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Difference between revisions of "Papillary renal cell carcinoma"
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| Caption = Papillary renal cell carcinoma. [[H&E stain]]. | | 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 | | Micro = cuboidal or low columnar cells (simple or pseudostratified) on papillae, interstitial foam cells in the vascular cores | ||
| Subtypes = type 1 | | Subtypes = no ''World Health Organization'' recognized subtypes; defunct subtypes: "type 1" and "type 2", oncocytic variant | ||
| LMDDx = [[clear cell renal cell carcinoma]], [[clear cell papillary renal cell carcinoma]], [[metanephric adenoma]] (esp. solid PaRCC type 1), [[collecting duct carcinoma]] (esp. PaRCC type 2), [[renal papillary adenoma]], [[acquired cystic disease-associated renal cell carcinoma]], [[urothelial carcinoma]], [[renal mucinous tubular and spindle cell carcinoma]] | | LMDDx = [[clear cell renal cell carcinoma]], [[clear cell papillary renal cell carcinoma]], [[metanephric adenoma]] (esp. solid PaRCC type 1), [[collecting duct carcinoma]] (esp. PaRCC type 2), [[renal papillary adenoma]], [[acquired cystic disease-associated renal cell carcinoma]], [[urothelial carcinoma]], [[renal mucinous tubular and spindle cell carcinoma]], [[ALK translocation renal cell carcinoma]], [[Xp11 translocation renal cell carcinoma]] | ||
| Stains = | | Stains = | ||
| IHC = | | IHC = | ||
| EM = | | EM = | ||
| Molecular = | | Molecular = MET mutation (hereditary papillary renal cell carcinoma), FH mutation (hereditary leiomyomatosis and renal cell cancer) | ||
| IF = | | IF = | ||
| Gross = may be multifocal, must be > | | Gross = may be multifocal, must be >1.5 cm (if low ISUP grade), often necrotic-appearing (brown, soft, friable) | ||
| Grossing = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]] | | Grossing = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]] | ||
| Staging = [[kidney cancer staging]] | | Staging = [[kidney cancer staging]] | ||
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==General== | ==General== | ||
* | *Historically, PaRCC was subclassified<ref name=Ref_GUP289>{{Ref GUP|289}}</ref> into ''type 1'' and ''type 2''. | ||
**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> | **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> | ||
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*Associated with ''[[acquired renal cystic disease]]''.<ref name=Ref_DARP438>{{Ref DARP|438}}</ref> | *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> | *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 | **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 (previously ''PaRCC type 1''). | ||
==Gross== | ==Gross== | ||
*Renal cortical mass > | *Renal cortical mass usually >1.5 cm. | ||
*May be multifocal. ‡ | *May be multifocal. ‡ | ||
*Often necrotic appearing - light-to-dark brown, soft/mushy, friable. | *Often necrotic appearing - light-to-dark brown, soft/mushy, friable. | ||
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Size criterion: | Size criterion: | ||
*Papillary lesions '''''must''''' be > | *Papillary lesions with low [[ISUP nucleolar grade|ISUP grade]] '''''must''''' be >1.5 cm to be called ''carcinoma''; smaller lesions (<=1.5 cm) with low ISUP grade are called ''[[renal papillary adenoma|papillary adenoma]]s''.<ref name=pmid26935559>{{Cite journal | last1 = Moch | first1 = H. | last2 = Cubilla | first2 = AL. | last3 = Humphrey | first3 = PA. | last4 = Reuter | first4 = VE. | last5 = Ulbright | first5 = TM. | title = The 2016 WHO Classification of Tumours of the Urinary System and Male Genital Organs-Part A: Renal, Penile, and Testicular Tumours. | journal = Eur Urol | volume = | issue = | pages = | month = Feb | year = 2016 | doi = 10.1016/j.eururo.2016.02.029 | PMID = 26935559 }}</ref><ref name=Ref_GUP288>{{Ref GUP|288}}</ref> † | ||
