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Sarcomatoid renal cell carcinoma

MedGen UID:
220411
•Concept ID:
C1266043
•
Neoplastic Process
Synonym: Sarcomatoid Renal Cell Carcinoma
SNOMED CT: Renal cell carcinoma, spindle cell (128668003); Renal cell carcinoma, sarcomatoid (128668003)
 
Monarch Initiative: MONDO:0003012

Definition

A high grade carcinoma of the kidney. It is not a distinct clinicopathological entity and includes a diverse group of renal cell carcinomas which have been transformed from a lower to a higher grade. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSarcomatoid renal cell carcinoma

Professional guidelines

PubMed

Efficacy and Safety of Nivolumab Plus Ipilimumab versus Sunitinib in First-line Treatment of Patients with Advanced Sarcomatoid Renal Cell Carcinoma.
Tannir NM, Signoretti S, Choueiri TK, McDermott DF, Motzer RJ, Flaifel A, Pignon JC, Ficial M, Frontera OA, George S, Powles T, Donskov F, Harrison MR, Barthélémy P, Tykodi SS, Kocsis J, Ravaud A, Rodriguez-Cid JR, Pal SK, Murad AM, Ishii Y, Saggi SS, McHenry MB, Rini BI
Clin Cancer Res 2021 Jan 1;27(1):78-86. Epub 2020 Sep 1 doi: 10.1158/1078-0432.CCR-20-2063. PMID: 32873572Free PMC Article
Epidemiology, biology and treatment of sarcomatoid RCC: current state of the art.
Lebacle C, Pooli A, Bessede T, Irani J, Pantuck AJ, Drakaki A
World J Urol 2019 Jan;37(1):115-123. Epub 2018 Jun 1 doi: 10.1007/s00345-018-2355-y. PMID: 29858701
Sarcomatoid renal cell carcinoma: Biology and treatment advances.
Mouallem NE, Smith SC, Paul AK
Urol Oncol 2018 Jun;36(6):265-271. Epub 2018 Jan 3 doi: 10.1016/j.urolonc.2017.12.012. PMID: 29306556

Recent clinical studies

Etiology

Sarcomatoid renal cell carcinoma: MRI features and their association with survival.
Cheng M, Duzgol C, Kim TH, Ghafoor S, Becker AS, Causa Andrieu PI, Gangai N, Jiang H, Hakimi AA, Vargas HA, Woo S
Cancer Imaging 2023 Feb 15;23(1):16. doi: 10.1186/s40644-023-00535-0. PMID: 36793052Free PMC Article
Long-term outcomes with nivolumab plus ipilimumab versus sunitinib in first-line treatment of patients with advanced sarcomatoid renal cell carcinoma.
Rini BI, Signoretti S, Choueiri TK, McDermott DF, Motzer RJ, George S, Powles T, Donskov F, Tykodi SS, Pal SK, Gupta S, Lee CW, Jiang R, Tannir NM
J Immunother Cancer 2022 Dec;10(12) doi: 10.1136/jitc-2022-005445. PMID: 36549781Free PMC Article
Immunotherapy in metastatic sarcomatoid renal cell carcinoma: A single institution experience.
Park JJ, Kellezi O, Hamasha R, Ali A, Alva AS
Cancer Treat Res Commun 2020;25:100251. Epub 2020 Nov 23 doi: 10.1016/j.ctarc.2020.100251. PMID: 33310369
Sarcomatoid Renal Cell Carcinoma: Population-Based Study of 879 Patients.
Alevizakos M, Gaitanidis A, Nasioudis D, Msaouel P, Appleman LJ
Clin Genitourin Cancer 2019 Jun;17(3):e447-e453. Epub 2019 Jan 17 doi: 10.1016/j.clgc.2019.01.005. PMID: 30799129
Sarcomatoid renal cell carcinoma: an examination of underlying histologic subtype and an analysis of associations with patient outcome.
Billis A
Int Braz J Urol 2004 Jul-Aug;30(4):347-8. PMID: 15679982

