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Grade 4 Clear Cell Renal Cell Carcinoma

MedGen UID:
272679
Concept ID:
C1333852
Neoplastic Process
Synonym: Grade 4 Conventional (Clear Cell) Renal Cell Carcinoma

Definition

A clear cell renal cell carcinoma characterized by extreme nuclear pleomorphism and/or sarcomatoid and/or rhabdoid differentiation and/or tumor giant cells. (WHO, 1982 /ISUP 2014) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVGrade 4 Clear Cell Renal Cell Carcinoma

Professional guidelines

PubMed

Ljungberg B, Albiges L, Abu-Ghanem Y, Bedke J, Capitanio U, Dabestani S, Fernández-Pello S, Giles RH, Hofmann F, Hora M, Klatte T, Kuusk T, Lam TB, Marconi L, Powles T, Tahbaz R, Volpe A, Bex A
Eur Urol 2022 Oct;82(4):399-410. Epub 2022 Mar 26 doi: 10.1016/j.eururo.2022.03.006. PMID: 35346519
Trpkov K, Hes O, Williamson SR, Adeniran AJ, Agaimy A, Alaghehbandan R, Amin MB, Argani P, Chen YB, Cheng L, Epstein JI, Cheville JC, Comperat E, da Cunha IW, Gordetsky JB, Gupta S, He H, Hirsch MS, Humphrey PA, Kapur P, Kojima F, Lopez JI, Maclean F, Magi-Galluzzi C, McKenney JK, Mehra R, Menon S, Netto GJ, Przybycin CG, Rao P, Rao Q, Reuter VE, Saleeb RM, Shah RB, Smith SC, Tickoo S, Tretiakova MS, True L, Verkarre V, Wobker SE, Zhou M, Gill AJ
Mod Pathol 2021 Jul;34(7):1392-1424. Epub 2021 Mar 4 doi: 10.1038/s41379-021-00779-w. PMID: 33664427
Spain L, Diem S, Larkin J
Cancer Treat Rev 2016 Mar;44:51-60. Epub 2016 Feb 6 doi: 10.1016/j.ctrv.2016.02.001. PMID: 26874776

Recent clinical studies

Etiology

Gui CP, Chen YH, Zhao HW, Cao JZ, Liu TJ, Xiong SW, Yu YF, Liao B, Cao Y, Li JY, Huang KB, Han H, Zhang ZL, Chen WF, Jiang ZY, Gao Y, Han GP, Tang Q, Ouyang K, Qu GM, Wu JT, Guo JP, Li CX, Li PX, Liu ZP, Hsieh JT, Cai MY, Li XS, Wei JH, Luo JH
Lancet Digit Health 2023 Aug;5(8):e515-e524. Epub 2023 Jun 29 doi: 10.1016/S2589-7500(23)00095-X. PMID: 37393162
Motzer RJ, Powles T, Burotto M, Escudier B, Bourlon MT, Shah AY, Suárez C, Hamzaj A, Porta C, Hocking CM, Kessler ER, Gurney H, Tomita Y, Bedke J, Zhang J, Simsek B, Scheffold C, Apolo AB, Choueiri TK
Lancet Oncol 2022 Jul;23(7):888-898. Epub 2022 Jun 7 doi: 10.1016/S1470-2045(22)00290-X. PMID: 35688173Free PMC Article
Ravaud A, Motzer RJ, Pandha HS, George DJ, Pantuck AJ, Patel A, Chang YH, Escudier B, Donskov F, Magheli A, Carteni G, Laguerre B, Tomczak P, Breza J, Gerletti P, Lechuga M, Lin X, Martini JF, Ramaswamy K, Casey M, Staehler M, Patard JJ; S-TRAC Investigators
N Engl J Med 2016 Dec 8;375(23):2246-2254. Epub 2016 Oct 9 doi: 10.1056/NEJMoa1611406. PMID: 27718781
Choueiri TK, Escudier B, Powles T, Tannir NM, Mainwaring PN, Rini BI, Hammers HJ, Donskov F, Roth BJ, Peltola K, Lee JL, Heng DYC, Schmidinger M, Agarwal N, Sternberg CN, McDermott DF, Aftab DT, Hessel C, Scheffold C, Schwab G, Hutson TE, Pal S, Motzer RJ; METEOR investigators
Lancet Oncol 2016 Jul;17(7):917-927. Epub 2016 Jun 5 doi: 10.1016/S1470-2045(16)30107-3. PMID: 27279544
Motzer RJ, Hutson TE, Glen H, Michaelson MD, Molina A, Eisen T, Jassem J, Zolnierek J, Maroto JP, Mellado B, Melichar B, Tomasek J, Kremer A, Kim HJ, Wood K, Dutcus C, Larkin J
Lancet Oncol 2015 Nov;16(15):1473-1482. Epub 2015 Oct 22 doi: 10.1016/S1470-2045(15)00290-9. PMID: 26482279

