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.
 
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 the [[adrenal gland]]).
*[[Radical nephrectomy]] - includes Gerota's fascia.
**May include the [[adrenal gland]].<ref name=Ref_WMSP|288>{{Ref WMSP|288}}</ref>


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


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


Selected common tumours of the kidney:<ref>{{Ref GUP|281-304}}</ref><ref name=pmid19494850>{{Cite journal  | last1 = Srigley | first1 = JR. | last2 = Delahunt | first2 = B. | title = Uncommon and recently described renal carcinomas. | journal = Mod Pathol | volume = 22 Suppl 2 | issue =  | pages = S2-S23 | month = Jun | year = 2009 | doi = 10.1038/modpathol.2009.70 | PMID = 19494850 }}</ref>
Selected common tumours of the kidney:<ref name=Ref_GUP281>{{Ref GUP|281-304}}</ref><ref name=pmid19494850>{{Cite journal  | last1 = Srigley | first1 = JR. | last2 = Delahunt | first2 = B. | title = Uncommon and recently described renal carcinomas. | journal = Mod Pathol | volume = 22 Suppl 2 | issue =  | pages = S2-S23 | month = Jun | year = 2009 | doi = 10.1038/modpathol.2009.70 | PMID = 19494850 }}</ref>
{| class="wikitable"
{| class="wikitable"
|
|
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|-
|-
|IHC
|IHC
| CK7-, EMA+
| [[CK7]]-, EMA+
| AMACR+, EMA+, CK7+
| AMACR+, EMA+, CK7+
| AMACR+, E-cadherin+, CK7-
| AMACR+, E-cadherin+, CK7-
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|-
|-
|Image(s)
|Image(s)
| [http://commons.wikimedia.org/wiki/File:Renal_clear_cell_ca_%281%29_Nephrectomy.jpg CCRCC (WC)]
| [[Image:Renal_clear_cell_ca_%281%29_Nephrectomy.jpg|thumb|150px| CCRCC (WC)]]
| [http://commons.wikimedia.org/wiki/File:Papillary_renal_cell_carcinoma_intermed_mag.jpg PaRCC - intermed. (WC)], [http://commons.wikimedia.org/wiki/File:Papillary_renal_cell_carcinoma_very_high_mag.jpg PaRCC - high (WC)]
| [[Image:Papillary_renal_cell_carcinoma_intermed_mag.jpg|thumb|150px| PaRCC - intermed. (WC)]], [[Image:Papillary_renal_cell_carcinoma_very_high_mag.jpg | thumb|150px| PaRCC - high (WC)]]
|
|
|
|
| [http://commons.wikimedia.org/wiki/File:Oncocytic_chromophobe_rcc_high_mag.jpg ChRCC (WC)]
| [[Image:Oncocytic_chromophobe_rcc_high_mag.jpg|thumb|150px| ChRCC (WC)]]
| [http://commons.wikimedia.org/wiki/File:Renal_oncocytoma2.jpg Oncocytoma (WC)]
| [[Image:Renal_oncocytoma2.jpg|thumb|150px| Oncocytoma (WC)]]
|}
|}
Notes:
Notes:
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===Tabular comparison of oncocytoma and chromophobe RCC===
===Tabular comparison of oncocytoma and chromophobe RCC===
Histomorphologic features useful to distinguish chromophobe RCC (eosinophilic variant) and oncocytoma:<ref name=pmid9844591>{{Cite journal  | last1 = Tickoo | first1 = SK. | last2 = Amin | first2 = MB. | title = Discriminant nuclear features of renal oncocytoma and chromophobe renal cell carcinoma. Analysis of their potential utility in the differential diagnosis. | journal = Am J Clin Pathol | volume = 110 | issue = 6 | pages = 782-7 | month = Dec | year = 1998 | doi =  | PMID = 9844591 }}
Histomorphologic features useful to distinguish chromophobe RCC (eosinophilic variant) and oncocytoma:<ref name=pmid9844591>{{Cite journal  | last1 = Tickoo | first1 = SK. | last2 = Amin | first2 = MB. | title = Discriminant nuclear features of renal oncocytoma and chromophobe renal cell carcinoma. Analysis of their potential utility in the differential diagnosis. | journal = Am J Clin Pathol | volume = 110 | issue = 6 | pages = 782-7 | month = Dec | year = 1998 | doi =  | PMID = 9844591 }}</ref>
</ref>
{| class="wikitable sortable"
{| class="wikitable"
! Morphologic feature
|
! [[ChRCC]] (eosinophilic<br> variant)
