Papillary renal cell carcinoma: Difference between revisions
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| 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 = no ''World Health Organization'' recognized subtypes; defunct subtypes: "type 1" and "type 2", oncocytic variant | | 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]] | | LMDDx = [[clear cell renal cell carcinoma]], [[clear cell papillary renal cell carcinoma]], [[metanephric adenoma]], [[collecting duct carcinoma]], [[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]], [[FH-deficient renal cell carcinoma]] | ||
| Stains = | | Stains = | ||
| IHC = | | IHC = | ||
| EM = | | EM = | ||
| Molecular = | | Molecular = MET mutation (hereditary papillary renal cell carcinoma) | ||
| IF = | | IF = | ||
| Gross = may be multifocal, must be >1.5 cm (if low ISUP grade), often necrotic-appearing (brown, soft, friable) | | Gross = may be multifocal, must be >1.5 cm (if low ISUP grade), often necrotic-appearing (brown, soft, friable) | ||
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| Site = [[kidney]] - see [[kidney tumours]] | | Site = [[kidney]] - see [[kidney tumours]] | ||
| Assdx = [[acquired renal cystic disease]] ([[end-stage renal disease]]) | | Assdx = [[acquired renal cystic disease]] ([[end-stage renal disease]]) | ||
| Syndromes = hereditary papillary renal cell carcinoma | | Syndromes = hereditary papillary renal cell carcinoma | ||
| Clinicalhx = | | Clinicalhx = | ||
| Signs = | | Signs = | ||
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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== | ||
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</gallery> | </gallery> | ||
===Histological subtyping=== | ===Histological subtyping=== | ||
====Historical type 1 and type 2==== | |||
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> | 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'''. | ||
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The WHO GU Blue Book 5th Ed. recommends against subtyping. | The WHO GU Blue Book 5th Ed. recommends against subtyping. | ||
How are ''Type 1'' and ''Type 2'' now classified? | |||
*The defunct ''Type 1'' is ''papillary renal cell carcinoma''. It is classically associated with a MET gene alteration. | |||
*The defunct ''Type 2'' is a mix of different RCCs, e.g. ALK-RCC, translocation RCC, FH-deficient RCC. | |||
===Oncocytic subtype=== | |||
*''Oncocytic'' - oncocytic cytoplasm. | *''Oncocytic'' - oncocytic cytoplasm. | ||
**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> | **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> | ||
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Others: | 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> | *[[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> | ||
*[[ABCC2]] brush border staining = worse survival.<ref name=pmid37680023>{{cite journal |authors=Castillo VF, Masoomian M, Trpkov K, Downes M, Brimo F, van der Kwast T, Yousef GM, Zakhary A, Rotondo F, Saad G, Nguyen VN, Kidanewold W, Streutker C, Rowsell C, Hamdani M, Saleeb RM |title=ABCC2 brush-border expression predicts outcome in papillary renal cell carcinoma: a multi-institutional study of 254 cases |journal=Histopathology |volume=83 |issue=6 |pages=949–958 |date=December 2023 |pmid=37680023 |doi=10.1111/his.15042 |url=}}</ref> | |||
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> | 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> | ||
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Multiple small lesions, like the largest tumour, less than 1.5 cm are present. | Multiple small lesions, like the largest tumour, less than 1.5 cm are present. | ||
=====Alternate===== | |||
The sections show a tumour in the kidney with fibrovascular cores (papillae) that contain macrophages. The papillae predominantly have a single layer of tumour cells. Nucleoli are not prominent with the 10x objective (ISUP nucleolar grade 2). | |||
===Oncocytic variant=== | ===Oncocytic variant=== | ||