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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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===Sign out===
===Sign out===
====Missed renal biopsy====
====Missed renal biopsy====
<pre>
{{Main|Missed renal biopsy}}
KIDNEY (LESION), LEFT, CORE BIOPSY:
- RENAL PARENCHYMA.
- NEGATIVE FOR MASS LESION, SEE COMMENT.
 
COMMENT:
No mass lesion is apparent in the tissue sampled. A re-biopsy should be considered.
 
Renal parenchyma:
- Glomeruli: seven glomeruli sampled, no apparent glomerular pathology on the H&E sections.
- Interstitium: interstitial fibrosis is not identified.
- Tubules: no pathology is apparent.
- Vessels: mild atherosclerosis, no hyalinization of arterioles apparent.
</pre>


=Tabular comparison (selected tumours)=  
=Tabular comparison (selected tumours)=  
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|-
|-
|IHC
|IHC
| CK7-, EMA+
| [[CK7]]-, EMA+
| AMACR+, EMA+, CK7+
| AMACR+, EMA+, CK7+
| AMACR+, E-cadherin+, CK7-
| AMACR+, E-cadherin+, CK7-
Line 127: Line 113:


===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 sortable"
! Morphologic feature
! Morphologic feature
Line 164: Line 149:


===Common DDx===
===Common DDx===
Spindle cell tumours:
====Spindle cell tumours====
*Malignant:
Malignant spindle cell tumours of the kidney:
**[[Renal cell carcinoma with sarcomatoid differentiation]].
*[[Renal cell carcinoma with sarcomatoid differentiation]].
**[[Renal mucinous tubular and spindle cell carcinoma]].
*[[Renal mucinous tubular and spindle cell carcinoma]].
**[[Wilms tumour]].
*[[Wilms tumour]].
*Benign:
*[[Renal cell carcinoma, unclassified]].
**[[Angiomyolipoma]].
**[[Cystic nephroma]].


=WHO classification=
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].
*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==
===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]].
*[[Renal papillary adenoma|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:
*[[Multilocular clear cell renal cell carcinoma]].
*[[Carcinoma of the collecting ducts of Bellini]].
*[[Carcinoma of the collecting ducts of Bellini]].
*[[Renal cell carcinoma, unclassified]].
*[[Renal cell carcinoma, unclassified]].
Line 192: Line 182:
*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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*[[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 tumour]]s.
*[[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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*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]]''.


===Renal cell carcinoma with sarcomatoid differentiation===
===Renal cell carcinoma with sarcomatoid differentiation===
*[[AKA]] ''sarcomatoid renal cell carcinoma''.
*[[AKA]] ''sarcomatoid renal cell carcinoma''.
====General====
{{Main|Renal cell carcinoma with sarcomatoid differentiation}}
Features:<ref name=pmid11224597>{{Cite journal  | last1 = de Peralta-Venturina | first1 = M. | last2 = Moch | first2 = H. | last3 = Amin | first3 = M. | last4 = Tamboli | first4 = P. | last5 = Hailemariam | first5 = S. | last6 = Mihatsch | first6 = M. | last7 = Javidan | first7 = J. | last8 = Stricker | first8 = H. | last9 = Ro | first9 = JY. | title = Sarcomatoid differentiation in renal cell carcinoma: a study of 101 cases. | journal = Am J Surg Pathol | volume = 25 | issue = 3 | pages = 275-84 | month = Mar | year = 2001 | doi =  | PMID = 11224597 }}</ref>
*Not recognized as a distinct entity in 2004 WHO classification.<ref name=pmid16442207>{{Cite journal  | last1 = Lopez-Beltran | first1 = A. | last2 = Scarpelli | first2 = M. | last3 = Montironi | first3 = R. | last4 = Kirkali | first4 = Z. | title = 2004 WHO classification of the renal tumors of the adults. | journal = Eur Urol | volume = 49 | issue = 5 | pages = 798-805 | month = May | year = 2006 | doi = 10.1016/j.eururo.2005.11.035 | PMID = 16442207 }}</ref>
**It is considered an indicator of progression.
**Previously considered a distinct entity.<ref name=pmid16442207/><ref name=pmid10080595>{{Cite journal  | last1 = Cangiano | first1 = T. | last2 = Liao | first2 = J. | last3 = Naitoh | first3 = J. | last4 = Dorey | first4 = F. | last5 = Figlin | first5 = R. | last6 = Belldegrun | first6 = A. | title = Sarcomatoid renal cell carcinoma: biologic behavior, prognosis, and response to combined surgical resection and immunotherapy. | journal = J Clin Oncol | volume = 17 | issue = 2 | pages = 523-8 | month = Feb | year = 1999 | doi =  | PMID = 10080595 | URL = http://jco.ascopubs.org/content/17/2/523.full }}</ref>
*Tend to present at higher stage.
*Worse prognosis when adjusted for stage.
*[[Fuhrman grade]] 4 by definition.
 
