Difference between revisions of "Kidney tumours"

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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.


==Renal specimens==
Pediatric kidney tumours are dealt with in the ''[[pediatric kidney tumours]]'' article.
*Renal biopsy - usually of medical disease (see ''[[medical kidney]]'').
*Partial nephrectomy.
*Nephrectomy.
*Nephroureterectomy (includes ureter) - done for [[urothelial cell carcinoma]] (UCC) of the renal pelvis and ureteric UCC.
*Radical nephrectomy (includes the [[adrenal gland]]).


In excisions done for tumours, a comment should be made about kidney distant from the tumour. People with less renal mass, i.e. less kidney, are predisposed to focal segmental glomerulosclerosis (FSGS).<ref>NEED REF.</ref>
=Renal specimens=
*[[Renal biopsy]] - usually for [[renal oncocytoma]] vs. renal cell carcinoma (RCC) ''or'' medical diseases - see ''[[medical kidney]]''.
*[[Partial nephrectomy]].
*[[Nephrectomy]].
*[[Nephroureterectomy]] (includes [[ureter]]) - done for [[urothelial cell carcinoma]] (UCC) of the renal pelvis and ureteric UCC.
*[[Radical nephrectomy]] - includes Gerota's fascia.
**May include the [[adrenal gland]].<ref name=Ref_WMSP|288>{{Ref WMSP|288}}</ref>


==Renal cell carcinoma==
In excisions done for tumours, a comment should be made about kidney distant from the tumour. People with less renal mass, i.e. less kidney, are predisposed to focal segmental glomerulosclerosis (FSGS).
===General===
*Relatively common form of cancer.
*Often abbreviated '''RCC'''.
 
===Origin===
*Proximal renal tubule.
 
===Clinical===
*Classically described as a triad:<ref name=schmid>{{cite journal |author=Schmid HP, Szabo J |title=[Renal cell carcinoma--a current review] |language=German |journal=Praxis (Bern 1994) |volume=86 |issue=20 |pages=837?3 |year=1997 |month=May |pmid=9312811 |doi= |url=}}</ref>
**Hematuria (most common symptom).
**Abdominal mass.
**Flank pain.
*Frequently picked-up on imaging (incidentaloma) ~ 1/3 of cases.
 
===Risk factors===
*Smoking.
*Some chemicals.
*Hemodialysis.
*Male>female (~2:1).
*Hereditary - familial syndromes (see [[Hereditary RCC]]).
 
===Subtypes===
RCC (renal cell carcinoma) comes in different subtypes:<ref>PBoD P.1016.</ref>
*Clear cell carcinoma (70-80% of RCC) -- abbrev. CCRCC,
*Papillary renal cell carcinoma (10-15% of RCC) -- abbrev. PRCC,
*Chromophobe renal carcinoma (5% of RCC) -- abbrev. ChRCC,  
*Collecting duct (Bellini duct) carcinoma (1% of RCC).
 
Notes:
*Subtype is an independent predictor of mortality - but adds very little to multivariate models with staging information.<ref>PMID 19076149</ref>
*CCRCC tends to be worse than ChRCC and PRCC, probably due to higher incidence of mets.<ref>PMID 17886093</ref>
===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>


===IHC - differentiation of types===
==Anatomy==
*clear cell RCC vs. papillary RCC
Layers (superficial to deep):
** CK7 (-ve CCRCC), AMACR (+ve in PRCC)<ref name=pmid15848735>{{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>
#Renal fascia (Gerota's fascia).
*papillary RCC type 1 vs. papillary RCC type 2
#Perinephric fat.
** 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>
#Renal capsule.  
**EMA (MUC1) +ve in PRCC type 1<ref name=pmid14657952/>
#Renal parenchyma (cortex).
*ChRCC vs. oncocytoma (ONC)
** CK7 (ChRCC +ve membrane), CK20, CD15<ref name=pmid15848735/>
** CK7 -- ChRCC 86% +ve vs. ONC 0% +ve<ref name=pmid17683191>{{cite journal |author=Liu L, Qian J, Singh H, Meiers I, Zhou X, Bostwick DG |title=Immunohistochemical analysis of chromophobe renal cell carcinoma, renal oncocytoma, and clear cell carcinoma: an optimal and practical panel for differential diagnosis |journal=Arch. Pathol. Lab. Med. |volume=131 |issue=8 |pages=1290? |year=2007 |month=August |pmid=17683191 |doi= |url=http://journals.allenpress.com/jrnlserv/?request=get-abstract&issn=0003-9985&volume=131&page=1290 }}</ref>
** CD15 -- ChRCC 11% +ve vs. ONC 57% +ve<ref name=pmid15500648>{{cite journal |author=Pan CC, Chen PC, Ho DM |title=The diagnostic utility of MOC31, BerEP4, RCC marker and CD10 in the classification of renal cell carcinoma and renal oncocytoma: an immunohistochemical analysis of 328 cases |journal=Histopathology |volume=45 |issue=5 |pages=452? |year=2004 |month=November |pmid=15500648 |doi=10.1111/j.1365-2559.2004.01962.x |url=}}</ref>
** Hale's colloidal iron +ve in ChRCC, usually neg. in ONC<ref name=pmid18603673>{{cite journal |author=Geramizadeh B, Ravanshad M, Rahsaz M |title=Useful markers for differential diagnosis of oncocytoma, chromophobe renal cell carcinoma and conventional renal cell carcinoma |journal=Indian J Pathol Microbiol |volume=51 |issue=2 |pages=167?1 |year=2008 |pmid=18603673 |doi= |url=http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2008;volume=51;issue=2;spage=167;epage=171;aulast=Geramizadeh }}</ref>
*ChRCC & renal oncocytoma vs. others
** CD117 (ckit) +ve (100% membrane, ~75% cytoplasmic)<ref>{{cite journal |author=Krueger S, Sotlar K, Kausch I, Horny HP |title=Expression of KIT (CD117) in renal cell carcinoma and renal oncocytoma |journal=Oncology |volume=68 |issue=2-3 |pages=269-75 |year=2005 |pmid=16015044 |doi=10.1159/000086783 |url=}}</ref>
*clear cell RCC vs. chromophobe RCC
**Hale's colloidal iron (+ve in ChRCC)<ref name=pmid18603673/>
**CK7 (cell membrane +ve in ChRCC)


Notes:
===Sign out===
*One paper<ref>{{cite journal |author=Martignoni G, Brunelli M, Gobbo S, ''et al'' |title=Role of molecular markers in diagnosis and prognosis of renal cell carcinoma |journal=Anal. Quant. Cytol. Histol. |volume=29 |issue=1 |pages=41? |year=2007 |month=February |pmid=17375873 |doi= |url=}}</ref> describes CD10, parvalbumin, AMACR, CK7 and S100A1 as being useful.
====Missed renal biopsy====
*Another paper I came across:<ref>{{cite journal |author=Avery AK, Beckstead J, Renshaw AA, Corless CL |title=Use of antibodies to RCC and CD10 in the differential diagnosis of renal neoplasms |journal=Am. J. Surg. Pathol. |volume=24 |issue=2 |pages=203?0 |year=2000 |month=February |pmid=10680888 |doi= |url=http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0147-5185&volume=24&issue=2&spage=203}}</ref>
{{Main|Missed renal biopsy}}
*ckit (CD117) not useful for differentiating ONC and ChRCC.