| '''ChRCC (eosinophilic<br> variant)'''
! [[Renal oncocytoma]]
| '''Renal oncocytoma'''
|-
|-
| Nuclear morphology
| Nuclear morphology
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|-
|-
| Image
| Image
| [http://commons.wikimedia.org/wiki/File:Oncocytic_chromophobe_rcc_high_mag.jpg ChRCC (WC)]
| [[Image:Oncocytic_chromophobe_rcc_high_mag.jpg |thumb|150px|ChRCC (WC/Nephron)]]
| [http://commons.wikimedia.org/wiki/File:Renal_oncocytoma2.jpg Oncocytoma (WC)]
| [[Image:Renal_oncocytoma2.jpg |thumb|150px|Oncocytoma (WC/Nephron)]]
|}
|}
===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]].
Benign spindle cell tumours of the kidney:
*[[Angiomyolipoma]].
*[[Cystic nephroma]].
====Renal tumours with eosinophilic cytoplasm====
{{Main|Renal tumours with eosinophilic cytoplasm}}
=WHO classification of renal neoplasia=
*Based on 2004 iteration - as per WMSP, slightly modified.<ref>{{Ref WMSP|291}}</ref> Online, the classification can [http://www.urosource.com/fileadmin/user_upload/european_urology/PIIS0302283805008316.pdf found here].
===Renal cell tumours===
Common:
*[[Clear cell renal cell carcinoma]].
*[[Papillary renal cell carcinoma]].
*[[Renal papillary adenoma|Papillary adenoma]].
*[[Chromophobe renal cell carcinoma]].
*[[Renal oncocytoma|Oncocytoma]].
Less common:
*[[Multilocular clear cell renal cell carcinoma]].
*[[Carcinoma of the collecting ducts of Bellini]].
*[[Renal cell carcinoma, unclassified]].
*[[Renal medullary carcinoma]].
*[[Renal tumour with Xp11.2 translocation|Xp11 translocation carcinoma]].
*[[Mucinous tubular and spindle cell carcinoma]].
*Carcinoma associated with neuroblastoma.
===Metanephric tumours===
*[[Metanephric adenoma]].
*[[Metanephric stromal tumour]].
*[[Metanephric adenofibroma]].
===Nephroblastic tumours===
*Nephrogenic rests.
*[[Nephroblastoma]].
===Mesenchymal tumours===
Childhood:
*[[Clear cell sarcoma of the kidney]].
*[[Rhabdoid tumour]].
*[[Mesoblastic nephroma|Congenital mesoblastic nephroma]].
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]].
===Mixed mesenchymal and epithelial tumours===
*[[Cystic nephroma]].
*[[Mixed epithelial and stromal tumour]].
*[[Synovial sarcoma]].
===Others===
*[[Neuroendocrine tumours]].
*Hematologic tumours.
*[[Germ cell tumour]]s.
*[[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]].
==An entity proposed after Vancouver==
*[[Eosinophilic, solid and cystic renal cell carcinoma]].
*[[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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*Often abbreviated '''RCC'''.
*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>
*[[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===
===Origin===
*Proximal renal tubule.
*Proximal renal tubule.
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===Risk factors===
===Risk factors===
*[[Smoking]].
*[[Smoking]] - most important.<ref name=Ref_WMSP289>{{Ref WMSP|289}}</ref>
*Some chemicals.
*Chemical exposures (arsenic, asbestos, cadmium, organic solvents, pesticides, fungal toxins).<ref name=Ref_WMSP289>{{Ref WMSP|289}}</ref>
*Hemodialysis.
*Chronic renal failure.
**[[Acquired cystic renal disease]].
*Male>female (~2:1).
*Male>female (~2:1).
*Hereditary - familial syndromes (see [[Hereditary RCC]]).
*Hereditary - familial syndromes (see [[Hereditary RCC]]).
*[[Obesity]].<ref name=pmid8770461>{{Cite journal  | last1 = Chow | first1 = WH. | last2 = McLaughlin | first2 = JK. | last3 = Mandel | first3 = JS. | last4 = Wacholder | first4 = S. | last5 = Niwa | first5 = S. | last6 = Fraumeni | first6 = JF. | title = Obesity and risk of renal cell cancer. | journal = Cancer Epidemiol Biomarkers Prev | volume = 5 | issue = 1 | pages = 17-21 | month = Jan | year = 1996 | doi =  | PMID = 8770461 }}</ref>


