [[mnemonic]]):<ref>{{Ref PBoD|1016-7}}</ref><ref name=Ref_GUP293>{{Ref GUP|293}}</ref>
*Pale cytoplasm, with wisps of eosinophilic material; the cells are not completely clear, they have "cobwebs".
*Perinuclear clearing, i.e. a pale halo surrounds the nucleus - '''key feature'''.
*Periphery of cell distinct, i.e. cell membrane is easy to discern.
 
Notes:
*May have [[psammoma bodies]].
 
DDx:
*Clear cell RCC (classic).
**Perinuclear clearing is ''not'' seen in clear cell RCC.
**ChRCC has wisps in the cytoplasm.
 
====Eosinophilic variant====
Features - eosinophilic variant:<ref name=Ref_GUP293>{{Ref GUP|293}}</ref>
*'''Eosinophilic''' (finely granular) cytoplasm.
*Perinuclear clearing - '''key feature'''.
*Periphery of cell distinct.
*'''Smaller cells''' than classic subtype.
 
Notes:
#May have [[psammoma bodies]].
 
DDx:
#[[Renal oncocytoma|Oncocytoma]] - particularly the eosinophilic variant.
#*IHC may be useful to differentiate (CK7: oncocytoma = cytoplasm +ve, chromophobe = cell membrane +ve).
#*A comparison based on histomorphology: ''[[Kidney_tumours#Tabular_comparison_of_oncocytoma_and_chromophobe_RCC|Tabular comparison between ChRCC & oncocytoma]]''.
#**Oncocytoma typically has: no perinuclear clearing, no raisinoid nuclei, no binucleation.
#Clear cell RCC, eosinophilic variant.
#*Perinuclear clearing is ''not'' seen in clear cell RCC.
#*ChRCC has wisps in the cytoplasm.
 
Image:
*[http://commons.wikimedia.org/wiki/File:Oncocytic_chromophobe_rcc_high_mag.jpg Oncocytic chromophobe RCC (WC)].
 
===Stains===
*[[Hale's colloidal iron]] +ve (blue granular cytoplasmic).
 
Images:
*[http://www.ultrapath.org/oldsite/cases99/sep99/images/figure-3.jpg ChRCC Hale's colloidal iron (ultrapath.org)].<ref>URL: [http://www.ultrapath.org/oldsite/cases99/sep99/cotm9-2.html http://www.ultrapath.org/oldsite/cases99/sep99/cotm9-2.html]. Accessed on: 9 October 2011.</ref>
*[http://www.diagnosticpathology.org/content/4/1/21/figure/f1?highres=n ChRCC Hale's colloidal iron (diagnosticpathology.org)].
 
===IHC===
*CK7 +ve cell membrane.<ref name=Ref_GUP293>{{Ref GUP|293}}</ref>
*CD117 +ve.
*Vimentin -ve.
 
===Molecular===
*Extensive aneusomy (monosomy?):<ref name=Ref_WMSP292>{{Ref WMSP|292}}</ref>
**Loss of chromosomes: 1, 2, 6, 10, 13, 17, 21.
 
==Clear cell papillary renal cell carcinoma==
*[[AKA]] ''clear cell tubulopapillary renal cell carcinoma''.<ref name=pmid21602815>{{Cite journal  | last1 = Rohan | first1 = SM. | last2 = Xiao | first2 = Y. | last3 = Liang | first3 = Y. | last4 = Dudas | first4 = ME. | last5 = Al-Ahmadie | first5 = HA. | last6 = Fine | first6 = SW. | last7 = Gopalan | first7 = A. | last8 = Reuter | first8 = VE. | last9 = Rosenblum | first9 = MK. | title = Clear-cell papillary renal cell carcinoma: molecular and immunohistochemical analysis with emphasis on the von Hippel-Lindau gene and hypoxia-inducible factor pathway-related proteins. | journal = Mod Pathol | volume = 24 | issue = 9 | pages = 1207-20 | month = Sep | year = 2011 | doi = 10.1038/modpathol.2011.80 | PMID = 21602815 }}</ref>
 
===General===
*New entity not in the WHO classification.
 
===Microscopic===
Features:<ref name=pmid21602815/>
*Features of both:
*#Clear cell RCC.
*#Papillary RCC.
*#*Often do not have true papillae.
 
DDx:
*[[Papillary renal cell carcinoma]].
*[[Clear cell renal cell carcinoma]]
*[[Xp11.2 translocation carcinoma]].
 
===IHC===
Features:<ref name=pmid21602815/>
*CK7 +ve.
**Usu. -ve in CRCC.
*CD10 -ve.
**Usu. +ve in RCC.
*TFE3 -ve.
**By definition +ve in [[Xp11.2 translocation carcinoma]].
*AMACR -ve.
**Usu. +ve in PaRCC.
 
Others:<ref name=pmid21602815/>
*HIF-1alpha +ve.
*GLUT-1 +ve.