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


Selected common tumours of the kidney:<ref>GUP PP.281-304.</ref><ref name=pmid19494850>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"
| ||'''Clear cell RCC''' ||'''Papillary RCC<br>type 1''' ||'''Papillary RCC<br>type 2''' ||'''Chromophobe RCC<br>classic variant''' || '''Chromophobe RCC<br>eosinophilic variant''' || '''Oncocytoma'''
|
| '''Clear cell RCC'''
| '''Papillary RCC<br>type 1'''
| '''Papillary RCC<br>type 2'''  
| '''Chromophobe RCC<br>classic variant'''
| '''Chromophobe RCC<br>eosinophilic variant'''
| '''Oncocytoma'''
|-
|-
|Gross ||Golden yellow, solid || friable || friable ||light brown ||light brown ||mahogany/brown, +/-central scar
|Gross
| Golden yellow, solid
| friable
| friable
| light brown
| light brown
| mahogany/brown, +/-central scar
|-
|-
|Architecture ||nests, sheets || papillary, simple || papillary,<br>pseudostratified ||nests, sheets ||nests, sheets ||nests, sheets
|Architecture
| nests, sheets
| papillary, simple
| papillary,<br>pseudostratified
| nests, sheets
| nests, sheets
| nests, sheets
|-
|-
|Nuclear atypia ||low-high<br>typically medium-high ||low-medium || medium-high ||low-high ||low-high ||low-medium, round nuclei
|Nuclear atypia
| low-high<br>typically medium-high  
| low-medium
| medium-high  
| low-high, "raisinoid" <br>nuc. membrane
| low-high, "raisinoid" <br>nuc. membrane
| low-medium, round nuclei
|-
|-
|Cytoplasm ||clear ||eosinophilic ||eosinophilic ||cobwebs/clear ||eosinophilic/cobwebs ||eosinophilic/<br>granular & abundant
|Cytoplasm
| clear
| eosinophilic
| eosinophilic
| cobwebs/clear
| eosinophilic/cobwebs
| eosinophilic/<br>granular & abundant
|-
|-
|Other ||delicate vessels,<br>necrosis common ||histiocytes<br> in fibrovascular cores ||histiocytes<br> in fibrovascular cores ||perinuclear clearing, thick vessels ||perinuclear clearing, thick vessels ||in loose fibrous/hyaline stroma
|Other
| delicate vessels,<br>[[necrosis]] common
| histiocytes<br> in fibrovascular cores, >0.5 cm
| histiocytes<br> in fibrovascular cores, >0.5 cm
| perinuclear clearing, thick vessels
| perinuclear clearing, thick vessels
| in loose fibrous/hyaline stroma
|-
|-
|IHC ||CK7-, EMA+ ||AMACR+, EMA+, CK7+ ||AMACR+, E-cadherin+, CK7- ||CD117+, CK7+ (membrane) ||CD117+, CK7+ (membrane) ||Vimentin-, EMA+
|IHC
| [[CK7]]-, EMA+
| AMACR+, EMA+, CK7+
| AMACR+, E-cadherin+, CK7-
| CD117+, CK7+ (membrane)
| CD117+, CK7+ (membrane)
| Vimentin-, EMA+
|-
|-
|Main DDx ||chromophobe<br>classic variant ||PaRCC type 2, mets ||PaRCC type 1, mets||clear cell RCC ||oncocytoma ||chromophobe<br>eosinophilic variant
|Main DDx
| chromophobe<br>classic variant
| PaRCC type 2, mets  
| PaRCC type 1, mets
| clear cell RCC
| oncocytoma
| chromophobe<br>eosinophilic variant
|-
|-
|Key features ||clear cells, vascular || papillae, histiocytes<br>simple epithelium ||papillae, histiocytes,<br>stratified ||perinuc. clearing<br>wispy cytoplasm ||wispy eosinophilic<br>cytoplasm ||eosinophilic, granular cytoplasm
|Key features
| clear cells, vascular  
| papillae, histiocytes<br>simple epithelium  
| papillae, histiocytes,<br>stratified
| perinuc. clearing,<br>wispy cytoplasm
| perinuc. clearing,<br>wispy eosinophilic<br>cytoplasm
| eosinophilic, granular cytoplasm
|-
|Image(s)
| [[Image:Renal_clear_cell_ca_%281%29_Nephrectomy.jpg|thumb|150px| CCRCC (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)]]
|
|
| [[Image:Oncocytic_chromophobe_rcc_high_mag.jpg|thumb|150px| ChRCC (WC)]]
| [[Image:Renal_oncocytoma2.jpg|thumb|150px| Oncocytoma (WC)]]
|}
|}
Notes:
Notes:
*Cell shape: all have epithelioid morphology.
*Cell shape: all have epithelioid morphology.


==Hereditary RCC==
===Tabular comparison of oncocytoma and chromophobe RCC===
# [[Von Hippel-Lindau syndrome]] (clear cell tumours),  
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>
# hereditary clear cell carcinoma (VHL gene),
{| class="wikitable sortable"
# hereditary papillary carcinoma (MET proto-oncogene mutation)
! Morphologic feature
! [[ChRCC]] (eosinophilic<br> variant)
! [[Renal oncocytoma]]
|-
| Nuclear morphology
| "raisinoid"/wrinkled appearance
| round with small nucleolus, <br>usu. little size variation
|-
| Multinucleation
| common - binucleation
| uncommon
|-
| Chromatin
| coarse
| fine
|-
| Architecture
| solid, crowded nests
| spaced nests / <br>archipelago-like, solid
|-
| Cytoplasm
| perinuclear halo, may be focal
| no perinuclear halo
|-
| Degenerative foci<br>(focal atypia & pleomorphism)
| absent
| present in ~20% of cases
|-
| Image
| [[Image:Oncocytic_chromophobe_rcc_high_mag.jpg |thumb|150px|ChRCC (WC/Nephron)]]
| [[Image:Renal_oncocytoma2.jpg |thumb|150px|Oncocytoma (WC/Nephron)]]
|}


Note: ''all'' of the above are autosomal dominant<ref>PBoD P.1016.</ref>
===Common DDx===
====Spindle 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]].


==Grading==
Benign spindle cell tumours of the kidney:
Grading based on '''Fuhrman system''' which considers:<ref>GUP P.282.</ref>
*[[Angiomyolipoma]].
*Nuclear pleomorphism (size, shape),
*[[Cystic nephroma]].
*Chromatin pattern,
*Nucleoli prominence.


===Criteria & grades===
====Renal tumours with eosinophilic cytoplasm====
*Grade 1: no nucleoli, near 'normal' appearance.
{{Main|Renal tumours with eosinophilic cytoplasm}}
*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===
=WHO classification of renal neoplasia=
*1 vs. 2: 2 has granular chromatin.
*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].
*2 vs. 3: 3 has nucleoli @ 10x objective.
===Renal cell tumours===
*3 vs. 4: 4 has pleomorphism/hyperchromasia.
Common:
*[[Clear cell renal cell carcinoma]].
*[[Papillary renal cell carcinoma]].
*[[Renal papillary adenoma|Papillary adenoma]].
*[[Chromophobe renal cell carcinoma]].
*[[Renal oncocytoma|Oncocytoma]].


