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Advanced Unclassified Renal Cell Carcinoma

MedGen UID:
1765814
•Concept ID:
C5419191
•
Neoplastic Process

Definition

Unclassified renal cell carcinoma that has spread extensively to other anatomic sites or is no longer responding to treatment. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdvanced Unclassified Renal Cell Carcinoma

Professional guidelines

PubMed

Advances in non-clear cell renal cell carcinoma management: From heterogeneous biology to treatment options.
Wilson NR, Acikgoz Y, Hasanov E
Int J Cancer 2024 Mar 15;154(6):947-961. Epub 2023 Oct 12 doi: 10.1002/ijc.34756. PMID: 37823185
Novel, emerging and provisional renal entities: The Genitourinary Pathology Society (GUPS) update on renal neoplasia.
Trpkov K, Williamson SR, Gill AJ, 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, Hes O
Mod Pathol 2021 Jun;34(6):1167-1184. Epub 2021 Feb 1 doi: 10.1038/s41379-021-00737-6. PMID: 33526874
Advanced Non-Clear Cell Kidney Cancer: In Search of Rational Treatment Approaches.
Ornstein MC, Hutson TE
Cancer J 2020 Sep/Oct;26(5):441-447. doi: 10.1097/PPO.0000000000000474. PMID: 32947312

Recent clinical studies

Etiology

The 2022 revision of the World Health Organization classification of tumors of the urinary system and male genital organs: advances and challenges.
Mohanty SK, Lobo A, Cheng L
Hum Pathol 2023 Jun;136:123-143. Epub 2022 Sep 6 doi: 10.1016/j.humpath.2022.08.006. PMID: 36084769
Safety and efficacy of nivolumab plus ipilimumab in patients with advanced non-clear cell renal cell carcinoma: results from the phase 3b/4 CheckMate 920 trial.
Tykodi SS, Gordan LN, Alter RS, Arrowsmith E, Harrison MR, Percent I, Singal R, Van Veldhuizen P, George DJ, Hutson T, Zhang J, Zoco J, Johansen JL, Rezazadeh Kalebasty A
J Immunother Cancer 2022 Feb;10(2) doi: 10.1136/jitc-2021-003844. PMID: 35210307Free PMC Article
Open-Label, Single-Arm, Phase II Study of Pembrolizumab Monotherapy as First-Line Therapy in Patients With Advanced Non-Clear Cell Renal Cell Carcinoma.
McDermott DF, Lee JL, Ziobro M, Suarez C, Langiewicz P, Matveev VB, Wiechno P, Gafanov RA, Tomczak P, Pouliot F, Donskov F, Alekseev BY, Shin SJ, Bjarnason GA, Castellano D, Silverman RK, Perini RF, Schloss C, Atkins MB
J Clin Oncol 2021 Mar 20;39(9):1029-1039. Epub 2021 Feb 2 doi: 10.1200/JCO.20.02365. PMID: 33529058Free PMC Article
Novel, emerging and provisional renal entities: The Genitourinary Pathology Society (GUPS) update on renal neoplasia.
Trpkov K, Williamson SR, Gill AJ, 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, Hes O
Mod Pathol 2021 Jun;34(6):1167-1184. Epub 2021 Feb 1 doi: 10.1038/s41379-021-00737-6. PMID: 33526874
Contemporary Characterization and Recategorization of Adult Unclassified Renal Cell Carcinoma.
Kwon R, Argani P, Epstein JI, Lombardo KA, Wang X, Pierorazio PM, Mehra R, Matoso A
Am J Surg Pathol 2021 Apr 1;45(4):450-462. doi: 10.1097/PAS.0000000000001629. PMID: 33239504

