View source for Papillary renal cell carcinoma

{{ Infobox diagnosis
| Name       = {{PAGENAME}}
| Image      = Papillary renal cell carcinoma -- very high mag.jpg
| Width      =
| Caption    = Papillary renal cell carcinoma. [[H&E stain]].
| Micro      = cuboidal or low columnar cells (simple or pseudostratified) on papillae, interstitial foam cells in the vascular cores
| Subtypes   = type 1, type 2, oncocytic variant
| LMDDx      = [[clear cell renal cell carcinoma]], [[clear cell papillary renal cell carcinoma]], [[metanephric adenoma]] (esp. solid PRCC type 1), [[collecting duct carcinoma]] (esp. PRCC type 2), [[renal papillary adenoma]], [[acquired cystic disease-associated renal cell carcinoma]], [[urothelial carcinoma]], [[renal mucinous tubular and spindle cell carcinoma]]
| Stains     =
| IHC        =
| EM         =
| Molecular  =
| IF         =
| Gross      = may be multifocal, must be >0.5 cm (otherwise [[renal papillary adenoma]])
| Grossing   =
| Site       = [[kidney]] - see [[kidney tumours]]
| Assdx      = [[acquired renal cystic disease]] ([[end-stage renal disease]])
| Syndromes  = hereditary papillary renal cell carcinoma
| Clinicalhx =
| Signs      =
| Symptoms   =
| Prevalence = relatively common
| Bloodwork  =
| Rads       =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    = other [[kidney tumours]]
| Tx         = surgical excision, ablation
}}
'''Papillary renal cell carcinoma''', abbreviated '''PRCC''', '''PaRCC''' and '''papillary RCC''', is the second most common type of [[renal cell carcinoma]].

==General==
*Often subclassified<ref name=Ref_GUP289>{{Ref GUP|289}}</ref> into ''type 1'' and ''type 2'' -- see ''microscopic''.
**Type 1 and Type 2 are different on a cytogenetic and molecular basis.<ref>{{Cite journal  | last1 = Klatte | first1 = T. | last2 = Pantuck | first2 = AJ. | last3 = Said | first3 = JW. | last4 = Seligson | first4 = DB. | last5 = Rao | first5 = NP. | last6 = LaRochelle | first6 = JC. | last7 = Shuch | first7 = B. | last8 = Zisman | first8 = A. | last9 = Kabbinavar | first9 = FF. | title = Cytogenetic and molecular tumor profiling for type 1 and type 2 papillary renal cell carcinoma. | journal = Clin Cancer Res | volume = 15 | issue = 4 | pages = 1162-9 | month = Feb | year = 2009 | doi = 10.1158/1078-0432.CCR-08-1229 | PMID = 19228721 }}</ref>

===Epidemiology===
*Associated with ''[[acquired renal cystic disease]]''.<ref name=Ref_DARP438>{{Ref DARP|438}}</ref>
*May be familial - uncommon.<ref name=pmid12629341>{{Cite journal  | last1 = Czene | first1 = K. | last2 = Hemminki | first2 = K. | title = Familial papillary renal cell tumors and subsequent cancers: a nationwide epidemiological study from Sweden. | journal = J Urol | volume = 169 | issue = 4 | pages = 1271-5 | month = Apr | year = 2003 | doi = 10.1097/01.ju.0000052373.36963.12 | PMID = 12629341 }}</ref>
**MET mutation<ref name=pmid22717761>{{Cite journal  | last1 = Wadt | first1 = KA. | last2 = Gerdes | first2 = AM. | last3 = Hansen | first3 = TV. | last4 = Toft | first4 = BG. | last5 = Friis-Hansen | first5 = L. | last6 = Andersen | first6 = MK. | title = Novel germline c-MET mutation in a family with hereditary papillary renal carcinoma. | journal = Fam Cancer | volume = 11 | issue = 3 | pages = 535-7 | month = Sep | year = 2012 | doi = 10.1007/s10689-012-9542-6 | PMID = 22717761 }}</ref> - autosomal dominant transmission, PaRCC type 1.

==Gross==
*Renal cortical mass > 0.5 cm.
*May be multifocal.