==Clear cell carcinoma==
Less common:
===Gross===
*[[Multilocular clear cell renal cell carcinoma]].
*Gold/yellow.
*[[Carcinoma of the collecting ducts of Bellini]].
*+/-Haemorrhage (common).
*[[Renal cell carcinoma, unclassified]].
*+/-Necrosis (common in large tumours).
*[[Renal medullary carcinoma]].
*[[Renal tumour with Xp11.2 translocation|Xp11 translocation carcinoma]].
*[[Mucinous tubular and spindle cell carcinoma]].
*Carcinoma associated with neuroblastoma.


===Histo.<ref>PBoD P.1017-8</ref>===
===Metanephric tumours===
*Clear cells,
*[[Metanephric adenoma]].
*Solid or trabecular pattern,
*[[Metanephric stromal tumour]].
*Delicate branching vasculature.
*[[Metanephric adenofibroma]].


*Hyaline bodies common<ref>AFIP Renal Tumours Book.</ref>
===Nephroblastic tumours===
**Not common in papillary RCC.
*Nephrogenic rests.
*[[Nephroblastoma]].


===IHC===
===Mesenchymal tumours===
*CK7-
Childhood:
*CK20-
*[[Clear cell sarcoma of the kidney]].
*Hale's colloidal iron-
*[[Rhabdoid tumour]].
**+ve in chromophobe
*[[Mesoblastic nephroma|Congenital mesoblastic nephroma]].


Note: ''[[Hale's colloidal iron]]'' does not stain iron... it stains hemosiderin.<ref>Latta Jan 27, '09</ref>
Adults:  
*Unique to kidney:
**[[Juxtaglomerular cell tumour]].
**[[Renomedullary interstitial cell tumour]].
Other:
*[[Angiomyolipoma]].
*[[Epithelioid angiomyolipoma]].
*[[Leiomyosarcoma]].
*[[Angiosarcoma]].
*[[Pleomorphic undifferentiated sarcoma]].
*[[Hemangiopericytoma]].
*[[Solitary fibrous tumour]].
*[[Osteosarcoma]].
*[[Schwannoma]].


Clear cell vs. chromophobe
===Mixed mesenchymal and epithelial tumours===
*chromophobe: "translucent" (NOT quite clear), reticulated, +Hale's colloidal iron stain, CK7+ (cell membrane).
*[[Cystic nephroma]].
*[[Mixed epithelial and stromal tumour]].
*[[Synovial sarcoma]].


Clear cell vs. [[adrenocortical carcinoma]]
===Others===
*ACC: EMA- (epithelial membrane antigen), cytokeratin mostly neg., inhibin+ (neg. in RCC).<ref>GUP P.285.</ref>
*[[Neuroendocrine tumours]].
*Hematologic tumours.
*[[Germ cell tumour]]s.
*[[Metastases]].


==Papillary RCC==
==Vancouver modification of WHO classification==
===General===
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>
*Often subclassified<ref>GUP P.289.</ref> into ''type 1'' and ''type 2'' -- see below.
*[[Tubulocystic renal cell carcinoma]].
*May be abbreviated ''PRCC''.
*[[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]].


===Epidemiology===
"Emerging" entities (as per Vancouver) are:
*Associated with ''dialysis associated cystic disease''.<ref>PBoD PP.1017-8</ref>
*[[Thyroid-like follicular renal cell carcinoma]].
*[[Succinate dehydrogenase-deficient renal cell carcinoma]].
*[[ALK translocation renal cell carcinoma]].


===Microsopy===
==An entity proposed after Vancouver==
Histo.<ref>PBoD P.1017-8.</ref>
*[[Eosinophilic, solid and cystic renal cell carcinoma]].
*Cuboidal or low columnar cell in papillae.
*[[Biphasic hyalinizing psammomatous renal cell carcinoma]].
*Interstitial foam cells in vascular cores - '''key feature'''.<ref>ALS Feb 9, 2009.</ref>
*[[Papillary renal neoplasm with reverse polarity]].
**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>
*[[Low-grade oncocytic renal tumour]].
*Highly vascular.
Mnemonic ''HIP'': '''h'''ighly vascular, '''i'''nterstitial foam cells, '''p'''apillae.


Histological subtyping:<ref>GUP P.289.</ref>
=Renal cell carcinoma=
*''Type 1'' - single layer of cells on basement membrane.
==Overview==
** usually low grade nuclear features, i.e. low Fuhrman grade.
===General===
*''Type 2'' - pseudostratification of cells.
*Relatively common form of cancer.
** Usually high grade nuclear features, i.e. high Fuhrman grade.
*Often abbreviated '''RCC'''.
*[[AKA]] ''hypernephroma''.<ref>URL:[http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001544/ http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001544/]. Accessed on: 14 July 2011.</ref>
*RCC represents approx. 90% of malignancies in kidneys of adults.<ref name=Ref_WMSP289>{{Ref WMSP|289}}</ref>
===Origin===
*Proximal renal tubule.


IHC:
===Clinical===
*Commonly AMACR+.<ref>ALS Feb 9, 2009.</ref>
*Classically described as a triad:<ref name=schmid>{{cite journal |author=Schmid HP, Szabo J |title=[Renal cell carcinoma--a current review] |language=German |journal=Praxis (Bern 1994) |volume=86 |issue=20 |pages=837?3 |year=1997 |month=May |pmid=9312811 |doi= |url=}}</ref>
**Hematuria (most common symptom).
**Abdominal mass.
**Flank pain.
*Frequently picked-up on imaging (incidentaloma) ~ 1/3 of cases.


More reading:
===Risk factors===
*[http://www.e-immunohistochemistry.info/web/Papillary_renal_cell_carcinoma.htm e-immunohistochemistry.info]
*[[Smoking]] - most important.<ref name=Ref_WMSP289>{{Ref WMSP|289}}</ref>
*Chemical exposures (arsenic, asbestos, cadmium, organic solvents, pesticides, fungal toxins).<ref name=Ref_WMSP289>{{Ref WMSP|289}}</ref>
*Chronic renal failure.
**[[Acquired cystic renal disease]].
*Male>female (~2:1).
*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>


===Clear cell vs. papillary===
===Subtypes of RCC===
*Papillary: +histiocytes, +intracellular hemosiderin, [[CK7]]+.
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.
*Papillary renal cell carcinoma (10-15% of RCC) -- abbrev. PRCC.
*Chromophobe renal carcinoma (5% of RCC) -- abbrev. ChRCC.
*Collecting duct (Bellini duct) carcinoma (1% of RCC).


==Chromophobe RCC==
Notes:
===General===
*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>
*Sometimes abbreviated ''ChRCC''.
*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.


There are two subtypes:<ref>GUP P.293.</ref>
===IHC - is it RCC?===
*Classic (type I???)
*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>
*Eosinophilic variant (type II???)