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


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


*ChRCC vs. oncocytoma (ONC):
*ChRCC vs. oncocytoma (ONC):
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*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>
*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>


==Hereditary RCC==
===RCC vs. Urothelial cell carcinoma===
The classics - which are ''all'' autosomal dominant:<ref name=Ref_PBoD1016>{{Ref PBoD|1016}}</ref>
# [[Von Hippel-Lindau syndrome]] (clear cell tumours).
# Hereditary clear cell carcinoma (VHL gene).
# Hereditary papillary carcinoma (MET proto-oncogene mutation).
 
Others:
* Hereditary papillary carcinoma (TFE3 related translocations).<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/314310 http://www.ncbi.nlm.nih.gov/omim/314310]. Accessed on: 16 June 2010.</ref>
 
Review paper:<br>
{{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 }}
 
==Grading RCC==
===General===
Some subtypes are graded based on the '''Fuhrman system''' which considers:<ref name=Ref_GUP282>{{Ref GUP|282}}</ref>
*Nuclear pleomorphism (size, shape),
*Chromatin pattern,
*Nucleoli prominence.
 
Notes:
*The system was validated for clear cell RCC. 
*Fuhrman nuclear grade is '''not''' prognostic in ''chromophobe RCC'' and should not be used in that context.<ref name=pmid1949485>{{cite journal |author=Brovkina AF, Val'skii VV |title=[Computer tomography in the diagnosis of intraocular melanomas] |language=Russian |journal=Vestn Oftalmol |volume=107 |issue=4 |pages=39–42 |year=1991 |pmid=1949485 |doi= |url=}}</ref>
 
===Criteria & grades===
*Grade 1: no nucleoli, near 'normal' appearance.
*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===
*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.
 
==Clear cell carcinoma==
===Gross===
*Gold/yellow.
*+/-Haemorrhage (common).
*+/-Necrosis (common in large tumours).
 
===Microscopic===
Features:<ref>{{Ref PBoD|1017-8}}</ref>
*Clear cells.
*Solid or [[trabecular]] pattern.
*Delicate branching vasculature.
 
*[[Hyaline bodies]] common.<ref>AFIP Renal Tumours Book.</ref>
**Not common in papillary RCC.
 
===IHC===
*CK7 -ve.
*CK20 -ve.
*Hale's colloidal iron -ve.
**+ve in chromophobe.
 
Note: ''[[Hale's colloidal iron]]'' does not stain iron... it stains hemosiderin.<ref>Latta. 27 January 2009.</ref>
Clear cell vs. chromophobe:
*Chromophobe: "translucent" (NOT quite clear), reticulated, +Hale's colloidal iron stain, CK7+ (cell membrane).
 
Clear cell vs. [[adrenocortical carcinoma]]:
*ACC: EMA- (epithelial membrane antigen), cytokeratin mostly neg., inhibin+ (neg. in RCC).<ref name=Ref_GUP285>{{Ref GUP|285}}</ref>
 
==Papillary RCC==
===General===
*Often subclassified<ref name=Ref_GUP289>{{Ref GUP|289}}</ref> into ''type 1'' and ''type 2'' -- see below.
*May be abbreviated ''PRCC''.
 
===Epidemiology===
*Associated with ''dialysis associated cystic disease''.<ref>{{Ref PBoD|1017-8}}</ref>
 
===Microsopy===
Features:<ref>{{Ref PBoD|1017-8}}</ref>
*Cuboidal or low columnar cell in papillae.
*Interstitial foam cells in vascular cores - '''key feature'''.<ref>ALS Feb 9, 2009.</ref>
**Most sensitive and specific feature of PRCC.<ref>{{cite journal |author=Granter SR, Perez-Atayde AR, Renshaw AA |title=Cytologic analysis of papillary renal cell carcinoma |journal=Cancer |volume=84 |issue=5 |pages=303?8 |year=1998 |month=October |pmid=9801205 |doi= |url=http://dx.doi.org/10.1002/(SICI)1097-0142(19981025)84:5<303::AID-CNCR6>3.0.CO;2-7}}</ref>
*Highly vascular.
Mnemonic ''HIP'':  '''h'''ighly vascular, '''i'''nterstitial foam cells, '''p'''apillae.
 