=Renal translocation carcinomas=
=Renal translocation carcinomas=
==Renal tumour with Xp11.2 translocation==
==Renal tumour with Xp11.2 translocation==
===General===
{{Main|Renal tumour with Xp11.2 translocation}}
*Defined by the presence of a fusion gene form with TFE3 @ Xp11.2.
*TFE3 is the gene involved in the translocation seen in [[alveolar soft part sarcoma]] (ASPS).
*Poor prognosis ~ 50% present at stage IV, majority of lymph node metastases.
*~1/3 of childhood RCC.<ref name=pmid17667536>{{Cite journal  | last1 = Argani | first1 = P. | last2 = Olgac | first2 = S. | last3 = Tickoo | first3 = SK. | last4 = Goldfischer | first4 = M. | last5 = Moch | first5 = H. | last6 = Chan | first6 = DY. | last7 = Eble | first7 = JN. | last8 = Bonsib | first8 = SM. | last9 = Jimeno | first9 = M. | title = Xp11 translocation renal cell carcinoma in adults: expanded clinical, pathologic, and genetic spectrum. | journal = Am J Surg Pathol | volume = 31 | issue = 8 | pages = 1149-60 | month = Aug | year = 2007 | doi = 10.1097/PAS.0b013e318031ffff | PMID = 17667536 }}</ref>
 
===Microscopic===
Features:<ref name=Ref_WMSP285>{{Ref WMSP|285}}</ref>
*Large cells.
*Clear or eosinophilic cytoplasm.
*Papillae or nests.
*[[Psammoma bodies]] - common.<ref name=pmid17102051>{{Cite journal  | last1 = Prasad | first1 = SR. | last2 = Humphrey | first2 = PA. | last3 = Catena | first3 = JR. | last4 = Narra | first4 = VR. | last5 = Srigley | first5 = JR. | last6 = Cortez | first6 = AD. | last7 = Dalrymple | first7 = NC. | last8 = Chintapalli | first8 = KN. | title = Common and uncommon histologic subtypes of renal cell carcinoma: imaging spectrum with pathologic correlation. | journal = Radiographics | volume = 26 | issue = 6 | pages = 1795-806; discussion 1806-10 | month =  | year =  | doi = 10.1148/rg.266065010 | PMID = 17102051 }}</ref>
**Calcification is considered the '''classic histomorphologic feature'''.
*[[Hyaline bodies]] - common.
 
Notes:
*Looks clear cell RCC ''or'' papillary RCC ''or'' a hybrid between the two.
*May resemble [[alveolar soft part sarcoma]].
 
DDx:
*Clear cell RCC.
*Papillary RCC.
*Epithelioid angiomyolipoma.
 
===IHC===
*TFE3 +ve (nucleus) - '''key feature'''.<ref name=Ref_WMSP285>{{Ref WMSP|285}}</ref>
*CD10 +ve.
*Vimentin +ve.
 
Others:
*HMB-45 & Melan A -ve.
**Positive in ''epithelioid [[angiomyolipoma]]''.
 
===Molecular===
*[[Translocation]] involving TFE3, e.g. t(X;1)(p11.2;q21).<ref name=Ref_WMSP285>{{Ref WMSP|285}}</ref>


==Renal tumour with t(6;11) translocation==
==Renal tumour with t(6;11) translocation==
===General===
*[[AKA]] ''t(6;11) renal cell carcinoma''.
*Not common.
{{main|Renal tumour with t(6;11) translocation}}
*[[Lymph node metastases]] are common.
*Essentially a pediatric tumour - case report of an adult.<ref name=pmid21884304>{{Cite journal  | last1 = Ishihara | first1 = A. | last2 = Yamashita | first2 = Y. | last3 = Takamori | first3 = H. | last4 = Kuroda | first4 = N. | title = Renal carcinoma with (6;11)(p21;q12) translocation: Report of an adult case. | journal = Pathol Int | volume = 61 | issue = 9 | pages = 539-45 | month = Sep | year = 2011 | doi = 10.1111/j.1440-1827.2011.02711.x | PMID = 21884304 }}</ref>
 
===Microscopic===
Features:
*Hyaline material between nests.
*Large cells with clear to eosinophilic cytoplasm.
 
===Molecular===
*t(6;11)(p21;q12) Alpha/TFEB.<ref name=Ref_WMSP281>{{Ref WMSP|281}}</ref>


=Benign tumours=
=Benign tumours=
==Papillary adenoma==
==Papillary adenoma of the kidney==
===General===
*[[AKA]] ''renal papillary adenoma''.
*Benign.
{{Main|Papillary adenoma of the kidney}}
 
===Microscopic===
Features:
#Histomorphology of ''[[papillary renal cell carcinoma]]''.
#'''''Must''''' be <=0.5 cm.<ref name=Ref_GUP288>{{Ref GUP|288}}</ref>
#*Larger lesions are ''papillary renal cell carcinoma''.


==Renal oncocytoma==
==Renal oncocytoma==
===General===
{{Main|Renal oncocytoma}}
*Can be difficult to distinguish radiologically from RCC (chromophobe subtype).
** ... and pathologists occasionally struggle like the radiologists.
*Benign tumour - the reason it is excised is... one cannot be certain it isn't a RCC.
 