===Gross===
===IHC - differentiation of types===
*Tan, light-brown.
*Clear cell RCC vs. papillary RCC:
*Solitary.
** CK7 (-ve CCRCC), AMACR (+ve in PRCC).<ref name=pmid15848735>{{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>
*Well-circumscribed.
*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>
**[[EMA]] (MUC1) +ve in PRCC type 1.<ref name=pmid14657952/>


===Microscopy===
*ChRCC vs. oncocytoma (ONC):
Classic Histo.<ref>PBoD P.1016-7</ref><ref>GUP P.293.</ref>, 3 P's [[mnemonic]]:
** CK7 (ChRCC +ve membrane), CK20, CD15.<ref name=pmid15848735/>
*Pale cytoplasm, with wisps of eosinophilic material; the cells are not completely clear, they have "cobwebs".
** CK7 -- ChRCC 86% +ve vs. ONC 0% +ve.<ref name=pmid17683191>{{cite journal |author=Liu L, Qian J, Singh H, Meiers I, Zhou X, Bostwick DG |title=Immunohistochemical analysis of chromophobe renal cell carcinoma, renal oncocytoma, and clear cell carcinoma: an optimal and practical panel for differential diagnosis |journal=Arch. Pathol. Lab. Med. |volume=131 |issue=8 |pages=1290? |year=2007 |month=August |pmid=17683191 |doi= |url=http://journals.allenpress.com/jrnlserv/?request=get-abstract&issn=0003-9985&volume=131&page=1290 }}</ref>
*Perinuclear clearing, i.e. a pale halo surrounds the nucleus - '''key feature'''.
** CD15 -- ChRCC 11% +ve vs. ONC 57% +ve.<ref name=pmid15500648>{{cite journal |author=Pan CC, Chen PC, Ho DM |title=The diagnostic utility of MOC31, BerEP4, RCC marker and CD10 in the classification of renal cell carcinoma and renal oncocytoma: an immunohistochemical analysis of 328 cases |journal=Histopathology |volume=45 |issue=5 |pages=452? |year=2004 |month=November |pmid=15500648 |doi=10.1111/j.1365-2559.2004.01962.x |url=}}</ref>
*Periphery of cell distinct, i.e. cell membrane is easy to discern.
** Hale's colloidal iron +ve in ChRCC, usually neg. in ONC.<ref name=pmid18603673>{{cite journal |author=Geramizadeh B, Ravanshad M, Rahsaz M |title=Useful markers for differential diagnosis of oncocytoma, chromophobe renal cell carcinoma and conventional renal cell carcinoma |journal=Indian J Pathol Microbiol |volume=51 |issue=2 |pages=167?1 |year=2008 |pmid=18603673 |doi= |url=http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2008;volume=51;issue=2;spage=167;epage=171;aulast=Geramizadeh }}</ref>
** PAX2 -- ChRCC (1/11) +ve vs. ONC (20/23) +ve.<ref name=pmid17210525>{{cite journal |author=Memeo L, Jhang J, Assaad AM, ''et al.'' |title=Immunohistochemical analysis for cytokeratin 7, KIT, and PAX2: value in the differential diagnosis of chromophobe cell carcinoma |journal=Am. J. Clin. Pathol. |volume=127 |issue=2 |pages=225–9 |year=2007 |month=February |pmid=17210525 |doi=10.1309/9KWEA4W9Y94D1AEE |url=http://ajcp.ascpjournals.org/cgi/pmidlookup?view=long&pmid=17210525}}</ref>
**Kidney-specific cadherin (Ksp-cadherin) -- ChRCC 97% +ve (distinctive membrane pattern) vs. ONC only 3% +ve.<ref name=pmid15712178>{{cite journal |author=Mazal PR, Exner M, Haitel A, ''et al.'' |title=Expression of kidney-specific cadherin distinguishes chromophobe renal cell carcinoma from renal oncocytoma |journal=Hum. Pathol. |volume=36 |issue=1 |pages=22–8 |year=2005 |month=January |pmid=15712178 |doi=10.1016/j.humpath.2004.09.011 |url=}}</ref>


Eosinophilic variant:<ref>GUP P.293.</ref>
*ChRCC & renal oncocytoma vs. others:
*Eosinophilic (finely granular) cytoplasm.
** CD117 (ckit) +ve (100% membrane, ~75% cytoplasmic).<ref>{{cite journal |author=Krueger S, Sotlar K, Kausch I, Horny HP |title=Expression of KIT (CD117) in renal cell carcinoma and renal oncocytoma |journal=Oncology |volume=68 |issue=2-3 |pages=269-75 |year=2005 |pmid=16015044 |doi=10.1159/000086783 |url=}}</ref>
*Perinuclear clearing - '''key feature'''.
*Clear cell RCC vs. chromophobe RCC:
*Periphery of cell distinct.
**Hale's colloidal iron (+ve in ChRCC).<ref name=pmid18603673/>
*Smaller cells than classic subtype.
**CK7 (cell membrane +ve in ChRCC).


Image: [http://commons.wikimedia.org/wiki/File:Oncocytic_chromophobe_rcc_high_mag.jpg Oncocytic chromophobe RCC (wikimedia.org)].
Notes:  
===IHC===
*One paper<ref>{{cite journal |author=Martignoni G, Brunelli M, Gobbo S, ''et al'' |title=Role of molecular markers in diagnosis and prognosis of renal cell carcinoma |journal=Anal. Quant. Cytol. Histol. |volume=29 |issue=1 |pages=41? |year=2007 |month=February |pmid=17375873 |doi= |url=}}</ref> describes CD10, parvalbumin, AMACR, CK7 and S100A1 as being useful.
*CK7+ cell membrane<ref>GUP P.293.</ref>
*Another paper I came across:<ref>{{cite journal |author=Avery AK, Beckstead J, Renshaw AA, Corless CL |title=Use of antibodies to RCC and CD10 in the differential diagnosis of renal neoplasms |journal=Am. J. Surg. Pathol. |volume=24 |issue=2 |pages=203?0 |year=2000 |month=February |pmid=10680888 |doi= |url=http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0147-5185&volume=24&issue=2&spage=203}}</ref>
*c-kit (CD117) not useful for differentiating ONC and ChRCC.<ref name=pmid17210525>{{cite journal |author=Memeo L, Jhang J, Assaad AM, ''et al.'' |title=Immunohistochemical analysis for cytokeratin 7, KIT, and PAX2: value in the differential diagnosis of chromophobe cell carcinoma |journal=Am. J. Clin. Pathol. |volume=127 |issue=2 |pages=225–9 |year=2007 |month=February |pmid=17210525 |doi=10.1309/9KWEA4W9Y94D1AEE |url=http://ajcp.ascpjournals.org/cgi/pmidlookup?view=long&pmid=17210525}}</ref>
*E-cadherin ''not'' useful for differentiating ChRCC and ONC.<ref name=pmid12613443>{{cite journal |author=Kim MK, Kim S |title=Immunohistochemical profile of common epithelial neoplasms arising in the kidney |journal=Appl. Immunohistochem. Mol. Morphol. |volume=10 |issue=4 |pages=332–8 |year=2002 |month=December |pmid=12613443 |doi= |url=}}</ref>


DDx:
===RCC vs. Urothelial cell carcinoma===
*May appear similar to oncotyoma -- particularily the eosinophilic variant.
{{main|Urothelium}}
**IHC: CK7: chromophobe = cell membrane CK7+; oncoctyoma = patchy cytoplasmic +ve
*Clinically/radiologically, it may not be possible to differentiate renal pelvis UCC and RCC if the tumour is large.
*Classic ChRCC may be challenging to differentiate from clear cell RCC.
*Pathologically, this is not very difficult. 
**Perinuclear clearing is ''not'' seen in clear cell RCC.
*On gross specimens, it is almost always obvious what one is dealing with:
**ChRCC has wisps in the cytoplasm
**[[UCC]] = ''[[nephroureterectomy]]''.
**[[RCC]] = ''[[partial nephrectomy]]'', ''nephrectomy'' or ''[[radical nephrectomy]]''.