Diagnosis

Comprehensive Genomic Profiling of NF2-Mutated Kidney Tumors Reveals Potential Targets for Therapy.
Hacking SM, Pavlick D, Wang Y, Carneiro BA, Mullally M, Lu S, Canepa M, Bratslavsky G, Jacob J, Necchi A, Spiess PE, Wang L, Yakirevich E, Ross J
Oncologist 2023 Jul 5;28(7):e508-e519. doi: 10.1093/oncolo/oyad040. PMID: 36917021Free PMC Article
Novel, emerging and provisional renal entities: The Genitourinary Pathology Society (GUPS) update on renal neoplasia.
Trpkov K, Williamson SR, Gill AJ, 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, Hes O
Mod Pathol 2021 Jun;34(6):1167-1184. Epub 2021 Feb 1 doi: 10.1038/s41379-021-00737-6. PMID: 33526874
Renal cell carcinoma in children and adolescents: Single-center experience and literature review.
He M, Cai J, Zhu K, Gu W, Li M, Xiong J, Guan Z, Wang J, Shu Q
Medicine (Baltimore) 2021 Jan 15;100(2):e23717. doi: 10.1097/MD.0000000000023717. PMID: 33466124Free PMC Article
Immunophenotypic and pathologic heterogeneity of unclassified renal cell carcinoma: a study of 300 cases.
Akgul M, Cheng L
Hum Pathol 2020 Aug;102:70-78. Epub 2020 Jun 30 doi: 10.1016/j.humpath.2020.06.007. PMID: 32619439
Clinicopathological characterisation of renal cell carcinoma in young adults: a contemporary update and review of literature.
Abdulfatah E, Kennedy JM, Hafez K, Davenport MS, Xiao H, Weizer AZ, Palapattu GS, Morgan TM, Mannan R, Wang XM, Dhanasekaran SM, Kaffenberger SD, Spratt DE, Kunju L, Wu A, Lew M, Udager AM, Chinnaiyan AM, Mehra R
Histopathology 2020 May;76(6):875-887. Epub 2020 Apr 27 doi: 10.1111/his.14051. PMID: 31872452

Therapy

Advances in non-clear cell renal cell carcinoma management: From heterogeneous biology to treatment options.
Wilson NR, Acikgoz Y, Hasanov E
Int J Cancer 2024 Mar 15;154(6):947-961. Epub 2023 Oct 12 doi: 10.1002/ijc.34756. PMID: 37823185
Phase II study of nivolumab and salvage nivolumab/ipilimumab in treatment-naïve patients with advanced non-clear cell renal cell carcinoma (HCRN GU16-260-Cohort B).
Atkins MB, Jegede OA, Haas NB, McDermott DF, Bilen MA, Stein M, Sosman JA, Alter R, Plimack ER, Ornstein MC, Hurwitz M, Peace DJ, Signoretti S, Denize T, Cimadamore A, Wu CJ, Braun D, Einstein D, Catalano PJ, Hammers H
J Immunother Cancer 2023 Mar;11(3) doi: 10.1136/jitc-2022-004780. PMID: 36948504Free PMC Article
Phase II Trial of Cabozantinib Plus Nivolumab in Patients With Non-Clear-Cell Renal Cell Carcinoma and Genomic Correlates.
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
Safety and efficacy of nivolumab plus ipilimumab in patients with advanced non-clear cell renal cell carcinoma: results from the phase 3b/4 CheckMate 920 trial.
Tykodi SS, Gordan LN, Alter RS, Arrowsmith E, Harrison MR, Percent I, Singal R, Van Veldhuizen P, George DJ, Hutson T, Zhang J, Zoco J, Johansen JL, Rezazadeh Kalebasty A
J Immunother Cancer 2022 Feb;10(2) doi: 10.1136/jitc-2021-003844. PMID: 35210307Free PMC Article
Open-Label, Single-Arm, Phase II Study of Pembrolizumab Monotherapy as First-Line Therapy in Patients With Advanced Non-Clear Cell Renal Cell Carcinoma.
McDermott DF, Lee JL, Ziobro M, Suarez C, Langiewicz P, Matveev VB, Wiechno P, Gafanov RA, Tomczak P, Pouliot F, Donskov F, Alekseev BY, Shin SJ, Bjarnason GA, Castellano D, Silverman RK, Perini RF, Schloss C, Atkins MB
J Clin Oncol 2021 Mar 20;39(9):1029-1039. Epub 2021 Feb 2 doi: 10.1200/JCO.20.02365. PMID: 33529058Free PMC Article