===Gross===
*Brown, mahogany brown.
*1/3 have a characteristic central scar.<ref name=Ref_GUP302>{{Ref GUP|302}}</ref>
 
Image:
*[http://en.wikipedia.org/wiki/File:Renal_oncocytoma.jpg Renal oncocytoma with central scar (WP)].
 
===Microscopic===
Features:
*Eosinophilic cytoplasm - slightly granular '''key feature'''.
*Cells arranged in nests.
*Nuclei uniform and round.<ref name=Ref_GUP302>{{Ref GUP|302}}</ref>
**Slightly enlarged nuclei, but '''no significant''' pleomorphism (size variation) - '''important'''.
 
Notes:
*May look like eosinophilic variant of chromophobe RCC -- this is the main DDx.
**A comparison based on histomorphology: ''[[Kidney_tumours#Tabular_comparison_of_oncocytoma_and_chromophobe_RCC|Tabular comparison between ChRCC & oncocytoma]]''.
***Oncocytoma typically has: no perinuclear clearing, no raisinoid nuclei, no binucleation.
 
Images:
*[http://commons.wikimedia.org/wiki/File:Renal_oncocytoma2.jpg High mag. oncocytoma (WC)].
*[http://commons.wikimedia.org/wiki/File:Renal_oncocytoma3.jpg Intermed. mag. oncocytoma (WC)].
*[http://commons.wikimedia.org/wiki/File:Renal_oncocytoma4.jpg Low mag. oncocytoma (WC)].
 
===IHC===
*CK7 +ve (cytoplasm).
**Chromophobe renal cell carcinoma = cell membrane +ve.
*CD117 -ve. (???)


==Angiomyolipoma==
==Angiomyolipoma==
*Abbreviated ''AML''.
*Abbreviated ''AML''.
===General===
{{Main|Angiomyolipoma}}
*Benign mesenchymal tumour.
*Presentations: flank pain, hematuria, incidentaloma.<ref name=pmid18805573>{{Cite journal  | last1 = Seyam | first1 = RM. | last2 = Bissada | first2 = NK. | last3 = Kattan | first3 = SA. | last4 = Mokhtar | first4 = AA. | last5 = Aslam | first5 = M. | last6 = Fahmy | first6 = WE. | last7 = Mourad | first7 = WA. | last8 = Binmahfouz | first8 = AA. | last9 = Alzahrani | first9 = HM. | title = Changing trends in presentation, diagnosis and management of renal angiomyolipoma: comparison of sporadic and tuberous sclerosis complex-associated forms. | journal = Urology | volume = 72 | issue = 5 | pages = 1077-82 | month = Nov | year = 2008 | doi = 10.1016/j.urology.2008.07.049 | PMID = 18805573 }}</ref>
**Tumours >4 cm considered a risk for bleeding.<ref name=pmid21571778>{{Cite journal  | last1 = Abrams | first1 = J. | last2 = Yee | first2 = DC. | last3 = Clark | first3 = TW. | title = Transradial embolization of a bleeding renal angiomyolipoma. | journal = Vasc Endovascular Surg | volume = 45 | issue = 5 | pages = 470-3 | month = Jul | year = 2011 | doi = 10.1177/1538574411408352 | PMID = 21571778 }}</ref>
*AMLs occur may be elsewhere in the body, e.g. liver,<ref name=pmid15498214>{{Cite journal  | last1 = Zhang | first1 = SH. | last2 = Cong | first2 = WM. | last3 = Xian | first3 = ZH. | last4 = Wu | first4 = WQ. | last5 = Dong | first5 = H. | last6 = Wu | first6 = MC. | title = [Morphologic variants and immunohistochemical features of hepatic angiomyolipoma.] | journal = Zhonghua Bing Li Xue Za Zhi | volume = 33 | issue = 5 | pages = 437-40 | month = Oct | year = 2004 | doi =  | PMID = 15498214 }}
</ref> but are most common in the kidney.
*In the [[PEComa]] group of tumours.
 
====Epidemiology====
*May be associated with [[tuberous sclerosis]] -- 70% have an AML.
**When compared to sporadic cases:
***More often bilateral.
***Usually bigger.
 
===Microscopic===
Features:
*Smooth muscle.
*Adipose tissue - not always present<ref name=pmid15584043>{{Cite journal  | last1 = Crapanzano | first1 = JP. | title = Fine-needle aspiration of renal angiomyolipoma: cytological findings and diagnostic pitfalls in a series of five cases. | journal = Diagn Cytopathol | volume = 32 | issue = 1 | pages = 53-7 | month = Jan | year = 2005 | doi = 10.1002/dc.20179 | PMID = 15584043 }}</ref> - '''key feature'''.
*Abundant blood vessels.
 