==Collecting duct carcinoma==
===Renal cell carcinoma with sarcomatoid differentiation===
===Epidemiology===
*[[AKA]] ''sarcomatoid renal cell carcinoma''.
*Rare.
{{Main|Renal cell carcinoma with sarcomatoid differentiation}}


===Microscopy===
===Renal cell carcinoma with rhabdoid morphology===
Features:<ref>GUP P.295.</ref>
*[[AKA]] ''renal cell carcinoma with rhabdoid change''.
*Tubular structures with tapered ends.
{{Main|Renal cell carcinoma with rhabdoid morphology}}
*High grade nuclear features (nuclear pleomorphism).
*High mitotic rate.
*[[Hobnail pattern]] - cell width smaller at basement membrane than free surface ??? <ref>PBoD P.1018.</ref>


==Hereditary renal cell carcinoma==
{{Main|Hereditary renal cell carcinoma}}


==Urothelial cell carcinoma==
==Renal cell carcinoma grading==
*Clinically/radiologically, it may not be possible to differentiate renal pelvis UCC and RCC if the tumour is large.
{{Main|Renal cell carcinoma grading}}
*Pathologically, this is not very difficult. 
*On gross specimens, it is almost always obvious what one is dealing with:
**UCC = ''nephroureterectomy''.
**RCC = ''nephrectomy'' or ''radical nephrectomy''.


=Benign & mimics=
==Renal cell carcinoma staging==
{{Main|Kidney cancer staging}}


==[[Oncocytoma]]==
===Renal sinus invasion===
===General===
{{Main|Kidney cancer staging}}
*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===
==Clear cell renal cell carcinoma==
*Brown, mahogany brown.
{{Main|Clear cell renal cell carcinoma}}
*1/3 have a characteristic central scar.<ref>GUP P.302.</ref>


*Images:
==Multilocular cystic renal cell carcinoma==
**[http://en.wikipedia.org/wiki/File:Renal_oncocytoma.jpg Renal oncocytoma with central scar (wikipedia.org)].
{{Main|Multilocular cystic renal cell carcinoma}}


===Histology===
==Papillary renal cell carcinoma==
*Eosinophilic cytoplasm '''key feature'''.
{{Main|Papillary renal cell carcinoma}}
*Cells arranged in nests.
*Slightly enlarged nuclei, but no significant pleomorphism (size variation).
*Nuclei uniform and round.<ref>GUP P.302.</ref>


Notes:
==Chromophobe renal cell carcinoma==
*May look like eosinophilic variant of chromophobe RCC -- this is the main DDx.
{{Main|Chromophobe renal cell carcinoma}}
**May need IHC to differentiate (CK7: oncocytoma = cytoplasm +ve, chromophobe = cell membrane +ve).


Images:
==Clear cell papillary renal cell tumour==
**[http://commons.wikimedia.org/wiki/File:Renal_oncocytoma2.jpg High mag. oncocytoma (mediawiki.org)].
In the past, it was known as ''clear cell papillary renal cell carcinoma''.
**[http://commons.wikimedia.org/wiki/File:Renal_oncocytoma3.jpg Intermed. mag. oncocytoma (mediawiki.org)].
{{Main|Clear cell papillary renal cell tumour}}
**[http://commons.wikimedia.org/wiki/File:Renal_oncocytoma4.jpg Low mag. oncocytoma (mediawiki.org)].


==Angiomyolipoma==
==Unclassified renal cell carcinoma==
===General===
*Abbreviated ''URCC''.
*Mostly benign mesenchymal tumour.
{{Main|Unclassified renal cell carcinoma}}
*Presentations: flank pain, hematuria, incidentaloma.<ref name=pmid18805573>PMID 18805573</ref>
*AMLs occur may be elsewhere in the body, e.g. liver,<ref name=pmid15498214>PMID 15498214</ref> but are most common in the kidney.


===Epidemiology===
=Renal translocation carcinomas=
*May be assoc. with tuberous sclerosis -- 70% have an AML.
==Renal tumour with Xp11.2 translocation==
**When compared to sporadic cases:
{{Main|Renal tumour with Xp11.2 translocation}}
***More often bilateral.
***Usually bigger.


===Microscopy===
==Renal tumour with t(6;11) translocation==
*Muscle.
*[[AKA]] ''t(6;11) renal cell carcinoma''.
*Adipose tissue - not always present.<ref name=pmid15584043>PMID 15584043</ref>
{{main|Renal tumour with t(6;11) translocation}}
*Blood vessels.


====Cytologic features<ref>PMID 15584043</ref>====
=Benign tumours=
*Nuclei - round/ovoid.
==Papillary adenoma of the kidney==
*Chromatin - bland.
*[[AKA]] ''renal papillary adenoma''.
{{Main|Papillary adenoma of the kidney}}


===IHC===
==Renal oncocytoma==
*Melanocytic markers +ve<ref>GUP P.324.</ref> (e.g. HMB-45 +ve).
{{Main|Renal oncocytoma}}
*Epithelial markers -ve.<ref>GUP P.324.</ref>
*SMA +ve.
*CD117 +/-.
Additional paper: <ref name=pmid15584043>PMID 15584043</ref>


Notes:
==Angiomyolipoma==
*There is a suggestion that an ''epithelioid'' variant is more worisome,<ref>PMID 12352384</ref> but this is not the case in all studies.<ref>PMID 18852677</ref>
*Abbreviated ''AML''.
Ki-67 can be used to help distinguish btw 'em -- epithelioid variant Ki-67 +ve.<ref>PMID 18839327</ref>
{{Main|Angiomyolipoma}}


=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/>


===Microscopy===
==Malakoplakia==
*Abundant macrophages.
{{main|Malakoplakia}}
*+/-Giant cells.


Image:
=Rare stuffs=
*[http://commons.wikimedia.org/wiki/File:Xanthogranulomatous_pyelonephritis_cd68.jpg Xanthogranulomatous_pyelonephritis - CD68 IHC (mediawiki.org)].
==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}}


===IHC===
==Renomedullary interstitial cell tumour==
*CD68+
*[[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>
*RCC markers (CD10, RCC) all negative
{{Main|Renomedullary interstitial cell tumour}}


DDx:
==Metanephric adenoma==
*[[Malacoplakia]].  
*Should '''not''' be confused ''[[mesonephric adenoma]]'', another term for ''nephrogenic adenoma''.
**Basophilic inclusions -- inside or outside of macrophages - often size of RBC or larger ([[Michaelis-Gutmann bodies]]).
**Memory device: me'''t'''anephric adenoma is a '''t'''umour.
*RCC.
{{Main|Metanephric adenoma}}
*Granulomatous disease.


==Malacoplakia==
==Renal epithelial and stromal tumour==
{{main|Malacoplakia}}
: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>
#[[Mixed epithelial and stromal tumour]].
#[[Cystic nephroma]].


