Subtype
Renal Cell Carcinoma
CI-CAN-00006578RCCExplore in graph →
Loading cancer entity…
Subtype
CI-CAN-00006578RCCExplore in graph →
Variants & evidence
167 evidence items mapped to this entity or its descendants, grouped by molecular profile, then therapy. 50 items per page.
| Therapy | Cancer | Type | Level | Direction · significance | Rating (1–5) | Status | Evidence | Source |
|---|---|---|---|---|---|---|---|---|
| ARID1A Underexpression1 | ||||||||
| (prognostic) | Clear Cell Renal Cell Carcinoma | Prognostic | B | Supports Poor Outcome | 3 | accepted | EID5676This study included 290 patients with Clear Cell Renal Cell Carcinoma whose tumor tissues were sampled and then analyzed. These samples were then divided according to ARID1A expression after immunohis… (full text at CIViC) PMID 25628030 · Park et al., 2015 · Open in CIViC | civic |
| TFE3 Fusion2 | ||||||||
| (diagnostic) | Renal Cell Carcinoma with MiT Translocations | Diagnostic | B | Supports Positive | 3 | rejected | EID11529Argani, et al. (2001) included eight morphologically distinctive renal cell carcinomas with the t(X;17) translocation as seen in alveolar soft part sarcoma (ASPS), with the distinction that the t(X;17… (full text at CIViC) PMID 28766250 · Ju et al., 2018 · Open in CIViC | civic |
| 〃 | Renal Cell Carcinoma with MiT Translocations | Diagnostic | B | Supports Positive | 3 | accepted | EID11532Argani, et al. (2001) included eight morphologically distinctive renal cell carcinomas with the t(X;17) translocation as seen in alveolar soft part sarcoma (ASPS), with the distinction that the t(X;17… (full text at CIViC) PMID 11438465 · Argani et al., 2001 · Open in CIViC | civic |
| B4GALT1 Overexpression1 | ||||||||
| (prognostic) | Renal Cell CarcinomaCURATED_BROADER | Prognostic | B | Supports Poor Outcome | 3 | accepted | EID1278438 patients with non-metastatic clear-cell renal carcinoma were were retrospectively analysed for B4GALT1 expression via IHC. High B4GALT1 expression was associated with poor overall survival (P<0.00… (full text at CIViC) PMID 27092876 · Xie et al., 2016 · Open in CIViC | civic |
| BAP1 Mutation4 | ||||||||
| (prognostic) | Clear Cell Renal Cell Carcinoma | Prognostic | B | Supports Poor Outcome | 5 | accepted | EID459In patients with clear cell renal cell carcinoma, in a multivariate analysis those with mutations in BAP1 had worse overall survival than wild-type. PMID 23797736 · Sato et al., 2013 · Open in CIViC | civic |
| 〃 | Renal Cell CarcinomaCURATED_BROADER | Prognostic | B | Supports Poor Outcome | 5 | accepted | EID460In patients with clear cell renal cell carcinoma, in a multivariate analysis those with mutations in BAP1 had worse overall survival than wild-type. PMID 23797736 · Sato et al., 2013 · Open in CIViC | civic |
| 〃 | Clear Cell Renal Cell Carcinoma | Prognostic | B | Supports Poor Outcome | 5 | accepted | ||
| BAP1 Q267fs1 | ||||||||
| Niraparib | Clear Cell Renal Cell Carcinoma | Predictive | C | Supports Sensitivity Response | 1 | submitted | EID11199A patient with Clear cell renal cell carcinoma previously treated with sorafenib, axitinib, and everolimus was found to harbor a BAP1 frameshift mutation described as c.799_800del (p.Q267fs). Based o… (full text at CIViC) PMID 35875073 · Lian et al., 2022 · Open in CIViC | civic |
| BRD4 Overexpression1 | ||||||||
| JQ1 | Clear Cell Renal Cell Carcinoma | Predictive | D | Supports Sensitivity Response | 2 | submitted | EID2957Renal cell carcinoma (RCC) remains an intractable genitourinary malignancy. Resistance to chemotherapy or targeted therapies in RCC is presumably due to the complicated underlying molecular mechanisms… (full text at CIViC) PMID 28391274 · Wu et al., 2017 · Open in CIViC | civic |
| CCND1 Amplification1 | ||||||||