Diagnosis

Nyman J, Denize T, Bakouny Z, Labaki C, Titchen BM, Bi K, Hari SN, Rosenthal J, Mehta N, Jiang B, Sharma B, Felt K, Umeton R, Braun DA, Rodig S, Choueiri TK, Signoretti S, Van Allen EM
Cell Rep Med 2023 Sep 19;4(9):101189. doi: 10.1016/j.xcrm.2023.101189. PMID: 37729872Free PMC Article
Gui CP, Chen YH, Zhao HW, Cao JZ, Liu TJ, Xiong SW, Yu YF, Liao B, Cao Y, Li JY, Huang KB, Han H, Zhang ZL, Chen WF, Jiang ZY, Gao Y, Han GP, Tang Q, Ouyang K, Qu GM, Wu JT, Guo JP, Li CX, Li PX, Liu ZP, Hsieh JT, Cai MY, Li XS, Wei JH, Luo JH
Lancet Digit Health 2023 Aug;5(8):e515-e524. Epub 2023 Jun 29 doi: 10.1016/S2589-7500(23)00095-X. PMID: 37393162
Lobo J, Ohashi R, Amin MB, Berney DM, Compérat EM, Cree IA, Gill AJ, Hartmann A, Menon S, Netto GJ, Raspollini MR, Rubin MA, Tan PH, Tickoo SK, Tsuzuki T, Turajlic S, Zhou M, Srigley JR, Moch H
Histopathology 2022 Oct;81(4):426-438. Epub 2022 Jun 10 doi: 10.1111/his.14700. PMID: 35596618
Delahunt B, Eble JN, Egevad L, Samaratunga H
Histopathology 2019 Jan;74(1):4-17. doi: 10.1111/his.13735. PMID: 30565310
Delahunt B, Cheville JC, Martignoni G, Humphrey PA, Magi-Galluzzi C, McKenney J, Egevad L, Algaba F, Moch H, Grignon DJ, Montironi R, Srigley JR; Members of the ISUP Renal Tumor Panel
Am J Surg Pathol 2013 Oct;37(10):1490-504. doi: 10.1097/PAS.0b013e318299f0fb. PMID: 24025520