Size criterion:
*Papillary lesions '''''must''''' be >0.5 cm to be called ''carcinoma''; smaller lesions (<=0.5 cm) are called ''papillary adenomas''.<ref name=Ref_GUP288>{{Ref GUP|288}}</ref>
 
Histological subtyping:<ref name=Ref_GUP289>{{Ref GUP|289}}</ref>
*''Type 1'' - single layer of cells on basement membrane.
** usually low grade nuclear features, i.e. low Fuhrman grade.
*''Type 2'' - pseudostratification of cells.
** Usually high grade nuclear features, i.e. high Fuhrman grade.
 
===IHC===
Features:<ref name=Ref_GUP289>{{Ref GUP|289}}</ref>
*AMACR +ve.<ref>ALS Feb 9, 2009.</ref>
*HMWCK (34betaE12) +ve.
*Panker (AE1/AE3) +ve.
*CK7 +ve ~90% of type 1, 20% of type 2.
 
More reading:
*[http://www.e-immunohistochemistry.info/web/Papillary_renal_cell_carcinoma.htm e-immunohistochemistry.info]
 
===Clear cell vs. papillary===
*Papillary: +histiocytes, +intracellular hemosiderin, [[CK7]]+.
 
==Chromophobe RCC==
===General===
*Sometimes abbreviated ''ChRCC''.
 
There are two subtypes:<ref name=Ref_GUP293>{{Ref GUP|293}}</ref>
*Classic.
*Eosinophilic variant.
 
===Gross===
*Tan, light-brown.
*Solitary.
*Well-circumscribed.
 
===Microscopy===
Classic Histo.<ref>{{Ref PBoD|1016-7}}</ref><ref name=Ref_GUP293>{{Ref GUP|293}}</ref>, 3 P's [[mnemonic]]:
*Pale cytoplasm, with wisps of eosinophilic material; the cells are not completely clear, they have "cobwebs".
*Perinuclear clearing, i.e. a pale halo surrounds the nucleus - '''key feature'''.
*Periphery of cell distinct, i.e. cell membrane is easy to discern.
 
Eosinophilic variant:<ref name=Ref_GUP293>{{Ref GUP|293}}</ref>
*Eosinophilic (finely granular) cytoplasm.
*Perinuclear clearing - '''key feature'''.
*Periphery of cell distinct.
*Smaller cells than classic subtype.
 
Notes:
*May have psammoma bodies.
 
Image: [http://commons.wikimedia.org/wiki/File:Oncocytic_chromophobe_rcc_high_mag.jpg Oncocytic chromophobe RCC (wikimedia.org)].
 
===IHC===
*CK7 +ve cell membrane.<ref name=Ref_GUP293>{{Ref GUP|293}}</ref>
 
DDx:
*May appear similar to oncotyoma -- particularily the eosinophilic variant.
**IHC: CK7: chromophobe = cell membrane CK7+; oncoctyoma = patchy cytoplasmic +ve
*Classic ChRCC may be challenging to differentiate from clear cell RCC.
**Perinuclear clearing is ''not'' seen in clear cell RCC.
**ChRCC has wisps in the cytoplasm
 
==Urothelial cell carcinoma==
{{main|Urothelium}}
{{main|Urothelium}}
*Clinically/radiologically, it may not be possible to differentiate renal pelvis UCC and RCC if the tumour is large.
*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.   
*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]]''.