Mnemonic ''HIP'': '''h'''ighly vascular, '''i'''nterstitial foam cells, '''p'''apillae. | Mnemonic ''HIP'': '''h'''ighly vascular, '''i'''nterstitial foam cells, '''p'''apillae. | ||
Note: | |||
*† The [[diagnostic size cut-off]] in the 2004 [[WHO]] GU (blue) book was 0.5 cm.<ref name=pmid26935559/><ref>{{Ref WHOGU|28}}</ref> | |||
DDx: | DDx: | ||
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*[[Acquired cystic disease-associated renal cell carcinoma]]. | *[[Acquired cystic disease-associated renal cell carcinoma]]. | ||
*[[Renal mucinous tubular and spindle cell carcinoma]]. | *[[Renal mucinous tubular and spindle cell carcinoma]]. | ||
*[[Hereditary leiomyomatosis and renal cell carcinoma]] - for ''type 2 PaRCC''; CK7 -ve, prominent eosinophilic nucleoli. | *[[Hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cell carcinoma]] - for ''type 2 PaRCC''; CK7 -ve, prominent eosinophilic nucleoli. | ||
*[[ALK translocation renal cell carcinoma]]. | |||
*[[Mixed epithelial and stromal tumour]].<ref name=pmid31862520>{{cite journal |authors=Rogala J, Kojima F, Alaghehbandan R, Agaimy A, Martinek P, Ondic O, Ulamec M, Sperga M, Michalova K, Pivovarcikova K, Pitra T, Hora M, Ferak I, Marečková J, Michal M, Hes O |title=Papillary renal cell carcinoma with prominent spindle cell stroma - tumor mimicking mixed epithelial and stromal tumor of the kidney: Clinicopathologic, morphologic, immunohistochemical and molecular genetic analysis of 6 cases |journal=Ann Diagn Pathol |volume=44 |issue= |pages=151441 |date=February 2020 |pmid=31862520 |doi=10.1016/j.anndiagpath.2019.151441 |url=}}</ref> | |||
*[[Papillary renal neoplasm with reverse polarity]] (PRNRP) - nuclei at luminal aspect of cell (rather than closer to the basement membrane).<ref name=pmid31135486>{{cite journal |authors=Al-Obaidy KI, Eble JN, Cheng L, Williamson SR, Sakr WA, Gupta N, Idrees MT, Grignon DJ |title=Papillary Renal Neoplasm With Reverse Polarity: A Morphologic, Immunohistochemical, and Molecular Study |journal=Am J Surg Pathol |volume=43 |issue=8 |pages=1099–1111 |date=August 2019 |pmid=31135486 |doi=10.1097/PAS.0000000000001288 |url=}}</ref> | |||
**May be considered a subtype of papillary renal cell carcinoma (rather than a distinct entity). | |||
*[[Xp11 translocation renal cell carcinoma]]. | |||
===Images=== | ===Images=== | ||
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===Histological subtyping=== | ===Histological subtyping=== | ||
Historically, PaRCC was subtyped:<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'''. | *''Type 1'' - single layer of cells on basement membrane - '''most important'''. | ||
** Usually low grade nuclear features, i.e. low [[ISUP nucleolar grade]]. | ** Usually low grade nuclear features, i.e. low [[ISUP nucleolar grade]]. | ||
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*** Foamy macrophages - uncommon. | *** Foamy macrophages - uncommon. | ||
*** Cells larger. | *** Cells larger. | ||
The WHO GU Blue Book 5th Ed. recommends against subtyping. | |||
Another subtype: | Another subtype: | ||
*''Oncocytic'' - oncocytic cytoplasm. | *''Oncocytic'' - oncocytic cytoplasm. | ||