==Microscopic==
Features:<ref name=Ref_PBoD1017-8>{{Ref PBoD|1017-8}}</ref>
*Cuboidal or low columnar cell in papillae.
*Interstitial foam cells in vascular cores - '''key feature'''.
**Most sensitive and specific feature of PRCC.<ref>{{cite journal |author=Granter SR, Perez-Atayde AR, Renshaw AA |title=Cytologic analysis of papillary renal cell carcinoma |journal=Cancer |volume=84 |issue=5 |pages=303?8 |year=1998 |month=October |pmid=9801205 |doi= |url=http://dx.doi.org/10.1002/(SICI)1097-0142(19981025)84:5<303::AID-CNCR6>3.0.CO;2-7}}</ref>
*Highly vascular.

Size criterion:
*Papillary lesions '''''must''''' be >0.5 cm to be called ''carcinoma''; smaller lesions (<=0.5 cm) are called ''[[renal papillary adenoma|papillary adenoma]]s''.<ref name=Ref_GUP288>{{Ref GUP|288}}</ref>

Mnemonic ''HIP'':  '''h'''ighly vascular, '''i'''nterstitial foam cells, '''p'''apillae.

DDx:
*[[Clear cell RCC]].
**Papillary: histiocytes, intracellular hemosiderin, [[CK7]] +ve.
*[[Clear cell papillary renal cell carcinoma]] - apical nuclei, usu. no true papillae.
*[[Metanephric adenoma]] (esp. solid PRCC type 1) - no histiocytes, WT-1 +ve.
*[[Collecting duct carcinoma]] - esp. PRCC type 2.
*[[Urothelial carcinoma]].
*[[Renal papillary adenoma]] - doesn't fulfill size criterion for PaRCC.
*[[Acquired cystic disease-associated renal cell carcinoma]].
*[[Renal mucinous tubular and spindle cell carcinoma]].

===Images===
<gallery>
Image:Papillary_renal_cell_carcinoma_intermed_mag.jpg| PaRCC - intermed. mag. (WC/Nephron) 
Image:Papillary_renal_cell_carcinoma_high_mag.jpg| PaRCC - high mag. (WC/Nephron)
Image:Papillary_renal_cell_carcinoma_very_high_mag.jpg| PaRCC - very high mag. (WC/Nephron)
</gallery>
<gallery>
Image: Papillary renal cell carcinoma - 2 -- intermed mag.jpg | PaRCC - intermed. mag. (WC)
Image: Papillary renal cell carcinoma - 2 -- high mag.jpg | PaRCC - high mag. (WC)
Image: Papillary renal cell carcinoma - 2 -- very high mag.jpg | PaRCC - very high mag. (WC)
</gallery>
<gallery>
Image: Papillary renal cell carcinoma -- high mag.jpg | PaRCC - high mag. (WC/Nephron)
Image: Papillary renal cell carcinoma -- very high mag.jpg | PaRCC - very high mag. (WC/Nephron)
</gallery>

===Histological subtyping===
Generally accepted subtypes:<ref name=Ref_GUP289>{{Ref GUP|289}}</ref><ref>{{Cite journal  | last1 = Delahunt | first1 = B. | last2 = Eble | first2 = JN. | title = Papillary renal cell carcinoma: a clinicopathologic and immunohistochemical study of 105 tumors. | journal = Mod Pathol | volume = 10 | issue = 6 | pages = 537-44 | month = Jun | year = 1997 | doi =  | PMID = 9195569 }}</ref>
*''Type 1'' - single layer of cells on basement membrane - '''most important'''.
** Usually low grade nuclear features, i.e. low Fuhrman grade.
** Other characteristics:
*** Clear cytoplasm.
*** Foamy macrophages - common.
*** Cells smaller.
*''Type 2'' - pseudostratification of cells - '''most important'''.
** Usually high grade nuclear features, i.e. high Fuhrman grade.
** Other characteristics:
*** Eosinophilic cytoplasm.
*** Foamy macrophages - uncommon.
*** Cells larger.

Another subtype:
*''Oncocytic'' - oncocytic cytoplasm.
**Extremely rare ~ largest series is 12 cases.<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>

==IHC==
Features:<ref name=Ref_GUP289>{{Ref GUP|289}}</ref>
*AMACR +ve.
*HMWCK (34betaE12) +ve.
*Panker (AE1/AE3) +ve.
*CK7 +ve ~90% of type 1, 20% of type 2.
*CD10 +ve.<ref>[http://surgpathcriteria.stanford.edu/kidney/papillary-renal-cell-carcinoma/differential-diagnosis.html http://surgpathcriteria.stanford.edu/kidney/papillary-renal-cell-carcinoma/differential-diagnosis.html]. Accessed on: 6 May 2014.</ref>
More reading: 
*[http://www.e-immunohistochemistry.info/web/Papillary_renal_cell_carcinoma.htm PaRCC (e-immunohistochemistry.info)].