Notes:
*There is a suggestion that an ''epithelioid'' variant is more worisome.<ref name=pmid12352384>{{Cite journal  | last1 = Nelson | first1 = CP. | last2 = Sanda | first2 = MG. | title = Contemporary diagnosis and management of renal angiomyolipoma. | journal = J Urol | volume = 168 | issue = 4 Pt 1 | pages = 1315-25 | month = Oct | year = 2002 | doi = 10.1097/01.ju.0000028200.86216.b2 | PMID = 12352384 }}</ref>
**This is not confirmed by all studies.<ref name=pmid18852677>{{Cite journal  | last1 = Aydin | first1 = H. | last2 = Magi-Galluzzi | first2 = C. | last3 = Lane | first3 = BR. | last4 = Sercia | first4 = L. | last5 = Lopez | first5 = JI. | last6 = Rini | first6 = BI. | last7 = Zhou | first7 = M. | title = Renal angiomyolipoma: clinicopathologic study of 194 cases with emphasis on the epithelioid histology and tuberous sclerosis association. | journal = Am J Surg Pathol | volume = 33 | issue = 2 | pages = 289-97 | month = Feb | year = 2009 | doi = 10.1097/PAS.0b013e31817ed7a6 | PMID = 18852677 }}</ref>
 
DDx:
*[[Clear cell renal cell carcinoma]] eosinophilic variant - esp. if epithelioid.
 
====Cytologic====
Features<ref name=pmid15584043>{{Cite journal  | last1 = Crapanzano | first1 = JP. | title = Fine-needle aspiration of renal angiomyolipoma: cytological findings and diagnostic pitfalls in a series of five cases. | journal = Diagn Cytopathol | volume = 32 | issue = 1 | pages = 53-7 | month = Jan | year = 2005 | doi = 10.1002/dc.20179 | PMID = 15584043 }}</ref>
*Nuclei - round/ovoid.
*Chromatin - bland.
 
===IHC===
*Melanocytic markers +ve<ref name=Ref_GUP324>{{Ref GUP|324}}</ref> (e.g. HMB-45 +ve).
*Epithelial markers -ve.<ref name=Ref_GUP324>{{Ref GUP|324}}</ref>
*SMA +ve.
*CD117 +/-.
 
*Ki-67:<ref name=pmid18839327>{{Cite journal  | last1 = Ooi | first1 = SM. | last2 = Vivian | first2 = JB. | last3 = Cohen | first3 = RJ. | title = The use of the Ki-67 marker in the pathological diagnosis of the epithelioid variant of renal angiomyolipoma. | journal = Int Urol Nephrol | volume = 41 | issue = 3 | pages = 559-65 | month =  | year = 2009 | doi = 10.1007/s11255-008-9473-1 | PMID = 18839327 }}</ref>
**Epithelioid variant of AML +ve.
**Conventional AML -ve.


=Mimics=
=Mimics=
==Xanthogranulomatous pyelonephritis==
==Xanthogranulomatous pyelonephritis==
===General===
*Abbreviated ''XGP''.
*May mimic RCC (esp. radiologically).
{{Main|Xanthogranulomatous pyelonephritis}}
*Usually lower pole ???
*Associated with:
**Diabetes mellitus,
**History of UTI,<ref name=pmid17987581>{{cite journal |author=Afgan F, Mumtaz S, Ather MH |title=Preoperative diagnosis of xanthogranulomatous pyelonephritis |journal=Urol J |volume=4 |issue=3 |pages=169–73 |year=2007 |pmid=17987581 |doi= |url=}}</ref>
**Nephrolithiasis,
**GU obstruction.<ref name=pmid17098659>{{cite journal |author=Al-Ghazo MA, Ghalayini IF, Matalka II, Al-Kaisi NS, Khader YS |title=Xanthogranulomatous pyelonephritis: Analysis of 18 cases |journal=Asian J Surg |volume=29 |issue=4 |pages=257–61 |year=2006 |month=October |pmid=17098659 |doi= |url=}}</ref>
*Occasionally an indication of nephrectomy.<ref name=pmid17987581/><ref name=pmid17098659/>
*Most common organism (in the context of nephrectomy specimens) - ''Proteus mirabilis''.<ref name=pmid17098659/>
 
===Microscopic===
*Abundant macrophages.
*+/-Giant cells.
 
Image:
*[http://commons.wikimedia.org/wiki/File:Xanthogranulomatous_pyelonephritis_cd68.jpg Xanthogranulomatous_pyelonephritis - CD68 IHC (mediawiki.org)].
 
===IHC===
*CD68 +ve.
*RCC markers (CD10, RCC) all negative.
 
DDx:
*[[Malakoplakia]].
**Basophilic inclusions -- inside or outside of macrophages - often size of RBC or larger ([[Michaelis-Gutmann bodies]]).
*RCC - esp. PaRCC (as this has foamy macrophages).
*Granulomatous disease.


==Malakoplakia==
==Malakoplakia==
Line 735: Line 388:


=Rare stuffs=
=Rare stuffs=
==Juxtaglomerular cell tumour==
*[[AKA]] juxtaglomerular tumour, reninoma.<ref name=pmid18192852>{{Cite journal  | last1 = Wong | first1 = L. | last2 = Hsu | first2 = TH. | last3 = Perlroth | first3 = MG. | last4 = Hofmann | first4 = LV. | last5 = Haynes | first5 = CM. | last6 = Katznelson | first6 = L. | title = Reninoma: case report and literature review. | journal = J Hypertens | volume = 26 | issue = 2 | pages = 368-73 | month = Feb | year = 2008 | doi = 10.1097/HJH.0b013e3282f283f3 | PMID = 18192852 }}</ref>
{{Main|Juxtaglomerular cell tumour}}
==Renomedullary interstitial cell tumour==
==Renomedullary interstitial cell tumour==
*[[AKA]] ''medullary fibroma''.<ref name=pmid11054036 >{{Cite journal  | last1 = Bircan | first1 = S. | last2 = Orhan | first2 = D. | last3 = Tulunay | first3 = O. | last4 = Safak | first4 = M. | title = Renomedullary interstitial cell tumor. | journal = Urol Int | volume = 65 | issue = 3 | pages = 163-6 | month =  | year = 2000 | doi =  | PMID = 11054036 }}</ref>
*[[AKA]] ''medullary fibroma''.<ref name=pmid11054036 >{{Cite journal  | last1 = Bircan | first1 = S. | last2 = Orhan | first2 = D. | last3 = Tulunay | first3 = O. | last4 = Safak | first4 = M. | title = Renomedullary interstitial cell tumor. | journal = Urol Int | volume = 65 | issue = 3 | pages = 163-6 | month =  | year = 2000 | doi =  | PMID = 11054036 }}</ref>
 
{{Main|Renomedullary interstitial cell tumour}}
===General===
*Benign.
*Common autopsy finding.<ref name=Ref_WMSP295>{{Ref WMSP|295}}</ref><ref name=pmid10689882>{{Cite journal  | last1 = Tsurukawa | first1 = H. | last2 = Iuchi | first2 = H. | last3 = Osanai | first3 = H. | last4 = Yamaguchi | first4 = S. | last5 = Hashimoto | first5 = H. | last6 = Kaneko | first6 = S. | last7 = Yachiku | first7 = S. | title = [Renomedullary interstitial cell tumor: a case report]. | journal = Nihon Hinyokika Gakkai Zasshi | volume = 91 | issue = 1 | pages = 37-40 | month = Jan | year = 2000 | doi =  | PMID = 10689882 }}</ref>
**This is somewhat in dispute.<ref>{{Cite journal  | last1 = Kozłowska | first1 = J. | last2 = Okoń | first2 = K. | title = Renal tumors in postmortem material. | journal = Pol J Pathol | volume = 59 | issue = 1 | pages = 21-5 | month =  | year = 2008 | doi =  | PMID = 18655367 }}
</ref>
 
===Gross===
*Small, white well-circumscribed nodule in medulla.
 
Image:
*[http://library.med.utah.edu/WebPath/RENAHTML/RENAL155.html Renal medullary fibroma (utah.edu)].
 
===Microscopic===
Features:<ref name=Ref_WMSP295>{{Ref WMSP|295}}</ref><ref>URL: [http://webpathology.com/image.asp?n=16&Case=71 http://webpathology.com/image.asp?n=16&Case=71]. Accessed on: 17 October 2011.</ref>
*Small polygonal/stellate cells.
*Abundant loose/myxoid stroma.
*+/-Entrapped renal tubules.<ref name=pmid12066202>{{Cite journal  | last1 = Kuroda | first1 = N. | last2 = Toi | first2 = M. | last3 = Miyazaki | first3 = E. | last4 = Hayashi | first4 = Y. | last5 = Nakayama | first5 = H. | last6 = Hiroi | first6 = M. | last7 = Enzan | first7 = H. | title = Participation of alpha-smooth muscle actin-positive cells in renomedullary interstitial cell tumors. | journal = Oncol Rep | volume = 9 | issue = 4 | pages = 745-50 | month =  | year =  | doi =  | PMID = 12066202 }}</ref>
 
Images:
*[http://webpathology.com/image.asp?case=71&n=15 Renomedullary interstitial cell tumour - low mag. (webpathology.com)].
*[http://webpathology.com/image.asp?n=16&Case=71 Renomedullary interstitial cell tumour - high mag. (webpathology.com)]


==Metanephric adenoma==
==Metanephric adenoma==
*Should '''not''' be confused ''[[mesonephric adenoma]]'', another term for ''nephrogenic adenoma''.
*Should '''not''' be confused ''[[mesonephric adenoma]]'', another term for ''nephrogenic adenoma''.
 
**Memory device: me'''t'''anephric adenoma is a '''t'''umour.
===General===
{{Main|Metanephric adenoma}}
*Benign.
*Afflicts adults and occasionally children.
*May be associated with [[polycythemia]].<ref name=pmid21220212>{{Cite journal  | last1 = Le Nué | first1 = R. | last2 = Marcellin | first2 = L. | last3 = Ripepi | first3 = M. | last4 = Henry | first4 = C. | last5 = Kretz | first5 = JM. | last6 = Geiss | first6 = S. | title = Conservative treatment of metanephric adenoma. A case report and review of the literature. | journal = J Pediatr Urol | volume = 7 | issue = 4 | pages = 399-403 | month = Aug | year = 2011 | doi = 10.1016/j.jpurol.2010.09.010 | PMID = 21220212 }}</ref>
 
===Microscopic===
Features:<ref name=Ref_WMSP284>{{Ref WMSP|284}}</ref>
*Small uniform cells with:
**Fine chromatin.
**No apparent [[nucleolus]].
**A relatively smooth nuclear membrane. 
*Variable architecture - may be sheets or ductal.
 