====Microscopic====
Features:<ref name=pmid11224597/>
#Renal cell carcinoma.
#Sarcomatoid component:
#*[[Fibrosarcoma]] - most common.
#*[[Pleomorphic undifferentiated sarcoma|Undifferentiated]] - common.
#*[[Rhabdomyosarcoma]] - very rare.
 
Note:
*In essence, any kidney tumour with [[spindle cell]]s<ref name=pmid15763002/> or rhabdoid cells should make one think of this.


Images:
===Renal cell carcinoma with rhabdoid morphology===
*[http://webpathology.com/image.asp?n=2&Case=70 Sarcomatoid change in RCC (webpathology.com)].
*[[AKA]] ''renal cell carcinoma with rhabdoid change''.
*[http://jco.ascopubs.org/content/27/2/235/F1.expansion Sarcomatoid changes in CCRCC (ascopubs.org)].<ref name=pmid19064974>{{Cite journal  | last1 = Golshayan | first1 = AR. | last2 = George | first2 = S. | last3 = Heng | first3 = DY. | last4 = Elson | first4 = P. | last5 = Wood | first5 = LS. | last6 = Mekhail | first6 = TM. | last7 = Garcia | first7 = JA. | last8 = Aydin | first8 = H. | last9 = Zhou | first9 = M. | title = Metastatic sarcomatoid renal cell carcinoma treated with vascular endothelial growth factor-targeted therapy. | journal = J Clin Oncol | volume = 27 | issue = 2 | pages = 235-41 | month = Jan | year = 2009 | doi = 10.1200/JCO.2008.18.0000 | PMID = 19064974 }}</ref>
{{Main|Renal cell carcinoma with rhabdoid morphology}}
*[http://www.scielo.br/img/revistas/ibju/v31n1/1a03f1.jpg Sarcomatoid changes in RCC (scielo.br)].<ref name=pmid15763002>{{Cite journal  | last1 = Dall'Oglio | first1 = MF. | last2 = Lieberknecht | first2 = M. | last3 = Gouveia | first3 = V. | last4 = Sant'Anna | first4 = AC. | last5 = Leite | first5 = KR. | last6 = Srougi | first6 = M. | title = Sarcomatoid differentiation in renal cell carcinoma: prognostic implications. | journal = Int Braz J Urol | volume = 31 | issue = 1 | pages = 10-6 | month =  | year =  | doi =  | PMID = 15763002 }}</ref>


==Hereditary renal cell carcinoma==
==Hereditary renal cell carcinoma==
The classics - which are ''all'' autosomal dominant:<ref name=Ref_PBoD1016>{{Ref PBoD|1016}}</ref>
{{Main|Hereditary renal cell carcinoma}}
# [[Von Hippel-Lindau syndrome]].
#* VHL gene mutation.
#* Clear cell RCC.
# Hereditary [[clear cell renal cell carcinoma]].
#* VHL gene mutation. 
# Hereditary [[papillary renal cell 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 [[leiomyoma]]s skin/[[uterine leiomyoma|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:
* Hereditary papillary carcinoma (TFE3 related translocations).<ref name=omim314310>{{OMIM|314310}}</ref>
 
Notes:<br>
*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>
 
===Molecular===
Recurrent molecular changes in RCC:
*Clear cell RCC:
**Loss of 3p - contains the VHL gene.
*Papillary RCC:
**Sporadic:
***Trisomy 7, 16, 17.
***Loss of Y.
**Familial:
***Trisomy 7 - contains MET gene.<ref>{{OMIM|164860}}</ref>


==Renal cell carcinoma grading==
==Renal cell carcinoma grading==
*[[AKA]] ''Fuhrman grade''.
{{Main|Renal cell carcinoma grading}}
===General===
Some RCC 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 staging==
*The system was validated for [[clear cell RCC]]. 
{{Main|Kidney cancer staging}}
*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>
*Anything with [[spindle cell]]s<ref name=pmid15763002/> or rhabdoid cells should make one think grade 4, as it is likely a ''[[renal cell carcinoma with sarcomatoid differentiation]]''.