==Medullary fibroma==
==Mixed epithelial and stromal tumour==
===General===
*Abbreviated ''MEST''.
*Rare.
{{Main|Mixed epithelial and stromal tumour of the kidney}}


===Epidemiology===
==Cystic nephroma==
*Benign.
{{Main|Cystic nephroma}}


===Gross===
==Renal mucinous tubular and spindle cell carcinoma==
*Small, white well circumscribed nodule in medulla.
*[[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>
{{Main|Renal mucinous tubular and spindle cell carcinoma}}


===Microscopy===
==Collecting duct carcinoma==
*Spindle cells.
{{Main|Collecting duct carcinoma}}


=Rare stuffs=
==Renal medullary carcinoma==
==Metanephric adenoma==
{{Main|Renal medullary carcinoma}}
*Benign.


===Micro===
==Tubulocystic carcinoma of the kidney==
*Nests of cells.
{{Main|Tubulocystic carcinoma of the kidney}}


==Cystic nephroma==
==Acquired cystic disease-associated renal cell carcinoma==
*AKA ''renal epithelial stromal tumour (REST)'' and ''mixed epithelial stromal tumour (MEST)''.
{{Main|Acquired cystic disease-associated renal cell carcinoma}}
*Rare - approx. 1.5% of renal neoplasms.<ref name=pmid17454754>PMID 17454754</ref>
*Benign.
*Prevalence: males > females.


===Micro===
==Kidney metastasis==
*Cysts lined by simple epithelium with hobnailing.
*[[AKA]] ''renal metastasis'', ''metastatic kidney disease''.
*Stroma has an ovarian look:
{{Main|Kidney metastasis}}
**''Blue'' (basophilic).
**''Spindle cells''.


Image: [http://commons.wikimedia.org/wiki/File:Cystic_nephroma_low_mag.jpg Cystic nephroma (wikimedia.org)].
=Pediatric=
===IHC===
{{Main|Pediatric kidney tumours}}
Features:<ref name=pmid17454754>PMID 17454754</ref>
*ER+.
*PR+.
*CD10+.


===DDx===
The most common is [[nephroblastoma]] (Wilms tumour).
*Tubulocystic carcinoma.


==Mucinous spindle cell carcinoma==
Others include:
*Rare.<ref name=pmid19494850>PMID 19494850</ref>
*[[Metanephric stromal tumour]].
*[[Metanephric adenofibroma]]
*[[Metanephric adenoma]].
*[[Clear cell sarcoma of the kidney]].


===Microscopy===
=See also=
*Mucin - may be scant.
*[[Urinary bladder]].
*Spindle cells.
*[[Medical kidney]].
*[[Malakoplakia]] - yellow lesion on gross; may mimic RCC.


==See also==
=References=
*[[Urinary bladder]]
{{reflist|2}}
*[[Medical kidney]]


==References==
=External links=
{{reflist|2}}
*[http://www.uscap.org/site~/99th/pdf/companion13h03.pdf Renal tumours with eosinophilic cytoplasm (uscap.org)].


[[Category: Genitourinary pathology]]
[[Category: Genitourinary pathology]]

Latest revision as of 21:41, 15 August 2024

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.

Renal specimens

In excisions done for tumours, a comment should be made about kidney distant from the tumour. People with less renal mass, i.e. less kidney, are predisposed to focal segmental glomerulosclerosis (FSGS).

Anatomy

Layers (superficial to deep):

  1. Renal fascia (Gerota's fascia).
  2. Perinephric fat.
  3. Renal capsule.
  4. Renal parenchyma (cortex).

Sign out

Missed renal biopsy

Main article: Missed renal biopsy

Tabular comparison (selected tumours)

Selected common tumours of the kidney:[2][3]

Clear cell RCC Papillary RCC
type 1 		Papillary RCC
type 2 		Chromophobe RCC
classic variant 		Chromophobe RCC
eosinophilic variant 		Oncocytoma
Gross Golden yellow, solid friable friable light brown light brown mahogany/brown, +/-central scar
Architecture nests, sheets papillary, simple papillary,
pseudostratified 		nests, sheets nests, sheets nests, sheets
Nuclear atypia low-high
typically medium-high 		low-medium medium-high low-high, "raisinoid"
nuc. membrane 		low-high, "raisinoid"
nuc. membrane 		low-medium, round nuclei
Cytoplasm clear eosinophilic eosinophilic cobwebs/clear eosinophilic/cobwebs eosinophilic/
granular & abundant
Other delicate vessels,
necrosis common 		histiocytes
in fibrovascular cores, >0.5 cm 		histiocytes
in fibrovascular cores, >0.5 cm 		perinuclear clearing, thick vessels perinuclear clearing, thick vessels in loose fibrous/hyaline stroma
IHC CK7-, EMA+ AMACR+, EMA+, CK7+ AMACR+, E-cadherin+, CK7- CD117+, CK7+ (membrane) CD117+, CK7+ (membrane) Vimentin-, EMA+
Main DDx chromophobe
classic variant 		PaRCC type 2, mets PaRCC type 1, mets clear cell RCC oncocytoma chromophobe
eosinophilic variant
Key features clear cells, vascular papillae, histiocytes
simple epithelium 		papillae, histiocytes,
stratified 		perinuc. clearing,
wispy cytoplasm 		perinuc. clearing,
wispy eosinophilic
cytoplasm 		eosinophilic, granular cytoplasm
Image(s)
CCRCC (WC)
PaRCC - intermed. (WC)
,
PaRCC - high (WC)
ChRCC (WC)
Oncocytoma (WC)

Notes:

  • Cell shape: all have epithelioid morphology.

Tabular comparison of oncocytoma and chromophobe RCC

Histomorphologic features useful to distinguish chromophobe RCC (eosinophilic variant) and oncocytoma:[4]

Morphologic feature ChRCC (eosinophilic
variant) 		Renal oncocytoma
Nuclear morphology "raisinoid"/wrinkled appearance round with small nucleolus,
usu. little size variation
Multinucleation common - binucleation uncommon
Chromatin coarse fine
Architecture solid, crowded nests spaced nests /
archipelago-like, solid
Cytoplasm perinuclear halo, may be focal no perinuclear halo
Degenerative foci
(focal atypia & pleomorphism) 		absent present in ~20% of cases
Image
ChRCC (WC/Nephron)
Oncocytoma (WC/Nephron)

Common DDx

Spindle cell tumours

Malignant spindle cell tumours of the kidney:

Benign spindle cell tumours of the kidney:

Renal tumours with eosinophilic cytoplasm

Main article: Renal tumours with eosinophilic cytoplasm

WHO classification of renal neoplasia

  • Based on 2004 iteration - as per WMSP, slightly modified.[5] Online, the classification can found here.

Renal cell tumours

Common:

Less common:

Metanephric tumours

Nephroblastic tumours

Mesenchymal tumours

Childhood:

Adults:

Other:

Mixed mesenchymal and epithelial tumours

Others

Vancouver modification of WHO classification

In 2012/2013, several additions were made:[6]

"Emerging" entities (as per Vancouver) are:

An entity proposed after Vancouver

Renal cell carcinoma

Overview

General

  • Relatively common form of cancer.
  • Often abbreviated RCC.
  • AKA hypernephroma.[7]
  • RCC represents approx. 90% of malignancies in kidneys of adults.[8]

Origin

  • Proximal renal tubule.

Clinical

  • Classically described as a triad:[9]
    • Hematuria (most common symptom).
    • Abdominal mass.
    • Flank pain.
  • Frequently picked-up on imaging (incidentaloma) ~ 1/3 of cases.