**Extremely rare ~ | **Extremely rare ~ a few dozen reported.<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><ref name=pmid16730306>{{Cite journal | last1 = Hes | first1 = O. | last2 = Brunelli | first2 = M. | last3 = Michal | first3 = M. | last4 = Cossu Rocca | first4 = P. | last5 = Hora | first5 = M. | last6 = Chilosi | first6 = M. | last7 = Mina | first7 = M. | last8 = Boudova | first8 = L. | last9 = Menestrina | first9 = F. | title = Oncocytic papillary renal cell carcinoma: a clinicopathologic, immunohistochemical, ultrastructural, and interphase cytogenetic study of 12 cases. | journal = Ann Diagn Pathol | volume = 10 | issue = 3 | pages = 133-9 | month = Jun | year = 2006 | doi = 10.1016/j.anndiagpath.2005.12.002 | PMID = 16730306 }}</ref> | ||
***One "large" series was 14 cases.<ref name=pmid27931799>{{Cite journal | last1 = Han | first1 = G. | last2 = Yu | first2 = W. | last3 = Chu | first3 = J. | last4 = Liu | first4 = Y. | last5 = Jiang | first5 = Y. | last6 = Li | first6 = Y. | last7 = Zhang | first7 = W. | title = Oncocytic papillary renal cell carcinoma: A clinicopathological and genetic analysis and indolent clinical course in 14 cases. | journal = Pathol Res Pract | volume = 213 | issue = 1 | pages = 1-6 | month = Jan | year = 2017 | doi = 10.1016/j.prp.2016.04.009 | PMID = 27931799 }}</ref> | |||
==IHC== | ==IHC== | ||
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*CK7 +ve ~90% of type 1, 20% of type 2. | *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> | *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> | ||
* | Others: | ||
*GATA3 +ve - oncocytic variant of PaRCC; negative in other PaRCC subtypes.<ref name=pmid28984673>{{cite journal |authors=Saleeb RM, Brimo F, Farag M, Rompré-Brodeur A, Rotondo F, Beharry V, Wala S, Plant P, Downes MR, Pace K, Evans A, Bjarnason G, Bartlett JMS, Yousef GM |title=Toward Biological Subtyping of Papillary Renal Cell Carcinoma With Clinical Implications Through Histologic, Immunohistochemical, and Molecular Analysis |journal=Am J Surg Pathol |volume=41 |issue=12 |pages=1618–1629 |date=December 2017 |pmid=28984673 |doi=10.1097/PAS.0000000000000962 |url=}}</ref> | |||
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> | 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> | ||
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==Sign out== | ==Sign out== | ||
<pre> | <pre> | ||
Kidney Tumour, Left, Partial Nephrectomy: | Kidney Tumour, Left, Partial Nephrectomy: | ||
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<pre> | <pre> | ||
KIDNEY, RIGHT, RADICAL NEPHRECTOMY: | KIDNEY, RIGHT, RADICAL NEPHRECTOMY: | ||
- PAPILLARY RENAL CELL CARCINOMA, | - PAPILLARY RENAL CELL CARCINOMA, WHO/ISUP NUCLEOLAR GRADE 3, pT2a(2), pNx. | ||
-- SURGICAL MARGINS NEGATIVE. | -- SURGICAL MARGINS NEGATIVE. | ||
-- PLEASE SEE TUMOUR SUMMARY. | -- PLEASE SEE TUMOUR SUMMARY. | ||
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A second tumour with the same morphology is present and measures 8 millimetres. | A second tumour with the same morphology is present and measures 8 millimetres. | ||
Multiple small lesions, like the largest tumour, less than | Multiple small lesions, like the largest tumour, less than 1.5 cm are present. | ||
===Oncocytic variant=== | ===Oncocytic variant=== | ||
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KIDNEY, RIGHT, NEPHRECTOMY: | KIDNEY, RIGHT, NEPHRECTOMY: | ||
- PAPILLARY RENAL CELL CARCINOMA, ONCOCYTIC -- SEE COMMENT; | - PAPILLARY RENAL CELL CARCINOMA, ONCOCYTIC -- SEE COMMENT; | ||
- | - WHO/ISUP GRADE 2; | ||
- SURGICAL MARGINS NEGATIVE; | - SURGICAL MARGINS NEGATIVE; | ||
- PLEASE SEE TUMOUR SUMMARY. | - PLEASE SEE TUMOUR SUMMARY. | ||