===Criteria & grades===
===Renal sinus invasion===
*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]].
**[[Spindle cell]]s<ref name=pmid15763002/> or rhabdoid cells also make something grade 4 (see ''[[renal cell carcinoma with sarcomatoid differentiation]]'').
 
Note:
*Most tumours are grade 2 & 3.
===Fuhrman grading in short===
*1 vs. 2: grade 2 has granular chromatin, grade 2 has nucleoli visible @ 20x objective.<ref>AE. June 2010.</ref>
*2 vs. 3: grade 3 has nucleoli @ 10x objective.
*3 vs. 4: grade 4 has pleomorphism/hyperchromasia (or spindle cells or rhabdoid cells).


==Clear cell renal cell carcinoma==
==Clear cell renal cell carcinoma==
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==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]].
*[[Tubulocystic carcinoma]].
 
===IHC===
*EMA +ve.
*Keratins +ve.
*CD68 -ve.


==Papillary renal cell carcinoma==
==Papillary renal cell carcinoma==
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{{Main|Chromophobe renal cell carcinoma}}
{{Main|Chromophobe renal cell carcinoma}}


==Clear cell papillary renal cell carcinoma==
==Clear cell papillary renal cell tumour==
{{Main|Clear cell papillary renal cell carcinoma}}
In the past, it was known as ''clear cell papillary renal cell carcinoma''.
{{Main|Clear cell papillary renal cell tumour}}


==Unclassified renal cell carcinoma==
==Unclassified renal cell carcinoma==
*Abbreviated ''URCC''.
*Abbreviated ''URCC''.
===General===
{{Main|Unclassified renal cell carcinoma}}
*Uncommon.
*A WHO classification ''diagnosis of exclusion''.
*Worse prognosis than [[clear cell renal cell carcinoma]].<ref name=pmid17822461>{{Cite journal  | last1 = Karakiewicz | first1 = PI. | last2 = Hutterer | first2 = GC. | last3 = Trinh | first3 = QD. | last4 = Pantuck | first4 = AJ. | last5 = Klatte | first5 = T. | last6 = Lam | first6 = JS. | last7 = Guille | first7 = F. | last8 = de La Taille | first8 = A. | last9 = Novara | first9 = G. | title = Unclassified renal cell carcinoma: an analysis of 85 cases. | journal = BJU Int | volume = 100 | issue = 4 | pages = 802-8 | month = Oct | year = 2007 | doi = 10.1111/j.1464-410X.2007.07148.x | PMID = 17822461 }}</ref>
*High variation in the prevalence (when comparing institutions); this suggests a lack of uniformity in the diagnosis of this subtype.<ref name=pmid17822461/>
 
===Microscopic===
Features:
*Malignant tumour that is one of the following:<ref name=Ref_WMSP293>{{Ref WMSP|293}}</ref><ref name=pmid22404824>{{Cite journal  | last1 = Lopez-Beltran | first1 = A. | last2 = Kirkali | first2 = Z. | last3 = Montironi | first3 = R. | last4 = Blanca | first4 = A. | last5 = Algaba | first5 = F. | last6 = Scarpelli | first6 = M. | last7 = Yorukoglu | first7 = K. | last8 = Hartmann | first8 = A. | last9 = Cheng | first9 = L. | title = Unclassified renal cell carcinoma: a report of 56 cases. | journal = BJU Int | volume = 110 | issue = 6 | pages = 786-93 | month = Sep | year = 2012 | doi = 10.1111/j.1464-410X.2012.10934.x | PMID = 22404824 }}</ref>
*#A combination of other RCC histologic types (~35% of cases of URCC).
*#Has a "non-identifiable" pattern/unrecognizable cell type (~60% of cases of URCC).
*#Pure [[sarcomatoid RCC]] without an identifiable (epithelioid) RCC subtype (~5% of cases of URCC).<ref name=pmid22404824/>
 
DDx:
*[[Clear cell renal cell carcinoma]].
*[[Collecting duct carcinoma]].
*Undifferentiated carcinoma.
*[[Metastatic]] carcinoma.