Therapy

Choueiri TK, Tomczak P, Park SH, Venugopal B, Ferguson T, Symeonides SN, Hajek J, Chang YH, Lee JL, Sarwar N, Haas NB, Gurney H, Sawrycki P, Mahave M, Gross-Goupil M, Zhang T, Burke JM, Doshi G, Melichar B, Kopyltsov E, Alva A, Oudard S, Topart D, Hammers H, Kitamura H, McDermott DF, Silva A, Winquist E, Cornell J, Elfiky A, Burgents JE, Perini RF, Powles T; KEYNOTE-564 Investigators
N Engl J Med 2024 Apr 18;390(15):1359-1371. doi: 10.1056/NEJMoa2312695. PMID: 38631003
Powles T, Tomczak P, Park SH, Venugopal B, Ferguson T, Symeonides SN, Hajek J, Gurney H, Chang YH, Lee JL, Sarwar N, Thiery-Vuillemin A, Gross-Goupil M, Mahave M, Haas NB, Sawrycki P, Burgents JE, Xu L, Imai K, Quinn DI, Choueiri TK; KEYNOTE-564 Investigators
Lancet Oncol 2022 Sep;23(9):1133-1144. doi: 10.1016/S1470-2045(22)00487-9. PMID: 36055304
Choueiri TK, Tomczak P, Park SH, Venugopal B, Ferguson T, Chang YH, Hajek J, Symeonides SN, Lee JL, Sarwar N, Thiery-Vuillemin A, Gross-Goupil M, Mahave M, Haas NB, Sawrycki P, Gurney H, Chevreau C, Melichar B, Kopyltsov E, Alva A, Burke JM, Doshi G, Topart D, Oudard S, Hammers H, Kitamura H, Bedke J, Perini RF, Zhang P, Imai K, Willemann-Rogerio J, Quinn DI, Powles T; KEYNOTE-564 Investigators
N Engl J Med 2021 Aug 19;385(8):683-694. doi: 10.1056/NEJMoa2106391. PMID: 34407342
Choueiri TK, Powles T, Burotto M, Escudier B, Bourlon MT, Zurawski B, Oyervides Juárez VM, Hsieh JJ, Basso U, Shah AY, Suárez C, Hamzaj A, Goh JC, Barrios C, Richardet M, Porta C, Kowalyszyn R, Feregrino JP, Żołnierek J, Pook D, Kessler ER, Tomita Y, Mizuno R, Bedke J, Zhang J, Maurer MA, Simsek B, Ejzykowicz F, Schwab GM, Apolo AB, Motzer RJ; CheckMate 9ER Investigators
N Engl J Med 2021 Mar 4;384(9):829-841. doi: 10.1056/NEJMoa2026982. PMID: 33657295Free PMC Article
Motzer RJ, Tannir NM, McDermott DF, Arén Frontera O, Melichar B, Choueiri TK, Plimack ER, Barthélémy P, Porta C, George S, Powles T, Donskov F, Neiman V, Kollmannsberger CK, Salman P, Gurney H, Hawkins R, Ravaud A, Grimm MO, Bracarda S, Barrios CH, Tomita Y, Castellano D, Rini BI, Chen AC, Mekan S, McHenry MB, Wind-Rotolo M, Doan J, Sharma P, Hammers HJ, Escudier B; CheckMate 214 Investigators
N Engl J Med 2018 Apr 5;378(14):1277-1290. Epub 2018 Mar 21 doi: 10.1056/NEJMoa1712126. PMID: 29562145Free PMC Article

Prognosis

Powles T, Tomczak P, Park SH, Venugopal B, Ferguson T, Symeonides SN, Hajek J, Gurney H, Chang YH, Lee JL, Sarwar N, Thiery-Vuillemin A, Gross-Goupil M, Mahave M, Haas NB, Sawrycki P, Burgents JE, Xu L, Imai K, Quinn DI, Choueiri TK; KEYNOTE-564 Investigators
Lancet Oncol 2022 Sep;23(9):1133-1144. doi: 10.1016/S1470-2045(22)00487-9. PMID: 36055304
Motzer RJ, Powles T, Burotto M, Escudier B, Bourlon MT, Shah AY, Suárez C, Hamzaj A, Porta C, Hocking CM, Kessler ER, Gurney H, Tomita Y, Bedke J, Zhang J, Simsek B, Scheffold C, Apolo AB, Choueiri TK
Lancet Oncol 2022 Jul;23(7):888-898. Epub 2022 Jun 7 doi: 10.1016/S1470-2045(22)00290-X. PMID: 35688173Free PMC Article
Delahunt B, Eble JN, Egevad L, Samaratunga H
Histopathology 2019 Jan;74(1):4-17. doi: 10.1111/his.13735. PMID: 30565310
Choueiri TK, Escudier B, Powles T, Tannir NM, Mainwaring PN, Rini BI, Hammers HJ, Donskov F, Roth BJ, Peltola K, Lee JL, Heng DYC, Schmidinger M, Agarwal N, Sternberg CN, McDermott DF, Aftab DT, Hessel C, Scheffold C, Schwab G, Hutson TE, Pal S, Motzer RJ; METEOR investigators
Lancet Oncol 2016 Jul;17(7):917-927. Epub 2016 Jun 5 doi: 10.1016/S1470-2045(16)30107-3. PMID: 27279544
Pierorazio PM, Johnson MH, Patel HD, Sozio SM, Sharma R, Iyoha E, Bass EB, Allaf ME
J Urol 2016 Oct;196(4):989-99. Epub 2016 May 6 doi: 10.1016/j.juro.2016.04.081. PMID: 27157369Free PMC Article