DDx:
*Epithelioid nephroblastoma ([[Wilms tumour]]) - these typically have:
**Irregular nuclear membrane.
**Nucleoli.
**Mitoses (rare in ''metanephric adenoma'').
*[[Papillary renal cell carcinoma|Papillary RCC]].<ref name=Ref_WMSP284>{{Ref WMSP|284}}</ref>
 
Images:
*www:
**[http://www.nature.com/modpathol/journal/v16/n10/fig_tab/3880886f1.html Metanephric adenoma (nature.com)].
*[[WC]]:
**[http://commons.wikimedia.org/wiki/File:Metanephric_adenoma_high_mag.jpg Metanephric adenoma (WC)].
**[http://commons.wikimedia.org/wiki/File:Metanephric_adenoma_high_mag_cropped.jpg Metanephric adenoma - cropped (WC)].
**[http://commons.wikimedia.org/wiki/File:Metanephric_adenoma_-_intermed_mag.jpg Metanephric adenoma - PaRCC-like - intermed. mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Metanephric_adenoma_-_very_high_mag.jpg Metanephric adenoma - PaRCC-like - very high mag. (WC)].
 
===IHC===
*WT-1 +ve.
*CD57 +ve.
*CK7 -ve.


==Renal epithelial and stromal tumour==
==Renal epithelial and stromal tumour==
:Abbreviated ''REST''.
The lumping term for both:<ref name=pmid17414095>{{Cite journal  | last1 = Turbiner | first1 = J. | last2 = Amin | first2 = MB. | last3 = Humphrey | first3 = PA. | last4 = Srigley | first4 = JR. | last5 = De Leval | first5 = L. | last6 = Radhakrishnan | first6 = A. | last7 = Oliva | first7 = E. | title = 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. | journal = Am J Surg Pathol | volume = 31 | issue = 4 | pages = 489-500 | month = Apr | year = 2007 | doi = 10.1097/PAS.0b013e31802bdd56 | PMID = 17414095 }}</ref>
The lumping term for both:<ref name=pmid17414095>{{Cite journal  | last1 = Turbiner | first1 = J. | last2 = Amin | first2 = MB. | last3 = Humphrey | first3 = PA. | last4 = Srigley | first4 = JR. | last5 = De Leval | first5 = L. | last6 = Radhakrishnan | first6 = A. | last7 = Oliva | first7 = E. | title = 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. | journal = Am J Surg Pathol | volume = 31 | issue = 4 | pages = 489-500 | month = Apr | year = 2007 | doi = 10.1097/PAS.0b013e31802bdd56 | PMID = 17414095 }}</ref>
#[[Mixed epithelial and stromal tumour]].
#[[Mixed epithelial and stromal tumour]].
Line 804: Line 409:
==Mixed epithelial and stromal tumour==
==Mixed epithelial and stromal tumour==
*Abbreviated ''MEST''.
*Abbreviated ''MEST''.
 
{{Main|Mixed epithelial and stromal tumour of the kidney}}
===General===
*Rare - approx. 1.5% of renal neoplasms.<ref name=pmid17454754>{{Cite journal  | last1 = Mai | first1 = KT. | last2 = Elkeilani | first2 = A. | last3 = Veinot | first3 = JP. | title = Mixed epithelial and stromal tumour (MEST) of the kidney: report of 14 cases with male and PEComatous variants and proposed histopathogenesis. | journal = Pathology | volume = 39 | issue = 2 | pages = 235-40 | month = Apr | year = 2007 | doi = 10.1080/00313020701230799 | PMID = 17454754 }}</ref>
*Benign.
*Prevalence: males > females.
 
Note:
*Turbiner ''et al.''<ref name=pmid17414095>{{Cite journal  | last1 = Turbiner | first1 = J. | last2 = Amin | first2 = MB. | last3 = Humphrey | first3 = PA. | last4 = Srigley | first4 = JR. | last5 = De Leval | first5 = L. | last6 = Radhakrishnan | first6 = A. | last7 = Oliva | first7 = E. | title = 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. | journal = Am J Surg Pathol | volume = 31 | issue = 4 | pages = 489-500 | month = Apr | year = 2007 | doi = 10.1097/PAS.0b013e31802bdd56 | PMID = 17414095 }}</ref>  have suggested that ''cystic nephroma'' and ''mixed epithelial and stromal tumour (MEST)'' are one tumour.
**The lumping term ''renal epithelial and stromal tumour'', abbreviated ''REST''.
 
===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.
 
Images:
*[http://commons.wikimedia.org/wiki/File:Cystic_nephroma_low_mag.jpg Cystic nephroma - low mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Cystic_nephroma_intermed_mag.jpg Cystic nephroma - intermed. mag. (WC)].
 
===IHC===
Features:<ref name=pmid17454754>{{Cite journal  | last1 = Mai | first1 = KT. | last2 = Elkeilani | first2 = A. | last3 = Veinot | first3 = JP. | title = Mixed epithelial and stromal tumour (MEST) of the kidney: report of 14 cases with male and PEComatous variants and proposed histopathogenesis. | journal = Pathology | volume = 39 | issue = 2 | pages = 235-40 | month = Apr | year = 2007 | doi = 10.1080/00313020701230799 | PMID = 17454754 }}
</ref>
*ER +ve.
*PR +ve.
*CD10 +ve.
 