=Renal translocation carcinomas=
=Renal translocation carcinomas=
Line 467: Line 364:


==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 of the kidney==
==Papillary adenoma of the kidney==
*[[AKA]] ''renal papillary adenoma''.
*[[AKA]] ''renal papillary adenoma''.
===General===
{{Main|Papillary adenoma of the kidney}}
*Benign.
*Considered a precursor for ''[[papillary renal cell carcinoma]]'' (PaRCC).<ref name=pmid17056094>{{Cite journal  | last1 = Wang | first1 = KL. | last2 = Weinrach | first2 = DM. | last3 = Luan | first3 = C. | last4 = Han | first4 = M. | last5 = Lin | first5 = F. | last6 = Teh | first6 = BT. | last7 = Yang | first7 = XJ. | title = Renal papillary adenoma--a putative precursor of papillary renal cell carcinoma. | journal = Hum Pathol | volume = 38 | issue = 2 | pages = 239-46 | month = Feb | year = 2007 | doi = 10.1016/j.humpath.2006.07.016 | PMID = 17056094 }}</ref>
**Stains like PaRCC.
**Found with an increased frequency within the content of PaRCC.
 
===Microscopic===
Features:
#Histomorphology of ''[[papillary renal cell carcinoma]]''.
#'''''Must''''' be <=0.5 cm.<ref name=Ref_GUP288>{{Ref GUP|288}}</ref><ref name=pmid18846240>{{Cite journal  | last1 = Algaba | first1 = F. | title = Renal adenomas: pathological differential diagnosis with malignant tumors. | journal = Adv Urol | volume =  | issue =  | pages = 974848 | month =  | year = 2008 | doi = 10.1155/2008/974848 | PMID = 18846240 | PMC = 2563151 | URL = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2563151/?tool=pubmed }}</ref>
#*[[Diagnostic size cutoff]] - larger lesions are ''papillary renal cell carcinoma''.
 
DDx:
*[[Papillary renal cell carcinoma]].
*[[Metanephric adenoma]].
 
Images:
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2563151/figure/fig2/ Renal papillary adenoma (nih.gov)].<ref name=pmid18846240/>
 
===IHC===
*AMACR +ve.<ref name=pmid17056094/>


==Renal oncocytoma==
==Renal oncocytoma==
Line 510: Line 377:
==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.
*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>
 
===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.
 
====Epithelioid angiomyolipoma====
Features:
*Carcinoma-like morphology.
*+/-Spindle cells.
*"High grade" nuclei.
**Pleomorphic nuclei.
 
DDx:
*[[Clear cell renal cell carcinoma]] eosinophilic variant - esp. if epithelioid.
 
Images:
*[http://bjr.birjournals.org/content/82/984/e249/F3.expansion.html Epithelioid AML (birjournals.org)].
*[http://radiographics.rsna.org/content/30/6/1525/F2.expansion.html Epithelioid AML (rsna.org)].
*[http://www.archivesofpathology.org/action/showFullPopup?id=i1543-2165-128-10-1176-f01&doi=10.1043%2F1543-2165%282004%29128%3C1176%3APQCAYW%3E2.0.CO%3B2 Atypical epithelioid AML (archivesofpathology.org)].<ref>{{Cite journal  | last1 = Aljerian | first1 = K. | last2 = Evans | first2 = AJ. | title = Pathologic quiz case: a 44-year-old woman with an incidental asymptomatic renal mass. Atypical epithelioid angiomyolipoma. | journal = Arch Pathol Lab Med | volume = 128 | issue = 10 | pages = 1176-8 | month = Oct | year = 2004 | doi = 10.1043/1543-2165(2004)1281176:PQCAYW2.0.CO;2 | PMID = 15387699 }}</ref>
 
====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 +ve/-ve.
 
*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]] - espically radiologically.
{{Main|Xanthogranulomatous pyelonephritis}}
*Usually lower pole.{{fact}}
*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.
 