Diagnosis

Sarcomatoid renal cell carcinoma: biology, natural history and management.
Blum KA, Gupta S, Tickoo SK, Chan TA, Russo P, Motzer RJ, Karam JA, Hakimi AA
Nat Rev Urol 2020 Dec;17(12):659-678. Epub 2020 Oct 13 doi: 10.1038/s41585-020-00382-9. PMID: 33051619Free PMC Article
Sarcomatoid Renal Cell Carcinoma: Population-Based Study of 879 Patients.
Alevizakos M, Gaitanidis A, Nasioudis D, Msaouel P, Appleman LJ
Clin Genitourin Cancer 2019 Jun;17(3):e447-e453. Epub 2019 Jan 17 doi: 10.1016/j.clgc.2019.01.005. PMID: 30799129
Sarcomatoid renal cell carcinoma: a comprehensive review of the biology and current treatment strategies.
Shuch B, Bratslavsky G, Linehan WM, Srinivasan R
Oncologist 2012;17(1):46-54. Epub 2012 Jan 10 doi: 10.1634/theoncologist.2011-0227. PMID: 22234634Free PMC Article
Xanthogranulomatous pyelonephritis.
Li L, Parwani AV
Arch Pathol Lab Med 2011 May;135(5):671-4. doi: 10.5858/2009-0769-RSR.1. PMID: 21526966
Sarcomatoid renal cell carcinoma.
Akhtar K, Shamshad A, Sufian Z, Tariq M
Saudi J Kidney Dis Transpl 2011 Jan;22(1):120-2. PMID: 21196626

Therapy

Case 332.
Adler C, Menias C
Radiology 2024 Aug;312(2):e232149. doi: 10.1148/radiol.232149. PMID: 39189900
Long-term outcomes with nivolumab plus ipilimumab versus sunitinib in first-line treatment of patients with advanced sarcomatoid renal cell carcinoma.
Rini BI, Signoretti S, Choueiri TK, McDermott DF, Motzer RJ, George S, Powles T, Donskov F, Tykodi SS, Pal SK, Gupta S, Lee CW, Jiang R, Tannir NM
J Immunother Cancer 2022 Dec;10(12) doi: 10.1136/jitc-2022-005445. PMID: 36549781Free PMC Article
Sarcomatoid renal cell carcinoma: biology, natural history and management.
Blum KA, Gupta S, Tickoo SK, Chan TA, Russo P, Motzer RJ, Karam JA, Hakimi AA
Nat Rev Urol 2020 Dec;17(12):659-678. Epub 2020 Oct 13 doi: 10.1038/s41585-020-00382-9. PMID: 33051619Free PMC Article
Systemic therapy for sarcomatoid renal cell carcinoma.
Pagliaro LC, Tannir N, Sircar K, Jonasch E
Expert Rev Anticancer Ther 2011 Jun;11(6):913-20. doi: 10.1586/era.11.39. PMID: 21707288
Xanthogranulomatous pyelonephritis.
Li L, Parwani AV
Arch Pathol Lab Med 2011 May;135(5):671-4. doi: 10.5858/2009-0769-RSR.1. PMID: 21526966