===DDx===
*Tubulocystic carcinoma.


==Cystic nephroma==
==Cystic nephroma==
 
{{Main|Cystic nephroma}}
===General===
*Turbiner ''et al.''<ref name=pmid17414095>{{Cite journal  | last1 = Turbiner | first1 = J. | last2 = Amin | first2 = MB. | last3 = Humphrey | first3 = PA. | last4 = Srigley | first4 = JR. | last5 = De Leval | first5 = L. | last6 = Radhakrishnan | first6 = A. | last7 = Oliva | first7 = E. | title = 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. | journal = Am J Surg Pathol | volume = 31 | issue = 4 | pages = 489-500 | month = Apr | year = 2007 | doi = 10.1097/PAS.0b013e31802bdd56 | PMID = 17414095 }}</ref>  has 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''.
 
===Gross===
*Bubble wrap-like appearance.
 
Image:
*[http://commons.wikimedia.org/wiki/File:Bubble_Wrap.jpg Bubble wrap (WC)].
 
===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.
 
Images:
*[http://commons.wikimedia.org/wiki/File:Cystic_nephroma_low_mag.jpg Cystic nephroma - low mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Cystic_nephroma_intermed_mag.jpg Cystic nephroma - intermed. mag. (WC)].
 
===IHC===
Features:
*ER +ve.
*PR +ve.
*CD10 +ve.


==Renal mucinous tubular and spindle cell carcinoma==
==Renal mucinous tubular and spindle cell carcinoma==
*[[AKA]] ''renal mucinous tubular spindle cell carcinoma''.
*[[AKA]] ''renal mucinous tubular spindle cell carcinoma''.
 
*[[AKA]] ''mucinous tubular and spindle cell carcinoma of the kidney''.<ref name=pmid16258504>{{Cite journal  | last1 = Brandal | first1 = P. | last2 = Lie | first2 = AK. | last3 = Bassarova | first3 = A. | last4 = Svindland | first4 = A. | last5 = Risberg | first5 = B. | last6 = Danielsen | first6 = H. | last7 = Heim | first7 = S. | title = Genomic aberrations in mucinous tubular and spindle cell renal cell carcinomas. | journal = Mod Pathol | volume = 19 | issue = 2 | pages = 186-94 | month = Feb | year = 2006 | doi = 10.1038/modpathol.3800499 | PMID = 16258504 }}</ref>
===General===
{{Main|Renal mucinous tubular and spindle cell carcinoma}}
*Rare.<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>
*Accepted by WHO in 2004 as a separate entity.<ref name=pmid19805959>{{cite journal |author=Geramizadeh B, Salehipour M, Moradi A |title=Mucinous tubular and spindle cell carcinoma of kidney: a rare case report and review of the literature |journal=Indian J Pathol Microbiol |volume=52 |issue=4 |pages=514–6 |year=2009 |pmid=19805959 |doi=10.4103/0377-4929.56144 |url=}}</ref>
*Relatively good prognosis.
 
===Microscopic===
Features:<ref name=pmid19066686>{{cite journal |author=Trabelsi A, Stita W, Yacoubi MT, Rammeh S, Hmissa S, Korbi S |title=Renal mucinous tubular and spindle cell carcinoma |journal=Can Urol Assoc J |volume=2 |issue=6 |pages=635–6 |year=2008 |month=December |pmid=19066686 |pmc=2593603 |doi= |url=}}</ref>
*Mucin - may be scant.
*Spindle cells.
 
DDx:
*Sarcomatoid papillary RCC. (???) <ref name=pmid18941398>{{cite journal |author=Dhillon J, Amin MB, Selbs E, Turi GK, Paner GP, Reuter VE |title=Mucinous tubular and spindle cell carcinoma of the kidney with sarcomatoid change |journal=Am. J. Surg. Pathol. |volume=33 |issue=1 |pages=44–9 |year=2009 |month=January |pmid=18941398 |doi=10.1097/PAS.0b013e3181829ed5 |url=}}</ref>
 
===IHC===
Features:<ref name=pmid16231179>{{cite journal |author=Ferlicot S, Allory Y, Compérat E, ''et al.'' |title=Mucinous tubular and spindle cell carcinoma: a report of 15 cases and a review of the literature |journal=Virchows Arch. |volume=447 |issue=6 |pages=978–83 |year=2005 |month=December |pmid=16231179 |doi=10.1007/s00428-005-0036-x |url=}}</ref>
*CD10 -ve.
*AE1/AE3 +ve.
*AMACR +ve.
*CK7 +ve.