DDx:
*[[Malakoplakia]].
**Basophilic inclusions -- inside or outside of macrophages - often size of RBC or larger ([[Michaelis-Gutmann bodies]]).
*RCC - especially [[PRCC]] (as this has foamy macrophages).
*Granulomatous disease.
*[[Chronic pyelonephritis]].
*[[Interstitial nephritis]].
 
====Image====
<gallery>
Image:Xanthogranulomatous_pyelonephritis_cd68.jpg | Xanthogranulomatous pyelonephritis - CD68 stain. (WC/Nephron)
</gallery>
===Stains===
*[[PAS-D]] -ve.
**Done to look for malakoplakia.
 
===IHC===
*CD68 +ve.
*RCC markers (CD10, RCC) all negative.
 
===Sign out===
====Compatible XGP====
<pre>
"KIDNEY" LESION, LEFT, BIOPSY:
- FIBROMUSCULAR TISSUE WITH A MIXED INFLAMMATORY INFILTRATE.
- CELLULAR DEBRIS WITH SURROUNDING LOOSELY AGGREGATED HISTIOCYTES.
- NO RENAL PARENCHYMA IDENTIFIED.
- NEGATIVE FOR MALIGNANCY.
 
COMMENT:
A SMA immunostain highlights the muscle component, and a CD68 immunostain marks
abundant histiocytes. No epithelial component is identified with a pankeratin
immunostain.
</pre>


==Malakoplakia==
==Malakoplakia==
Line 619: Line 389:
=Rare stuffs=
=Rare stuffs=
==Juxtaglomerular cell tumour==
==Juxtaglomerular cell tumour==
*[[AKA]] juxtaglomerular tumour, reninoma.<ref name=pmid18192852/>
*[[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>
===General===
{{Main|Juxtaglomerular cell tumour}}
*Rare.
*Etiology: increased renin.
 
Clinical:<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>
*[[Hypertension]].
*Increased aldosterone.
**Causes hypokalemia.
*Increased plasma renin.
 
===Microscopic===
Features:<ref name=pmid21191395/>
*Polygonal cells.
*Abundant granular, eosinophilic cytoplasm.<ref name=pmid436071/>
*Perinuclear halo.
 
DDx:
*[[Chromophobe renal cell carcinoma]], eosinophilic variant.
 
Image:
*[http://www.nature.com/ki/journal/v79/n2/fig_tab/ki2010445f1.html#figure-title Reninoma (nature.com)].<ref name=pmid21191395/>
 
===Stains===
Cytoplasmic granules.<ref name=pmid436071>{{Cite journal  | last1 = Hanna | first1 = W. | last2 = Tepperman | first2 = B. | last3 = Logan | first3 = AG. | last4 = Robinette | first4 = MA. | last5 = Colapinto | first5 = R. | last6 = Phillips | first6 = MJ. | title = Juxtaglomerular cell tumour (reninoma) with paroxysmal hypertension. | journal = Can Med Assoc J | volume = 120 | issue = 8 | pages = 957-9 | month = Apr | year = 1979 | doi =  | PMID = 436071 PMC = 1819229 | URL = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1819229/?tool=pubmed }}</ref>
*PAS +ve.
*PASD +ve.
 
===IHC===
Features:<ref name=pmid21191395>{{Cite journal  | last1 = Chao | first1 = CT. | last2 = Chang | first2 = FC. | last3 = Wu | first3 = VC. | last4 = Chen | first4 = JC. | title = Reninoma. | journal = Kidney Int | volume = 79 | issue = 2 | pages = 260 | month = Jan | year = 2011 | doi = 10.1038/ki.2010.445 | PMID = 21191395 }}</ref>
*Actin +ve.
*Cytokeratin -ve.
*HMB-45 -ve.
 
===EM===
Features:
*Vesicles - contain renin.<ref name=upmc29>URL: [http://path.upmc.edu/cases/case29/micro.html http://path.upmc.edu/cases/case29/micro.html]. Accessed on: 18 December 2011.</ref>


==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> - one review says 26-41% of individuals at autopsy.<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>
**The commonality is somewhat in dispute.<ref name=pmid18655367>{{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.
**Typically less than 3 mm.<ref name=pmid10689882/>
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====
<gallery>
Image:Renal_medullary_fibroma_-_low_mag.jpg | Renal medullary fibroma - low mag. (WC/Nephron)
Image:Renal_medullary_fibroma_-_intermed_mag.jpg | Renal medullary fibroma - intermed. mag. (WC/Nephron)
Image:Renal_medullary_fibroma_-_very_high_mag.jpg | Renal medullary fibroma - very high mag. (WC/Nephron)
</gallery>
www:
*[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.
**Memory device: me'''t'''anephric adenoma is a '''t'''umour.
 