=Benign & mimics=
===Renal cell carcinoma with sarcomatoid differentiation===
*[[AKA]] ''sarcomatoid renal cell carcinoma''.
{{Main|Renal cell carcinoma with sarcomatoid differentiation}}


==Oncocytoma==
===Renal cell carcinoma with rhabdoid morphology===
===General===
*[[AKA]] ''renal cell carcinoma with rhabdoid change''.
*Can be difficult to distinguish radiologically from RCC (chromophobe subtype).
{{Main|Renal cell carcinoma with rhabdoid morphology}}
** ... 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===
==Hereditary renal cell carcinoma==
*Brown, mahogany brown.
{{Main|Hereditary renal cell carcinoma}}
*1/3 have a characteristic central scar.<ref name=Ref_GUP302>{{Ref GUP|302}}</ref>


Image: [http://en.wikipedia.org/wiki/File:Renal_oncocytoma.jpg Renal oncocytoma with central scar (wikipedia.org)].
==Renal cell carcinoma grading==
{{Main|Renal cell carcinoma grading}}


===Histology===
==Renal cell carcinoma staging==
Features:
{{Main|Kidney cancer staging}}
*Eosinophilic cytoplasm - slightly granular '''key feature'''.
*Cells arranged in nests.
*Nuclei uniform and round.<ref name=Ref_GUP302>{{Ref GUP|302}}</ref>
**Slightly enlarged nuclei, but '''no significant''' pleomorphism (size variation) - '''important'''.


Notes:
===Renal sinus invasion===
*May look like eosinophilic variant of chromophobe RCC -- this is the main DDx.
{{Main|Kidney cancer staging}}
**May need IHC to differentiate (CK7: oncocytoma = cytoplasm +ve, chromophobe = cell membrane +ve).
**No perinuclear clearing -- this is seen in ChRCC.


Images:
==Clear cell renal cell carcinoma==
*[http://commons.wikimedia.org/wiki/File:Renal_oncocytoma2.jpg High mag. oncocytoma (WC)].
{{Main|Clear cell renal cell carcinoma}}
*[http://commons.wikimedia.org/wiki/File:Renal_oncocytoma3.jpg Intermed. mag. oncocytoma (WC)].
*[http://commons.wikimedia.org/wiki/File:Renal_oncocytoma4.jpg Low mag. oncocytoma (WC)].


==Angiomyolipoma==
==Multilocular cystic renal cell carcinoma==
===General===
{{Main|Multilocular cystic renal cell carcinoma}}
*Mostly 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>
*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===
==Papillary renal cell carcinoma==
*May be associated with [[tuberous sclerosis]] -- 70% have an AML.
{{Main|Papillary renal cell carcinoma}}
**When compared to sporadic cases:
***More often bilateral.
***Usually bigger.


===Microscopy===
==Chromophobe renal cell carcinoma==
*Muscle.
{{Main|Chromophobe renal cell carcinoma}}
*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>
*Blood vessels.


====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>====
==Clear cell papillary renal cell tumour==
*Nuclei - round/ovoid.
In the past, it was known as ''clear cell papillary renal cell carcinoma''.
*Chromatin - bland.
{{Main|Clear cell papillary renal cell tumour}}


===IHC===
==Unclassified renal cell carcinoma==
*Melanocytic markers +ve<ref name=Ref_GUP324>{{Ref GUP|324}}</ref> (e.g. HMB-45 +ve).
*Abbreviated ''URCC''.
*Epithelial markers -ve.<ref name=Ref_GUP324>{{Ref GUP|324}}</ref>
{{Main|Unclassified renal cell carcinoma}}
*SMA +ve.
*CD117 +/-.
Additional paper: <ref name=pmid15584043/>


Notes:
=Renal translocation carcinomas=
*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> but this is not the case in 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>
==Renal tumour with Xp11.2 translocation==
Ki-67 can be used to help distinguish btw 'em -- epithelioid variant Ki-67 +ve.<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>
{{Main|Renal tumour with Xp11.2 translocation}}


==Xanthogranulomatous pyelonephritis==
==Renal tumour with t(6;11) translocation==
===General===
*[[AKA]] ''t(6;11) renal cell carcinoma''.
*May mimic RCC (esp. radiologically).
{{main|Renal tumour with t(6;11) translocation}}
*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===
=Benign tumours=
*Abundant macrophages.
==Papillary adenoma of the kidney==
*+/-Giant cells.
*[[AKA]] ''renal papillary adenoma''.
{{Main|Papillary adenoma of the kidney}}


Image:
==Renal oncocytoma==
*[http://commons.wikimedia.org/wiki/File:Xanthogranulomatous_pyelonephritis_cd68.jpg Xanthogranulomatous_pyelonephritis - CD68 IHC (mediawiki.org)].
{{Main|Renal oncocytoma}}


===IHC===
==Angiomyolipoma==
*CD68 +ve.
*Abbreviated ''AML''.
*RCC markers (CD10, RCC) all negative.
{{Main|Angiomyolipoma}}


DDx:
=Mimics=
*[[Malakoplakia]].
==Xanthogranulomatous pyelonephritis==
**Basophilic inclusions -- inside or outside of macrophages - often size of RBC or larger ([[Michaelis-Gutmann bodies]]).
*Abbreviated ''XGP''.
*RCC - esp. PaRCC (as this has foamy macrophages).
{{Main|Xanthogranulomatous pyelonephritis}}
*Granulomatous disease.


