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Papillary renal cell carcinoma type 2

MedGen UID:
277837
•Concept ID:
C1336840
•
Neoplastic Process
Synonym: Renal cell carcinoma, papillary type 2
 
HPO: HP:0006732

Definition

A type of papillary renal cell carcinoma in which the papillae are covered by large eosinophilic cells with pleomorphic nuclei, prominent nucleoli, and nuclear pseudostratification. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPapillary renal cell carcinoma type 2

Professional guidelines

PubMed

The clinicopathologic and molecular features, and treatment outcome of fumarate hydratase-deficient renal cell carcinoma: a retrospective comparison with type 2 papillary renal cell carcinoma.
Bai J, Li X, Wen Y, Lu Q, Chen R, Liu R, Shangguan T, Ye Y, Lin J, Cai W, Kang D, Chen J
Aging (Albany NY) 2024 Feb 19;16(4):3631-3646. doi: 10.18632/aging.205549. PMID: 38376408Free PMC Article
The value of CT features and demographic data in the differential diagnosis of type 2 papillary renal cell carcinoma from fat-poor angiomyolipoma and oncocytoma.
Zhou C, Ban X, Luo L, Shi C
Abdom Radiol (NY) 2022 Nov;47(11):3838-3846. Epub 2022 Sep 9 doi: 10.1007/s00261-022-03644-9. PMID: 36085376
Tumors masquerading as type 2 papillary renal cell carcinoma: pathologists' ever-expanding differential diagnosis for a heterogeneous group of entities.
Taylor AS, Skala SL
Urol Oncol 2022 Dec;40(12):499-511. Epub 2021 Jun 8 doi: 10.1016/j.urolonc.2021.04.043. PMID: 34116938

Recent clinical studies

Etiology

Modulating ATP binding cassette transporters in papillary renal cell carcinoma type 2 enhances its response to targeted molecular therapy.
Saleeb RM, Farag M, Lichner Z, Brimo F, Bartlett J, Bjarnason G, Finelli A, Rontondo F, Downes MR, Yousef GM
Mol Oncol 2018 Oct;12(10):1673-1688. Epub 2018 Aug 23 doi: 10.1002/1878-0261.12346. PMID: 29896907Free PMC Article

Diagnosis

Exceptional response to everolimus in a novel tuberous sclerosis complex-2 mutation-associated metastatic renal-cell carcinoma.
Alsidawi S, Kasi PM
Cold Spring Harb Mol Case Stud 2018 Apr;4(2) Epub 2018 Apr 2 doi: 10.1101/mcs.a002220. PMID: 29610387Free PMC Article
Nivolumab-associated Nephrotic Syndrome in a Patient With Renal Cell Carcinoma: A Case Report.
Daanen RA, Maas RJH, Koornstra RHT, Steenbergen EJ, van Herpen CML, Willemsen AECAB
J Immunother 2017 Nov/Dec;40(9):345-348. doi: 10.1097/CJI.0000000000000189. PMID: 28961608
Fumarate Hydratase-deficient Cell Line NCCFH1 as a New In Vitro Model of Hereditary Papillary Renal Cell Carcinoma Type 2.
Perrier-Trudova V, Huimin BW, Kongpetch S, Huang D, Ong P, Le Formal A, Poon SL, Siew EY, Myint SS, Gad S, Gardie B, Couvé S, Foong YM, Choudhury Y, Poh J, Ong CK, Toh CK, Ooi A, Richard S, Tan MH, Teh BT
Anticancer Res 2015 Dec;35(12):6639-53. PMID: 26637880
Whole genome SNP arrays as a potential diagnostic tool for the detection of characteristic chromosomal aberrations in renal epithelial tumors.
Monzon FA, Hagenkord JM, Lyons-Weiler MA, Balani JP, Parwani AV, Sciulli CM, Li J, Chandran UR, Bastacky SI, Dhir R
Mod Pathol 2008 May;21(5):599-608. Epub 2008 Feb 8 doi: 10.1038/modpathol.2008.20. PMID: 18246049
Pathology case of the month. Young woman with abdominal pain and a right upper quadrant mass. Papillary renal cell carcinoma, type 2.
Bhardwaj H, Veillon DM, Heldmann M, Simoneaux WJ, Elmajian DA, Cotelingam JD
J La State Med Soc 2006 Mar-Apr;158(2):60-4; quiz 64, 111. PMID: 16774031

Therapy

Modulating ATP binding cassette transporters in papillary renal cell carcinoma type 2 enhances its response to targeted molecular therapy.
Saleeb RM, Farag M, Lichner Z, Brimo F, Bartlett J, Bjarnason G, Finelli A, Rontondo F, Downes MR, Yousef GM
Mol Oncol 2018 Oct;12(10):1673-1688. Epub 2018 Aug 23 doi: 10.1002/1878-0261.12346. PMID: 29896907Free PMC Article
Exceptional response to everolimus in a novel tuberous sclerosis complex-2 mutation-associated metastatic renal-cell carcinoma.
Alsidawi S, Kasi PM
Cold Spring Harb Mol Case Stud 2018 Apr;4(2) Epub 2018 Apr 2 doi: 10.1101/mcs.a002220. PMID: 29610387Free PMC Article
Nivolumab-associated Nephrotic Syndrome in a Patient With Renal Cell Carcinoma: A Case Report.
Daanen RA, Maas RJH, Koornstra RHT, Steenbergen EJ, van Herpen CML, Willemsen AECAB
J Immunother 2017 Nov/Dec;40(9):345-348. doi: 10.1097/CJI.0000000000000189. PMID: 28961608

Prognosis

Nivolumab-associated Nephrotic Syndrome in a Patient With Renal Cell Carcinoma: A Case Report.
Daanen RA, Maas RJH, Koornstra RHT, Steenbergen EJ, van Herpen CML, Willemsen AECAB
J Immunother 2017 Nov/Dec;40(9):345-348. doi: 10.1097/CJI.0000000000000189. PMID: 28961608
Keap1/Nrf2 pathway in kidney cancer: frequent methylation of KEAP1 gene promoter in clear renal cell carcinoma.
Fabrizio FP, Costantini M, Copetti M, la Torre A, Sparaneo A, Fontana A, Poeta L, Gallucci M, Sentinelli S, Graziano P, Parente P, Pompeo V, De Salvo L, Simone G, Papalia R, Picardo F, Balsamo T, Flammia GP, Trombetta D, Pantalone A, Kok K, Paranita F, Muscarella LA, Fazio VM
Oncotarget 2017 Feb 14;8(7):11187-11198. doi: 10.18632/oncotarget.14492. PMID: 28061437Free PMC Article
Whole genome SNP arrays as a potential diagnostic tool for the detection of characteristic chromosomal aberrations in renal epithelial tumors.
Monzon FA, Hagenkord JM, Lyons-Weiler MA, Balani JP, Parwani AV, Sciulli CM, Li J, Chandran UR, Bastacky SI, Dhir R
Mod Pathol 2008 May;21(5):599-608. Epub 2008 Feb 8 doi: 10.1038/modpathol.2008.20. PMID: 18246049

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