==Collecting duct carcinoma==
==Collecting duct carcinoma==
*[[AKA]] ''Bellini duct carcinoma'',<ref name=pmid21393935>{{Cite journal  | last1 = Stamatiou | first1 = K. | last2 = Zizi-Sermpetzoglou | first2 = A. | title = Bellini duct carcinoma accidentally found upon investigation of uric acid lithiasis. | journal = Indian J Pathol Microbiol | volume = 54 | issue = 1 | pages = 229-30 | month =  | year =  | doi = 10.4103/0377-4929.77425 | PMID = 21393935 }}</ref> [[AKA]] ''carcinoma of the collecting ducts of Bellini''.
{{Main|Collecting duct carcinoma}}
 
===General===
*Rare.
*Poor prognosis.
*Usu. central location.
*Typically young adults.
 
===Microscopic===
Features:<ref name=Ref_GUP295>{{Ref GUP|295}}</ref>
*Tubular structures with tapered ends.
**May be described as ''tubulopapillary''.
*[[Hobnail pattern]] - cell width smaller at basement membrane than free surface.<ref name=Ref_PBoD1018>{{Ref PBoD|1018}}</ref>
*High grade nuclear features (nuclear pleomorphism).
*High mitotic rate.
 
Notes:
*Benign urothelium must present to excluded urothelial carcinoma.
*Desmoplastic stroma may be prominent.
 
DDx:
*[[Papillary renal cell carcinoma]] - type 2.
*[[Urothelial carcinoma]].
 
===IHC===
Features:<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 | URL = http://www.nature.com/modpathol/journal/v22/n2s/full/modpathol200970a.html }}</ref>
*CD117 +ve.
*E-cadherin +ve.
*CD10 -ve.
*AMACR -ve.


==Renal medullary carcinoma==
==Renal medullary carcinoma==
===General===
{{Main|Renal medullary carcinoma}}
*Rare.
*Usually young adults.
*Associated with sickle cell trait (heterozygotes for the sickle cell allele).<ref name=pmid7528470>{{cite journal |author=Davis CJ, Mostofi FK, Sesterhenn IA |title=Renal medullary carcinoma. The seventh sickle cell nephropathy |journal=Am. J. Surg. Pathol. |volume=19 |issue=1 |pages=1–11 |year=1995 |month=January |pmid=7528470 |doi= |url=}}</ref>
*Aggressive/poor prognosis.<ref name=pmid17643096>{{Cite journal  | last1 = Watanabe | first1 = IC. | last2 = Billis | first2 = A. | last3 = Guimarães | first3 = MS. | last4 = Alvarenga | first4 = M. | last5 = de Matos | first5 = AC. | last6 = Cardinalli | first6 = IA. | last7 = Filippi | first7 = RZ. | last8 = de Castro | first8 = MG. | last9 = Suzigan | first9 = S. | title = Renal medullary carcinoma: report of seven cases from Brazil. | journal = Mod Pathol | volume = 20 | issue = 9 | pages = 914-20 | month = Sep | year = 2007 | doi = 10.1038/modpathol.3800934 | PMID = 17643096 |url = http://www.nature.com/modpathol/journal/v20/n9/full/3800934a.html }}</ref>
 
Aside:
*Kidney disease assoc. with sickle cell disorders:<ref name=pmid7528470/>
*#Papillary necrosis.
*#Nephrotic syndrome.
*#Renal infarction.
*#Pyelonephritis.
 
===Gross===
Features:<ref name=pmid17643096>{{cite journal |author=Watanabe IC, Billis A, Guimarães MS, ''et al.'' |title=Renal medullary carcinoma: report of seven cases from Brazil |journal=Mod. Pathol. |volume=20 |issue=9 |pages=914–20 |year=2007 |month=September |pmid=17643096 |doi=10.1038/modpathol.3800934 |url=}}</ref>
*Well circumscribed.
*Renal medulla.
 
===Microscopic===
Features:<ref name=pmid7528470/><ref name=pmid17643096>{{cite journal |author=Watanabe IC, Billis A, Guimarães MS, ''et al.'' |title=Renal medullary carcinoma: report of seven cases from Brazil |journal=Mod. Pathol. |volume=20 |issue=9 |pages=914–20 |year=2007 |month=September |pmid=17643096 |doi=10.1038/modpathol.3800934 |url=}}</ref>
*Variable architecture:
**Reticular - classic.
**[[Adenoid cystic carcinoma]]-like appearance:
***Cystic spaces.
**Yolk sac-like.
**Tubular.
*Desmoplastic stroma - prominent.
*Inflammation:
**Lymphocytes.
**Neutrophils - margination in vessels.


DDx:
==Tubulocystic carcinoma of the kidney==
*[[Yolk sac tumour]].
{{Main|Tubulocystic carcinoma of the kidney}}
*[[Collecting duct carcinoma]].


Image:
==Acquired cystic disease-associated renal cell carcinoma==
*[http://www.nature.com/modpathol/journal/v20/n9/fig_tab/3800934f3.html#figure-title RMC (nature.com)].
{{Main|Acquired cystic disease-associated renal cell carcinoma}}


===IHC===
==Kidney metastasis==
*SMARCB1 (INI1) -ve.<ref name=Ref_WMSP286>{{Ref WMSP|286}}</ref>
*[[AKA]] ''renal metastasis'', ''metastatic kidney disease''.
{{Main|Kidney metastasis}}


=Pediatric=
=Pediatric=
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