==Malakoplakia==
==Malakoplakia==
Line 454: Line 388:


=Rare stuffs=
=Rare stuffs=
==Medullary fibroma==
==Juxtaglomerular cell tumour==
===General===
*[[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>
*Rare.
{{Main|Juxtaglomerular cell tumour}}


===Epidemiology===
==Renomedullary interstitial cell tumour==
*Benign.
*[[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}}
===Gross===
*Small, white well circumscribed nodule in medulla.
 
===Microscopy===
*Spindle cells.


==Metanephric adenoma==
==Metanephric adenoma==
===General===
*Should '''not''' be confused ''[[mesonephric adenoma]]'', another term for ''nephrogenic adenoma''.
*Benign.
**Memory device: me'''t'''anephric adenoma is a '''t'''umour.
 
{{Main|Metanephric adenoma}}
===Micro===
Features:
*Small uniform cells with:
**Fine chromatin.
**No apparent [[nucleolus]].
**A relatively smooth nuclear membrane.  
*Variable architecture - may be sheets or ductal.


DDx:
==Renal epithelial and stromal tumour==
*Wilm tumour (nephroblastoma) - these typically have:
:Abbreviated ''REST''.
**Irregular nuclear membrane.
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>
**Nucleoli.
#[[Mixed epithelial and stromal tumour]].
**Mitoses.
#[[Cystic nephroma]].


Images:
==Mixed epithelial and stromal tumour==
*[http://commons.wikimedia.org/wiki/File:Metanephric_adenoma_high_mag.jpg Metanephric adenoma (WC)].
*Abbreviated ''MEST''.
*[http://commons.wikimedia.org/wiki/File:Metanephric_adenoma_high_mag_cropped.jpg Metanephric adenoma - cropped (WC)].
{{Main|Mixed epithelial and stromal tumour of the kidney}}
*[http://www.nature.com/modpathol/journal/v16/n10/fig_tab/3880886f1.html Metanephric adenoma (nature.com)].


==Cystic nephroma==
==Cystic nephroma==
===General===
{{Main|Cystic nephroma}}
*AKA ''renal epithelial stromal tumour (REST)'' and ''mixed epithelial stromal tumour (MEST)''.
*Rare - approx. 1.5% of renal neoplasms.<ref name=pmid17454754>PMID 17454754.</ref>
*Benign.
*Prevalence: males > females.
 
===Microscopic===
Features:
*Cysts lined by simple epithelium with hobnailing.
*Stroma has an ovarian look:
**''Blue'' (basophilic).
**''Spindle cells''.
 
Imagea:
*[http://commons.wikimedia.org/wiki/File:Cystic_nephroma_low_mag.jpg Cystic nephroma - low mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Cystic_nephroma_intermed_mag.jpg Cystic nephroma - intermed. mag. (WC)].
 
===IHC===
Features:<ref name=pmid17454754>PMID 17454754</ref>
*ER +ve.
*PR +ve.
*CD10 +ve.
 
===DDx===
*Tubulocystic carcinoma.


==Renal mucinous tubular and spindle cell carcinoma==
==Renal mucinous tubular and spindle cell carcinoma==
===General===
*[[AKA]] ''renal mucinous tubular 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>
*[[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>
*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>
{{Main|Renal mucinous tubular and spindle cell carcinoma}}
 
===Microscopy===
Features:<ref name=pmid19066686>{{cite journal |author=Trabelsi A, Stita W, Yacoubi MT, Rammeh S, Hmissa S, Korbi S |title=Renal mucinous tubular and spindle cell carcinoma |journal=Can Urol Assoc J |volume=2 |issue=6 |pages=635–6 |year=2008 |month=December |pmid=19066686 |pmc=2593603 |doi= |url=}}</ref>
*Mucin - may be scant.
*Spindle cells.
 