Clinical prediction guides

Gui CP, Chen YH, Zhao HW, Cao JZ, Liu TJ, Xiong SW, Yu YF, Liao B, Cao Y, Li JY, Huang KB, Han H, Zhang ZL, Chen WF, Jiang ZY, Gao Y, Han GP, Tang Q, Ouyang K, Qu GM, Wu JT, Guo JP, Li CX, Li PX, Liu ZP, Hsieh JT, Cai MY, Li XS, Wei JH, Luo JH
Lancet Digit Health 2023 Aug;5(8):e515-e524. Epub 2023 Jun 29 doi: 10.1016/S2589-7500(23)00095-X. PMID: 37393162
Choueiri TK, McDermott DF, Merchan J, Bauer TM, Figlin R, Heath EI, Michaelson MD, Arrowsmith E, D'Souza A, Zhao S, Roy A, Perini R, Vickery D, Tykodi SS
Lancet Oncol 2023 May;24(5):553-562. Epub 2023 Mar 31 doi: 10.1016/S1470-2045(23)00097-9. PMID: 37011650
Lee CH, Voss MH, Carlo MI, Chen YB, Zucker M, Knezevic A, Lefkowitz RA, Shapnik N, Dadoun C, Reznik E, Shah NJ, Owens CN, McHugh DJ, Aggen DH, Laccetti AL, Kotecha R, Feldman DR, Motzer RJ
J Clin Oncol 2022 Jul 20;40(21):2333-2341. Epub 2022 Mar 17 doi: 10.1200/JCO.21.01944. PMID: 35298296Free PMC Article
Chen S, Jiang L, Gao F, Zhang E, Wang T, Zhang N, Wang X, Zheng J
Br J Cancer 2022 Mar;126(5):771-777. Epub 2021 Nov 25 doi: 10.1038/s41416-021-01640-2. PMID: 34824449Free PMC Article
Delahunt B, Eble JN, Egevad L, Samaratunga H
Histopathology 2019 Jan;74(1):4-17. doi: 10.1111/his.13735. PMID: 30565310

Recent systematic reviews

Wu Z, Chen Q, Qu L, Li M, Wang L, Mir MC, Carbonara U, Pandolfo SD, Black PC, Paul AK, Di Lorenzo G, Porpiglia F, Mari A, Necchi A, Rouprêt M, Psutka SP, Autorino R
Eur Urol 2022 Apr;81(4):414-425. Epub 2022 Jan 31 doi: 10.1016/j.eururo.2022.01.028. PMID: 35101302
Hussaini S, Chehade R, Boldt RG, Raphael J, Blanchette P, Maleki Vareki S, Fernandes R
Cancer Treat Rev 2021 Jan;92:102134. Epub 2020 Dec 3 doi: 10.1016/j.ctrv.2020.102134. PMID: 33302134
El Rassy E, Aoun F, Sleilaty G, Kattan J, Banyurwabuke B, Zanaty M, Bakouny Z, Albisinni S, Peltier A, Roumeguere T
Future Oncol 2017 Dec;13(29):2709-2717. Epub 2017 Nov 29 doi: 10.2217/fon-2017-0268. PMID: 29183164
Khoja L, Day D, Wei-Wu Chen T, Siu LL, Hansen AR
Ann Oncol 2017 Oct 1;28(10):2377-2385. doi: 10.1093/annonc/mdx286. PMID: 28945858
Pierorazio PM, Johnson MH, Patel HD, Sozio SM, Sharma R, Iyoha E, Bass EB, Allaf ME
J Urol 2016 Oct;196(4):989-99. Epub 2016 May 6 doi: 10.1016/j.juro.2016.04.081. PMID: 27157369Free PMC Article

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