Risk factors

Subtypes of RCC

RCC (renal cell carcinoma) comes in different subtypes:[11]

  • Clear cell carcinoma (70-80% of RCC) -- abbrev. CCRCC.
  • Papillary renal cell carcinoma (10-15% of RCC) -- abbrev. PRCC.
  • Chromophobe renal carcinoma (5% of RCC) -- abbrev. ChRCC.
  • Collecting duct (Bellini duct) carcinoma (1% of RCC).

Notes:

  • Subtype is an independent predictor of mortality - but adds very little to multivariate models with staging information.[12]
  • CCRCC tends to be worse than ChRCC and PRCC, probably due to higher incidence of mets.[13]
  • The exam answer (worst to best): clear cell RCC, papillary RCC, chromophobe RCC.

IHC - is it RCC?

  • RCC Ma (+), CD10 (+) -- specific for RCC[14]

IHC - differentiation of types

  • Clear cell RCC vs. papillary RCC:
    • CK7 (-ve CCRCC), AMACR (+ve in PRCC).[15]
  • Papillary RCC type 1 vs. papillary RCC type 2:
    • E-cadherin +ve in PRCC type 2.[16]
    • EMA (MUC1) +ve in PRCC type 1.[16]
  • ChRCC vs. oncocytoma (ONC):
    • CK7 (ChRCC +ve membrane), CK20, CD15.[15]
    • CK7 -- ChRCC 86% +ve vs. ONC 0% +ve.[17]
    • CD15 -- ChRCC 11% +ve vs. ONC 57% +ve.[18]
    • Hale's colloidal iron +ve in ChRCC, usually neg. in ONC.[19]
    • PAX2 -- ChRCC (1/11) +ve vs. ONC (20/23) +ve.[20]
    • Kidney-specific cadherin (Ksp-cadherin) -- ChRCC 97% +ve (distinctive membrane pattern) vs. ONC only 3% +ve.[21]
  • ChRCC & renal oncocytoma vs. others:
    • CD117 (ckit) +ve (100% membrane, ~75% cytoplasmic).[22]
  • Clear cell RCC vs. chromophobe RCC:
    • Hale's colloidal iron (+ve in ChRCC).[19]
    • CK7 (cell membrane +ve in ChRCC).

Notes:

  • One paper[23] describes CD10, parvalbumin, AMACR, CK7 and S100A1 as being useful.
  • Another paper I came across:[24]
  • c-kit (CD117) not useful for differentiating ONC and ChRCC.[20]
  • E-cadherin not useful for differentiating ChRCC and ONC.[25]

RCC vs. Urothelial cell carcinoma

Main article: Urothelium
  • Clinically/radiologically, it may not be possible to differentiate renal pelvis UCC and RCC if the tumour is large.
  • Pathologically, this is not very difficult.
  • On gross specimens, it is almost always obvious what one is dealing with:

Renal cell carcinoma with sarcomatoid differentiation

  • AKA sarcomatoid renal cell carcinoma.
Main article: Renal cell carcinoma with sarcomatoid differentiation

Renal cell carcinoma with rhabdoid morphology

  • AKA renal cell carcinoma with rhabdoid change.
Main article: Renal cell carcinoma with rhabdoid morphology

Hereditary renal cell carcinoma

Main article: Hereditary renal cell carcinoma

Renal cell carcinoma grading

Main article: Renal cell carcinoma grading

Renal cell carcinoma staging

Main article: Kidney cancer staging

Renal sinus invasion

Main article: Kidney cancer staging

Clear cell renal cell carcinoma

Main article: Clear cell renal cell carcinoma

Multilocular cystic renal cell carcinoma

Main article: Multilocular cystic renal cell carcinoma

Papillary renal cell carcinoma

Main article: Papillary renal cell carcinoma

Chromophobe renal cell carcinoma

Main article: Chromophobe renal cell carcinoma

Clear cell papillary renal cell tumour

In the past, it was known as clear cell papillary renal cell carcinoma.

Main article: Clear cell papillary renal cell tumour

Unclassified renal cell carcinoma

  • Abbreviated URCC.
Main article: Unclassified renal cell carcinoma

Renal translocation carcinomas

Renal tumour with Xp11.2 translocation

Main article: Renal tumour with Xp11.2 translocation

Renal tumour with t(6;11) translocation

  • AKA t(6;11) renal cell carcinoma.
Main article: Renal tumour with t(6;11) translocation

Benign tumours

Papillary adenoma of the kidney

  • AKA renal papillary adenoma.
Main article: Papillary adenoma of the kidney

Renal oncocytoma

Main article: Renal oncocytoma

Angiomyolipoma

  • Abbreviated AML.
Main article: Angiomyolipoma

Mimics

Xanthogranulomatous pyelonephritis

  • Abbreviated XGP.
Main article: Xanthogranulomatous pyelonephritis

Malakoplakia

Main article: Malakoplakia

Rare stuffs

Juxtaglomerular cell tumour

  • AKA juxtaglomerular tumour, reninoma.[26]
Main article: Juxtaglomerular cell tumour

Renomedullary interstitial cell tumour

Main article: Renomedullary interstitial cell tumour

Metanephric adenoma

  • Should not be confused mesonephric adenoma, another term for nephrogenic adenoma.
    • Memory device: metanephric adenoma is a tumour.
Main article: Metanephric adenoma

Renal epithelial and stromal tumour

Abbreviated REST.

The lumping term for both:[28]

  1. Mixed epithelial and stromal tumour.
  2. Cystic nephroma.

Mixed epithelial and stromal tumour

  • Abbreviated MEST.
Main article: Mixed epithelial and stromal tumour of the kidney

Cystic nephroma

Main article: Cystic nephroma

Renal mucinous tubular and spindle cell carcinoma

  • AKA renal mucinous tubular spindle cell carcinoma.
  • AKA mucinous tubular and spindle cell carcinoma of the kidney.[29]
Main article: Renal mucinous tubular and spindle cell carcinoma

Collecting duct carcinoma

Main article: Collecting duct carcinoma

Renal medullary carcinoma

Main article: Renal medullary carcinoma

Tubulocystic carcinoma of the kidney

Main article: Tubulocystic carcinoma of the kidney

Acquired cystic disease-associated renal cell carcinoma

Main article: Acquired cystic disease-associated renal cell carcinoma

Kidney metastasis

  • AKA renal metastasis, metastatic kidney disease.
Main article: Kidney metastasis

Pediatric

Main article: Pediatric kidney tumours

The most common is nephroblastoma (Wilms tumour).