DDx:
*Sarcomatoid papillary RCC. (???) <ref name=pmid18941398>{{cite journal |author=Dhillon J, Amin MB, Selbs E, Turi GK, Paner GP, Reuter VE |title=Mucinous tubular and spindle cell carcinoma of the kidney with sarcomatoid change |journal=Am. J. Surg. Pathol. |volume=33 |issue=1 |pages=44–9 |year=2009 |month=January |pmid=18941398 |doi=10.1097/PAS.0b013e3181829ed5 |url=}}</ref>
 
===IHC===
Features:<ref name=pmid16231179>{{cite journal |author=Ferlicot S, Allory Y, Compérat E, ''et al.'' |title=Mucinous tubular and spindle cell carcinoma: a report of 15 cases and a review of the literature |journal=Virchows Arch. |volume=447 |issue=6 |pages=978–83 |year=2005 |month=December |pmid=16231179 |doi=10.1007/s00428-005-0036-x |url=}}</ref>
*CD10 -ve.
*AE1/AE3 +ve.
*AMACR +ve.
*CK7 +ve.


==Collecting duct carcinoma==
==Collecting duct carcinoma==
===Epidemiology===
{{Main|Collecting duct carcinoma}}
*Rare.
 
===Microscopy===
Features:<ref name=Ref_GUP295>{{Ref GUP|295}}</ref>
*Tubular structures with tapered ends.
*High grade nuclear features (nuclear pleomorphism).
*High mitotic rate.
*[[Hobnail pattern]] - cell width smaller at basement membrane than free surface ??? <ref>{{Ref PBoD|1018}}</ref>


==Renal medullary carcinoma==
==Renal medullary carcinoma==
===General===
{{Main|Renal medullary carcinoma}}
*Rare.
*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>


===Gross===
==Tubulocystic carcinoma of the kidney==
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>
{{Main|Tubulocystic carcinoma of the kidney}}
*Well circumscribed.
*Medulla.


===Microscopic===
==Acquired cystic disease-associated renal cell carcinoma==
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>
{{Main|Acquired cystic disease-associated renal cell carcinoma}}
*Variable architecture:
**Reticular - classic.
**[[Adenoid cystic carcinoma]]-like appearance:
***Cystic spaces.
**Yolk sac-like.
**Tubular.
*Desmoplastic stroma - prominent.
*Inflammation:
**Lymphocytes.
**Neutrophils - margination in vessels.


Kidney disease assoc. with sickle cell disorders:<ref name=pmid7528470/>
==Kidney metastasis==
*Papillary necrosis.
*[[AKA]] ''renal metastasis'', ''metastatic kidney disease''.
*Nephrotic syndrome.
{{Main|Kidney metastasis}}
*Renal infarction.
*Pyelonephritis.
 
Image:
*[http://www.nature.com/modpathol/journal/v20/n9/fig_tab/3800934f3.html#figure-title RMC (nature.com)].


=Pediatric=
=Pediatric=
==Wilms tumour==
{{Main|Pediatric kidney tumours}}
*[[AKA]] nephroblastoma, AKA Wilms' tumour.
 
===General===
*Common abdominal [[pediatric pathology|pediatric]] tumour.
*May be associated with a syndrome:<ref>URL: [http://emedicine.medscape.com/article/989398-overview http://emedicine.medscape.com/article/989398-overview]. Accessed on: 9 March 2011.</ref>
**WAGR syndrome (Wilms tumour, Aniridia (absence of iris), GU abnormalities, Retardation).<ref>{{OMIM|194072}}</ref>
**[[Beckwith-Wiedemann syndrome]].<ref>{{OMIM|130650}}</ref>
**Denys-Drash syndrome.<ref>{{OMIM|194080}}</ref>
 
===Gross===
*Lobulated tan mass.
 
Image: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/rnfrm.html Wilms tumour (med.utah.edu)].
 