Prognosis

Advances in non-clear cell renal cell carcinoma management: From heterogeneous biology to treatment options.
Wilson NR, Acikgoz Y, Hasanov E
Int J Cancer 2024 Mar 15;154(6):947-961. Epub 2023 Oct 12 doi: 10.1002/ijc.34756. PMID: 37823185
Phase II Trial of Cabozantinib Plus Nivolumab in Patients With Non-Clear-Cell Renal Cell Carcinoma and Genomic Correlates.
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
Open-Label, Single-Arm, Phase II Study of Pembrolizumab Monotherapy as First-Line Therapy in Patients With Advanced Non-Clear Cell Renal Cell Carcinoma.
McDermott DF, Lee JL, Ziobro M, Suarez C, Langiewicz P, Matveev VB, Wiechno P, Gafanov RA, Tomczak P, Pouliot F, Donskov F, Alekseev BY, Shin SJ, Bjarnason GA, Castellano D, Silverman RK, Perini RF, Schloss C, Atkins MB
J Clin Oncol 2021 Mar 20;39(9):1029-1039. Epub 2021 Feb 2 doi: 10.1200/JCO.20.02365. PMID: 33529058Free PMC Article
Novel, emerging and provisional renal entities: The Genitourinary Pathology Society (GUPS) update on renal neoplasia.
Trpkov K, Williamson SR, Gill AJ, 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, Hes O
Mod Pathol 2021 Jun;34(6):1167-1184. Epub 2021 Feb 1 doi: 10.1038/s41379-021-00737-6. PMID: 33526874
Contemporary Characterization and Recategorization of Adult Unclassified Renal Cell Carcinoma.
Kwon R, Argani P, Epstein JI, Lombardo KA, Wang X, Pierorazio PM, Mehra R, Matoso A
Am J Surg Pathol 2021 Apr 1;45(4):450-462. doi: 10.1097/PAS.0000000000001629. PMID: 33239504

Clinical prediction guides

Phase II Trial of Cabozantinib Plus Nivolumab in Patients With Non-Clear-Cell Renal Cell Carcinoma and Genomic Correlates.
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
Open-Label, Single-Arm, Phase II Study of Pembrolizumab Monotherapy as First-Line Therapy in Patients With Advanced Non-Clear Cell Renal Cell Carcinoma.
McDermott DF, Lee JL, Ziobro M, Suarez C, Langiewicz P, Matveev VB, Wiechno P, Gafanov RA, Tomczak P, Pouliot F, Donskov F, Alekseev BY, Shin SJ, Bjarnason GA, Castellano D, Silverman RK, Perini RF, Schloss C, Atkins MB
J Clin Oncol 2021 Mar 20;39(9):1029-1039. Epub 2021 Feb 2 doi: 10.1200/JCO.20.02365. PMID: 33529058Free PMC Article
Advanced Non-Clear Cell Kidney Cancer: In Search of Rational Treatment Approaches.
Ornstein MC, Hutson TE
Cancer J 2020 Sep/Oct;26(5):441-447. doi: 10.1097/PPO.0000000000000474. PMID: 32947312
Cabozantinib in advanced non-clear-cell renal cell carcinoma: a multicentre, retrospective, cohort study.
Martínez Chanzá N, Xie W, Asim Bilen M, Dzimitrowicz H, Burkart J, Geynisman DM, Balakrishnan A, Bowman IA, Jain R, Stadler W, Zakharia Y, Narayan V, Beuselinck B, McKay RR, Tripathi A, Pachynski R, Hahn AW, Hsu J, Shah SA, Lam ET, Rose TL, Mega AE, Vogelzang N, Harrison MR, Mortazavi A, Plimack ER, Vaishampayan U, Hammers H, George S, Haas N, Agarwal N, Pal SK, Srinivas S, Carneiro BA, Heng DYC, Bosse D, Choueiri TK, Harshman LC
Lancet Oncol 2019 Apr;20(4):581-590. Epub 2019 Feb 28 doi: 10.1016/S1470-2045(18)30907-0. PMID: 30827746Free PMC Article
Unclassified renal cell carcinoma: a report of 56 cases.
Lopez-Beltran A, Kirkali Z, Montironi R, Blanca A, Algaba F, Scarpelli M, Yorukoglu K, Hartmann A, Cheng L
BJU Int 2012 Sep;110(6):786-93. Epub 2012 Mar 8 doi: 10.1111/j.1464-410X.2012.10934.x. PMID: 22404824

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