Kidney tumours: Difference between revisions

'''Kidney tumours''' - includes '''malignant kidney tumours''' and '''benign kidney tumours'''.  Medical renal diseases are dealt with in the [[medical renal diseases]] article.

*Renal biopsy - usually of medical disease (see ''[[medical kidney]]'').

*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>

*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>

===Risk factors===

*Hereditary - familial syndromes (see [[Hereditary RCC]]).

 

===Subtypes===

Notes:

*Subtype is an independent predictor of mortality - but adds very little to multivariate models with staging information.<ref>PMID 19076149</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>

 

** 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>

*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>

** 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>

** 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>

 

*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.

 

 

Selected common tumours of the kidney:<ref>GUP PP.281-304.</ref><ref name=pmid19494850>PMID 19494850.</ref>

| ||'''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

|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

|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

|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

|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

==Hereditary RCC==

The classics - which are ''all'' autosomal dominant:<ref>PBoD P.1016.</ref>

# [[Von Hippel-Lindau syndrome]] (clear cell tumours).  

# Hereditary papillary carcinoma (MET proto-oncogene mutation).

 

* 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>

==Grading==

Grading based on '''Fuhrman system''' which considers:<ref>GUP P.282.</ref>

*Nuclear pleomorphism (size, shape),

*Chromatin pattern,

*Nucleoli prominence.

*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===

==Clear cell carcinoma==

===Gross===

*Gold/yellow.

*+/-Haemorrhage (common).

*+/-Necrosis (common in large tumours).

Features:<ref>PBoD P.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.

**+ve in chromophobe.

Note: ''[[Hale's colloidal iron]]'' does not stain iron... it stains hemosiderin.<ref>Latta Jan 27, '09</ref>

 

*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>GUP P.285.</ref>

 

*Often subclassified<ref>GUP P.289.</ref> into ''type 1'' and ''type 2'' -- see below.

*May be abbreviated ''PRCC''.

*Associated with ''dialysis associated cystic disease''.<ref>PBoD PP.1017-8</ref>

===Microsopy===

*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.

*''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>GUP P. 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==

*Sometimes abbreviated ''ChRCC''.

There are two subtypes:<ref>GUP P.293.</ref>

*Classic.

*Eosinophilic variant.

===Gross===

*Tan, light-brown.

*Solitary.

*Well-circumscribed.

===Microscopy===

Classic Histo.<ref>PBoD P.1016-7</ref><ref>GUP P.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>GUP P.293.</ref>

*Eosinophilic (finely granular) cytoplasm.

*Perinuclear clearing - '''key feature'''.

*Periphery of cell distinct.

*Smaller cells than classic subtype.

*May have psammoma bodies.

Image: [http://commons.wikimedia.org/wiki/File:Oncocytic_chromophobe_rcc_high_mag.jpg Oncocytic chromophobe RCC (wikimedia.org)].

===IHC===

*CK7+ cell membrane<ref>GUP P.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

==Collecting duct carcinoma==

===Epidemiology===

*Rare.

===Microscopy===

Features:<ref>GUP P.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>PBoD P.1018.</ref>

 

==Urothelial cell carcinoma==

**UCC = ''nephroureterectomy''.

**RCC = ''nephrectomy'' or ''radical nephrectomy''.

=Benign & mimics=

==Oncocytoma==

===General===

*Can be difficult to distinguish radiologically from RCC (chromophobe subtype).

** ... and pathologists occasionally struggle like the radiologists.

===Gross===

Image: [http://en.wikipedia.org/wiki/File:Renal_oncocytoma.jpg Renal oncocytoma with central scar (wikipedia.org)].

===Histology===

**May need IHC to differentiate (CK7: oncocytoma = cytoplasm +ve, chromophobe = cell membrane +ve).

==Angiomyolipoma==

===Epidemiology===

===Microscopy===

====Cytologic features<ref>PMID 15584043</ref>====

*Nuclei - round/ovoid.

===IHC===

*Epithelial markers -ve.<ref>GUP P.324.</ref>

*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>

Ki-67 can be used to help distinguish btw 'em -- epithelioid variant Ki-67 +ve.<ref>PMID 18839327</ref>

==Xanthogranulomatous pyelonephritis==

===General===

*May mimic RCC (esp. radiologically).

**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>

===Microscopy===

*Abundant macrophages.

===IHC===

*CD68 +ve.

**Basophilic inclusions -- inside or outside of macrophages - often size of RBC or larger ([[Michaelis-Gutmann bodies]]).

==Medullary fibroma==

===General===

===Epidemiology===

===Gross===

*Small, white well circumscribed nodule in medulla.

===Microscopy===

*Spindle cells.

==Metanephric adenoma==

===General===

*Benign.

===Micro===

**No apparent [[w:nucleolus|nucleolus]].

*Wilm tumour (nephroblastoma) - these typically have:

**Irregular nuclear membrane.

**Nucleoli.

==Cystic nephroma==

===Microscopic===

*[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===

*ER +ve.

===DDx===

*Rare.<ref name=pmid19494850>PMID 19494850</ref>

*Mucin - may be scant.

*Spindle cells.

==See also==

==References==