===Microscopic===
Features - classically three components (blastema, immature stroma, tubules):<ref name=Ref_PCPBoD8_254-5>{{Ref PCPBoD8|254-5}}</ref>
#Malignant [[Small round blue cell tumours|small round blue cells]] ("blastema"):
#*Size = ~ 2x RBC diameter.
#*Nuclear pleomorphism (variation of size, shape and staining).
#**Irregular nuclear membrane - '''important'''.
#*Scant/difficult to discern cytoplasm - basophilic (light blue).
#*Mitoses - common.
#Stroma ("immature stroma"):
#*Spindle cells:
#**Elliptical nuclear membrane.
#**Abundant loose cytoplasm.
#Tubular structures ("tubules"):
#*Usually clustered.
#*Vaguely resemble a glomerulus.
#*Usu. have a central (clear/white) space surrounded by a rim of intensely eosinophilic cytoplasm.
#*Nuclei of tubular structures often elongated and palisaded.
 
Other findings:
*Commonly seen in association with ''nephrogenic rests''.
**Cluster of cells small (blue) cells; lack nuclear atypia seen in Wilms tumour.<ref>URL: [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970416-8 http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970416-8]. Accessed on: 28 March 2011.</ref>
 
 
DDx:
*Metanephric adenoma.
*Nephrogenic nests.
*Other small round cell tumours.
*[[Synovial sarcoma]] - esp. in adults.
 
Notes:
*+/-Distinct nucleoli. (???)
*Palisade = fence made of stakes driven into the ground.<ref>URL: [http://www.thefreedictionary.com/palisaded http://www.thefreedictionary.com/palisaded]. Accessed on: 2 February 2011.</ref>
*Approximately 30-40% Wilms tumour cases have nephrogenic rests.<ref name=pmid8047084>{{cite journal |author=Coppes MJ, Haber DA, Grundy PE |title=Genetic events in the development of Wilms' tumor |journal=N. Engl. J. Med. |volume=331 |issue=9 |pages=586–90 |year=1994 |month=September |pmid=8047084 |doi=10.1056/NEJM199409013310906 |url=}}</ref>
 
Images:
*[http://www.biologydisease.com/images/kidney/nephrogenic-rests/nephrogenic-rest.jpg.php Nephrogenic rests (biologydisease.com)].
*[http://www.webpathology.com/image.asp?n=1&Case=73 Wilms tumour (webpathology.com)].
*[http://commons.wikimedia.org/wiki/File:Wilms_tumour_-_low_mag.jpg Wilms tumour - low mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Wilms_tumour_-_very_high_mag.jpg Wilms tumour - very high mag. (WC)].


====Anaplasia====
The most common is [[nephroblastoma]] (Wilms tumour).
Any of the following:
#Atypical mitoses.
#Hyperchromasia.
#Nuclear size variation (of the tumour cells) > 3x.


===IHC===
Others include:
*WT-1 +ve.
*[[Metanephric stromal tumour]].
*[[Metanephric adenofibroma]]
*[[Metanephric adenoma]].
*[[Clear cell sarcoma of the kidney]].


=See also=
=See also=

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).

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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

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

  • 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.

Renal cell carcinoma with rhabdoid morphology

  • AKA renal cell carcinoma with rhabdoid change.

Hereditary renal cell carcinoma

Renal cell carcinoma grading

Renal cell carcinoma staging

Renal sinus invasion

Clear cell renal cell carcinoma

Multilocular cystic renal cell carcinoma

Papillary renal cell carcinoma

Chromophobe renal cell carcinoma

Clear cell papillary renal cell tumour

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

Unclassified renal cell carcinoma

  • Abbreviated URCC.

Renal translocation carcinomas

Renal tumour with Xp11.2 translocation

Renal tumour with t(6;11) translocation

  • AKA t(6;11) renal cell carcinoma.

Benign tumours

Papillary adenoma of the kidney

  • AKA renal papillary adenoma.

Renal oncocytoma

Angiomyolipoma

  • Abbreviated AML.

Mimics

Xanthogranulomatous pyelonephritis

  • Abbreviated XGP.

Malakoplakia

Rare stuffs

Juxtaglomerular cell tumour

  • AKA juxtaglomerular tumour, reninoma.[26]

Renomedullary interstitial cell tumour

Metanephric adenoma

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

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.

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]

Collecting duct carcinoma

Renal medullary carcinoma

Tubulocystic carcinoma of the kidney

Acquired cystic disease-associated renal cell carcinoma

Kidney metastasis

  • AKA renal metastasis, metastatic kidney disease.

Pediatric

The most common is nephroblastoma (Wilms tumour).

Others include:

See also

References

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  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.
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  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.
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  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.
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  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.
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External links