Others include:

See also

References

  1. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 288. ISBN 978-0781765275.
  2. ↑ Zhou, Ming; Magi-Galluzzi, Cristina (2006). Genitourinary Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 281-304. ISBN 978-0443066771.
  3. ↑ Srigley, JR.; Delahunt, B. (Jun 2009). "Uncommon and recently described renal carcinomas.". Mod Pathol 22 Suppl 2: S2-S23. doi:10.1038/modpathol.2009.70. PMID 19494850.
  4. ↑ Tickoo, SK.; Amin, MB. (Dec 1998). "Discriminant nuclear features of renal oncocytoma and chromophobe renal cell carcinoma. Analysis of their potential utility in the differential diagnosis.". Am J Clin Pathol 110 (6): 782-7. PMID 9844591.
  5. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 291. ISBN 978-0781765275.
  6. ↑ Srigley, JR.; Delahunt, B.; Eble, JN.; Egevad, L.; Epstein, JI.; Grignon, D.; Hes, O.; Moch, H. et al. (Oct 2013). "The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia.". Am J Surg Pathol 37 (10): 1469-89. doi:10.1097/PAS.0b013e318299f2d1. PMID 24025519.
  7. ↑ URL:http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001544/. Accessed on: 14 July 2011.
  8. ↑ 8.0 8.1 8.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 289. ISBN 978-0781765275.
  9. ↑ Schmid HP, Szabo J (May 1997). "[Renal cell carcinoma--a current review]" (in German). Praxis (Bern 1994) 86 (20): 837?3. PMID 9312811.
  10. ↑ Chow, WH.; McLaughlin, JK.; Mandel, JS.; Wacholder, S.; Niwa, S.; Fraumeni, JF. (Jan 1996). "Obesity and risk of renal cell cancer.". Cancer Epidemiol Biomarkers Prev 5 (1): 17-21. PMID 8770461.
  11. ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 1016. ISBN 0-7216-0187-1.
  12. ↑ Capitanio, U.; Cloutier, V.; Zini, L.; Isbarn, H.; Jeldres, C.; Shariat, SF.; Perrotte, P.; Antebi, E. et al. (Jun 2009). "A critical assessment of the prognostic value of clear cell, papillary and chromophobe histological subtypes in renal cell carcinoma: a population-based study.". BJU Int 103 (11): 1496-500. doi:10.1111/j.1464-410X.2008.08259.x. PMID 19076149.
  13. ↑ Delahunt, B.; Bethwaite, PB.; Nacey, JN. (Oct 2007). "Outcome prediction for renal cell carcinoma: evaluation of prognostic factors for tumours divided according to histological subtype.". Pathology 39 (5): 459-65. doi:10.1080/00313020701570061. PMID 17886093.
  14. ↑ Zhou M, Roma A, Magi-Galluzzi C (June 2005). "The usefulness of immunohistochemical markers in the differential diagnosis of renal neoplasms". Clin. Lab. Med. 25 (2): 247?7. doi:10.1016/j.cll.2005.01.004. PMID 15848735.
  15. ↑ 15.0 15.1 Zhou M, Roma A, Magi-Galluzzi C (June 2005). "The usefulness of immunohistochemical markers in the differential diagnosis of renal neoplasms". Clin. Lab. Med. 25 (2): 247?7. doi:10.1016/j.cll.2005.01.004. PMID 15848735.
  16. ↑ 16.0 16.1 Langner C, Ratschek M, Rehak P, Schips L, Zigeuner R (February 2004). "Expression of MUC1 (EMA) and E-cadherin in renal cell carcinoma: a systematic immunohistochemical analysis of 188 cases". Mod. Pathol. 17 (2): 180?. doi:10.1038/modpathol.3800032. PMID 14657952.
  17. ↑ Liu L, Qian J, Singh H, Meiers I, Zhou X, Bostwick DG (August 2007). "Immunohistochemical analysis of chromophobe renal cell carcinoma, renal oncocytoma, and clear cell carcinoma: an optimal and practical panel for differential diagnosis". Arch. Pathol. Lab. Med. 131 (8): 1290?. PMID 17683191. http://journals.allenpress.com/jrnlserv/?request=get-abstract&issn=0003-9985&volume=131&page=1290.
  18. ↑ Pan CC, Chen PC, Ho DM (November 2004). "The diagnostic utility of MOC31, BerEP4, RCC marker and CD10 in the classification of renal cell carcinoma and renal oncocytoma: an immunohistochemical analysis of 328 cases". Histopathology 45 (5): 452?. doi:10.1111/j.1365-2559.2004.01962.x. PMID 15500648.
  19. ↑ 19.0 19.1 Geramizadeh B, Ravanshad M, Rahsaz M (2008). "Useful markers for differential diagnosis of oncocytoma, chromophobe renal cell carcinoma and conventional renal cell carcinoma". Indian J Pathol Microbiol 51 (2): 167?1. PMID 18603673. http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2008;volume=51;issue=2;spage=167;epage=171;aulast=Geramizadeh.
  20. ↑ 20.0 20.1 Memeo L, Jhang J, Assaad AM, et al. (February 2007). "Immunohistochemical analysis for cytokeratin 7, KIT, and PAX2: value in the differential diagnosis of chromophobe cell carcinoma". Am. J. Clin. Pathol. 127 (2): 225–9. doi:10.1309/9KWEA4W9Y94D1AEE. PMID 17210525. http://ajcp.ascpjournals.org/cgi/pmidlookup?view=long&pmid=17210525.
  21. ↑ Mazal PR, Exner M, Haitel A, et al. (January 2005). "Expression of kidney-specific cadherin distinguishes chromophobe renal cell carcinoma from renal oncocytoma". Hum. Pathol. 36 (1): 22–8. doi:10.1016/j.humpath.2004.09.011. PMID 15712178.
  22. ↑ Krueger S, Sotlar K, Kausch I, Horny HP (2005). "Expression of KIT (CD117) in renal cell carcinoma and renal oncocytoma". Oncology 68 (2-3): 269-75. doi:10.1159/000086783. PMID 16015044.
  23. ↑ Martignoni G, Brunelli M, Gobbo S, et al (February 2007). "Role of molecular markers in diagnosis and prognosis of renal cell carcinoma". Anal. Quant. Cytol. Histol. 29 (1): 41?. PMID 17375873.
  24. ↑ Avery AK, Beckstead J, Renshaw AA, Corless CL (February 2000). "Use of antibodies to RCC and CD10 in the differential diagnosis of renal neoplasms". Am. J. Surg. Pathol. 24 (2): 203?0. PMID 10680888. http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0147-5185&volume=24&issue=2&spage=203.
  25. ↑ Kim MK, Kim S (December 2002). "Immunohistochemical profile of common epithelial neoplasms arising in the kidney". Appl. Immunohistochem. Mol. Morphol. 10 (4): 332–8. PMID 12613443.
  26. ↑ Wong, L.; Hsu, TH.; Perlroth, MG.; Hofmann, LV.; Haynes, CM.; Katznelson, L. (Feb 2008). "Reninoma: case report and literature review.". J Hypertens 26 (2): 368-73. doi:10.1097/HJH.0b013e3282f283f3. PMID 18192852.
  27. ↑ Bircan, S.; Orhan, D.; Tulunay, O.; Safak, M. (2000). "Renomedullary interstitial cell tumor.". Urol Int 65 (3): 163-6. PMID 11054036.
  28. ↑ Turbiner, J.; Amin, MB.; Humphrey, PA.; Srigley, JR.; De Leval, L.; Radhakrishnan, A.; Oliva, E. (Apr 2007). "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.". Am J Surg Pathol 31 (4): 489-500. doi:10.1097/PAS.0b013e31802bdd56. PMID 17414095.
  29. ↑ Brandal, P.; Lie, AK.; Bassarova, A.; Svindland, A.; Risberg, B.; Danielsen, H.; Heim, S. (Feb 2006). "Genomic aberrations in mucinous tubular and spindle cell renal cell carcinomas.". Mod Pathol 19 (2): 186-94. doi:10.1038/modpathol.3800499. PMID 16258504.

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