{{Main|Metanephric adenoma}}
===General===
*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====
<gallery>
Image:Metanephric_adenoma_high_mag.jpg | Metanephric adenoma (WC/Nephron)
Image:Metanephric_adenoma_high_mag_cropped.jpg | Metanephric adenoma - cropped (WC/Nephron)
Image:Metanephric_adenoma_-_intermed_mag.jpg | Metanephric adenoma - PaRCC-like - intermed. mag. (WC/Nephron)
Image:Metanephric_adenoma_-_very_high_mag.jpg | Metanephric adenoma - PaRCC-like - very high mag. (WC/Nephron)
</gallery>
www:
*[http://www.nature.com/modpathol/journal/v16/n10/fig_tab/3880886f1.html Metanephric adenoma (nature.com)].
===IHC===
*WT-1 +ve.<ref name=pmid24083046>{{Cite journal  | last1 = Watanabe | first1 = S. | last2 = Naganuma | first2 = H. | last3 = Shimizu | first3 = M. | last4 = Ota | first4 = S. | last5 = Murata | first5 = S. | last6 = Nihei | first6 = N. | last7 = Matsushima | first7 = J. | last8 = Mikami | first8 = S. | last9 = Kuroda | first9 = N. | title = Adult nephroblastoma with predominant epithelial component: a differential diagnostic candidate of papillary renal cell carcinoma and metanephric adenoma-report of three cases. | journal = Case Rep Pathol | volume = 2013 | issue =  | pages = 675875 | month =  | year = 2013 | doi = 10.1155/2013/675875 | PMID = 24083046 }}</ref>
*CD57 +ve.<ref name=pmid24083046/>
*AMACR -ve.<ref name=pmid24083046/>


==Renal epithelial and stromal tumour==
==Renal epithelial and stromal tumour==
Line 733: 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====
<gallery>
Image:Cystic_nephroma_low_mag.jpg |Cystic nephroma - low mag. (WC/Nephron)
Image:Cystic_nephroma_intermed_mag.jpg |Cystic nephroma - intermed. mag. (WC/Nephron)
</gallery>
===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====
<gallery>
Image:Bubble_Wrap.jpg| Bubble wrap. (WC)
</gallery>
===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====
<gallery>
Image:Cystic_nephroma_low_mag.jpg |Cystic nephroma - low mag. (WC/Nephron)
Image:Cystic_nephroma_intermed_mag.jpg |Cystic nephroma - intermed. mag. (WC/Nephron)
</gallery>
 
===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>
*[[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 renal cell carcinoma]].<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>
 
Images:
*[http://moon.ouhsc.edu/kfung/jty1/OPAQ/PathQuiz-Answer/K1A001-PQ01-Ans.htm RMTSCC (ouhsc.edu)].
 
===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==
Line 832: Line 423:


==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:
*[[Yolk sac tumour]].
*[[Collecting duct carcinoma]].
 
Image:
*[http://www.nature.com/modpathol/journal/v20/n9/fig_tab/3800934f3.html#figure-title RMC (nature.com)].
 
===IHC===
*SMARCB1 (INI1) -ve.<ref name=Ref_WMSP286>{{Ref WMSP|286}}</ref>


==Tubulocystic carcinoma of the kidney==
==Tubulocystic carcinoma of the kidney==
Line 878: Line 430:
==Acquired cystic disease-associated renal cell carcinoma==
==Acquired cystic disease-associated renal cell carcinoma==
{{Main|Acquired cystic disease-associated renal cell carcinoma}}
{{Main|Acquired cystic disease-associated renal cell carcinoma}}
==Kidney metastasis==
*[[AKA]] ''renal metastasis'', ''metastatic kidney disease''.
{{Main|Kidney metastasis}}


=Pediatric=
=Pediatric=
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