Prognosis

Sarcomatoid renal cell carcinoma: MRI features and their association with survival.
Cheng M, Duzgol C, Kim TH, Ghafoor S, Becker AS, Causa Andrieu PI, Gangai N, Jiang H, Hakimi AA, Vargas HA, Woo S
Cancer Imaging 2023 Feb 15;23(1):16. doi: 10.1186/s40644-023-00535-0. PMID: 36793052Free PMC Article
Long-term outcomes with nivolumab plus ipilimumab versus sunitinib in first-line treatment of patients with advanced sarcomatoid renal cell carcinoma.
Rini BI, Signoretti S, Choueiri TK, McDermott DF, Motzer RJ, George S, Powles T, Donskov F, Tykodi SS, Pal SK, Gupta S, Lee CW, Jiang R, Tannir NM
J Immunother Cancer 2022 Dec;10(12) doi: 10.1136/jitc-2022-005445. PMID: 36549781Free PMC Article
Sarcomatoid renal cell carcinoma: biology, natural history and management.
Blum KA, Gupta S, Tickoo SK, Chan TA, Russo P, Motzer RJ, Karam JA, Hakimi AA
Nat Rev Urol 2020 Dec;17(12):659-678. Epub 2020 Oct 13 doi: 10.1038/s41585-020-00382-9. PMID: 33051619Free PMC Article
Sarcomatoid renal cell carcinoma: Biology and treatment advances.
Mouallem NE, Smith SC, Paul AK
Urol Oncol 2018 Jun;36(6):265-271. Epub 2018 Jan 3 doi: 10.1016/j.urolonc.2017.12.012. PMID: 29306556
Sarcomatoid renal cell carcinoma: a comprehensive review of the biology and current treatment strategies.
Shuch B, Bratslavsky G, Linehan WM, Srinivasan R
Oncologist 2012;17(1):46-54. Epub 2012 Jan 10 doi: 10.1634/theoncologist.2011-0227. PMID: 22234634Free PMC Article

Clinical prediction guides

Multiregion sequencing of sarcomatoid renal cell carcinoma arising from autosomal dominant polycystic kidney disease.
Lee E, Guan P, Lim AH, Loh JW, Tan GF, Loh T, Ng DYX, Lee JY, Goh S, Liu W, Ng CC, Teh BT, Chan JY
Mol Genet Genomic Med 2022 Mar;10(3):e1853. Epub 2022 Feb 5 doi: 10.1002/mgg3.1853. PMID: 35122417Free PMC Article
Efficacy and correlative analyses of avelumab plus axitinib versus sunitinib in sarcomatoid renal cell carcinoma: post hoc analysis of a randomized clinical trial.
Choueiri TK, Larkin J, Pal S, Motzer RJ, Rini BI, Venugopal B, Alekseev B, Miyake H, Gravis G, Bilen MA, Hariharan S, Chudnovsky A, Ching KA, Mu XJ, Mariani M, Robbins PB, Huang B, di Pietro A, Albiges L
ESMO Open 2021 Jun;6(3):100101. Epub 2021 Apr 23 doi: 10.1016/j.esmoop.2021.100101. PMID: 33901870Free PMC Article
Sarcomatoid Renal Cell Carcinoma Has a Distinct Molecular Pathogenesis, Driver Mutation Profile, and Transcriptional Landscape.
Wang Z, Kim TB, Peng B, Karam J, Creighton C, Joon A, Kawakami F, Trevisan P, Jonasch E, Chow CW, Canales JR, Tamboli P, Tannir N, Wood C, Monzon F, Baggerly K, Varella-Garcia M, Czerniak B, Wistuba I, Mills G, Shaw K, Chen K, Sircar K
Clin Cancer Res 2017 Nov 1;23(21):6686-6696. Epub 2017 Jul 14 doi: 10.1158/1078-0432.CCR-17-1057. PMID: 28710314Free PMC Article
Comparison of oncologic outcomes between sarcomatoid and clear cell renal cell carcinoma.
Trudeau V, Larcher A, Sun M, Boehm K, Dell'Oglio P, Sosa J, Tian Z, Fossati N, Briganti A, Shariat SF, Karakiewicz PI
World J Urol 2016 Oct;34(10):1429-36. Epub 2016 Feb 22 doi: 10.1007/s00345-016-1780-z. PMID: 26902877
Sarcomatoid renal cell carcinoma: clinical outcome and survival after treatment with sunitinib.
Kunene V, Miscoria M, Pirrie S, Islam MR, Afshar M, Porfiri E
Clin Genitourin Cancer 2014 Aug;12(4):251-5. Epub 2013 Dec 27 doi: 10.1016/j.clgc.2013.12.001. PMID: 24560086

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