Type 1 versus Type 2:<ref name=pmid9234615>{{Cite journal  | last1 = Ono | first1 = Y. | last2 = Ito | first2 = T. | last3 = Tsujino | first3 = S. | last4 = Aizawa | first4 = S. | last5 = Suzuki | first5 = M. | title = [A study of papillary renal cell carcinoma. Clinicopathological, immunohistochemical features and its typing]. | journal = Nihon Hinyokika Gakkai Zasshi | volume = 88 | issue = 6 | pages = 587-95 | month = Jun | year = 1997 | doi =  | PMID = 9234615 }}</ref>
*CK7: 
**Type 1 ~ 100%.
**Type 2 ~ 19%.
*CK19:
**Type 1 ~ 100%.
**Type 2 ~ 53%.

Metanephric adenoma vs. PaRCC type 1:<ref name=pmid24083046>{{Cite journal  | last1 = Watanabe | first1 = S. | last2 = Naganuma | first2 = H. | last3 = Shimizu | first3 = M. | last4 = Ota | first4 = S. | last5 = Murata | first5 = S. | last6 = Nihei | first6 = N. | last7 = Matsushima | first7 = J. | last8 = Mikami | first8 = S. | last9 = Kuroda | first9 = N. | title = Adult nephroblastoma with predominant epithelial component: a differential diagnostic candidate of papillary renal cell carcinoma and metanephric adenoma-report of three cases. | journal = Case Rep Pathol | volume = 2013 | issue =  | pages = 675875 | month =  | year = 2013 | doi = 10.1155/2013/675875 | PMID = 24083046 }}</ref>
*AMACR +ve.
*WT-1 -ve.
*CD57 -ve.

==Molecular==
Features:<ref name=Ref_PBoD1016>{{Ref PBoD|1016}}</ref>
*Sporadic: trisomies 7, 16, 17.
*Familial: trisomy 7.
**Chromosome 7 = location of MET gene.

Note:
*Not used for diagnosis.<ref>{{Ref WMSP|292}}</ref>

==Sign out==
<pre>
KIDNEY, RIGHT, RADICAL NEPHRECTOMY:
- PAPILLARY RENAL CELL CARCINOMA, TYPE 1, FUHRMANN GRADE 3, pT2a(2), pNx.
-- SURGICAL MARGINS NEGATIVE.
-- PLEASE SEE TUMOUR SUMMARY.
- RENAL PAPILLARY ADENOMAS.
</pre>

====Micro====
The sections show a tumour in the kidney with fibrovascular cores (papillae) that focally contain macrophages. Psammoma bodies are present. Siderophages are present.

The papillae predominantly have a single layer of tumour cells and the cytoplasm of the tumour cells is predominantly clear.

Nucleoli are visible focally with the 10x objective (Fuhrman grade 3).

A second tumour with the same morphology is present and measures 8 millimetres.

Multiple small lesions, like the largest tumour, less than 0.5 cm are present.

===Oncocytic variant===
<pre>
KIDNEY, RIGHT, NEPHRECTOMY:
- PAPILLARY RENAL CELL CARCINOMA, ONCOCYTIC -- SEE COMMENT;
- FUHRMANN GRADE 2;
- SURGICAL MARGINS NEGATIVE;
- PLEASE SEE TUMOUR SUMMARY.

COMMENT:
The oncocytic variant of papillary renal cell carcinoma (RCC) is uncommon and not widely
recognized as a subtype of papillary RCC.  The prognostic significance of the oncocytic 
cytoplasm is uncertain.[1]  The histomorphology in this case is compatible with a type 1 
papillary RCC.

1. Ann Diagn Pathol. 2006 Jun;10(3):133-9.
</pre>

==See also==
*[[Kidney tumours]].
*[[Acquired cystic disease-associated renal cell carcinoma]].

==References==
{{Reflist|2}}

[[Category:Kidney tumours]]
[[Category:Diagnosis]]