| Palbociclib | Renal Cell CarcinomaCURATED_BROADER | Predictive | D | Does Not Support Sensitivity Response | 2 | accepted | EID156028 renal cell carcinoma cell lines were tested for sensitivity to CDK4/6 inhibitor Palbociclib (PD0332991). 19 were reported sensitive, and 9 resistant. 15 of the 28 cell lines had high levels of Cycl… (full text at CIViC) PMID 23898052 · Logan et al., 2013 · Open in CIViC | civic |
| CDKN2A Loss1 | ||||||||
| Palbociclib | Renal Cell CarcinomaCURATED_BROADER | Predictive | D | Supports Sensitivity Response | 3 | accepted | EID1373Preclinical study of CDK4/6 inhibitor PD-0332991 in 28 renal carcinoma cell lines (25 malignant RCC and three non-malignant, transformed cell lines derived from kidney epithelium). Both p15 and p16 lo… (full text at CIViC) PMID 23898052 · Logan et al., 2013 · Open in CIViC | civic |
| CDKN2B Loss1 | ||||||||
| Palbociclib | Renal Cell CarcinomaCURATED_BROADER | Predictive | D | Supports Sensitivity Response | 3 | accepted | EID1374Preclinical study of CDK4/6 inhibitor PD-0332991 in 28 renal carcinoma cell lines (25 malignant RCC and three non-malignant, transformed cell lines derived from kidney epithelium). Both p15 and p16 lo… (full text at CIViC) PMID 23898052 · Logan et al., 2013 · Open in CIViC | civic |
| ALK e2::e201 | ||||||||
| Unspecified therapy | Renal Cell CarcinomaCURATED_BROADER | Predictive | E | Supports Sensitivity Response | 3 | accepted | EID1266Adenocarcinoma cells were detected via urinary cytology in a 53 year old woman. Cancer morphology suggested papillary renal cell carcinoma (RCC), type 2A. EML4-ALK variant 5 was discovered in tumor t… (full text at CIViC) PMID 22252991 · Sugawara et al., 2012 · Open in CIViC | civic |
| EPAS1 Overexpression1 | ||||||||
| Pazopanib | Renal Cell CarcinomaCURATED_BROADER | Predictive | B | Does Not Support Sensitivity Response | 2 | accepted | EID1673HIF2A (EPAS1) staining was conducted on tumors from 66 patients, of which 27 were classified as low expression and 39 were classified as high expression. The response rates of patients with low and hi… (full text at CIViC) PMID 23881929 · Choueiri et al., 2013 · Open in CIViC | civic |
| FBXW7 Loss-of-function1 | ||||||||
| Everolimus | Renal Cell CarcinomaCURATED_BROADER | Predictive | E | Supports Sensitivity Response | 2 | accepted | EID1628mTOR protein levels were found to underly a circadian 24-hour rhythm regulated by time-dependent Fbxw7-mediated ubiquitination. Survival rates of tumor-bearing mice with everolimus showed time-depende… (full text at CIViC) PMID 24253377 · Okazaki et al., 2014 · Open in CIViC | civic |
| FGFR2 Overexpression1 | ||||||||
| (prognostic) | Papillary Renal Cell Carcinoma | Prognostic | B | Supports Poor Outcome | 4 | submitted | EID6454Only 12/118(10.2%) of patients with type I papillary renal cell carcinoma expressed FGFR2 whereas 37/96 (38.5%) patients with type II papillary renal cell carcinoma(P=0.001). In addition, FGFR2 expre… (full text at CIViC) PMID 27379982 · Tsimafeyeu et al., 2017 · Open in CIViC | civic |
| FH Deficient2 | ||||||||
| Bevacizumab + ErlotinibCombination | Hereditary Renal Cell Carcinoma | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID12939In the open‑label phase II AVATAR trial (NCT01130519), patients with HLRCC‑associated papillary RCC harboring germline FH pathogenic variants (n = 43) received erlotinib plus bevacizumab, with ORR as … (full text at CIViC) PMID 40532152 · Srinivasan et al., 2025 · Open in CIViC | civic |
| 〃 | Papillary Renal Cell Carcinoma | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID12940In the open‑label phase II AVATAR trial (NCT01130519), patients with sporadic papillary RCC (n = 40) received erlotinib plus bevacizumab, with ORR as the primary endpoint. They demonstrated a lower OR… (full text at CIViC) PMID 40532152 · Srinivasan et al., 2025 · Open in CIViC | civic |
| FLCN c.1285dupC1 | ||||||||
| Everolimus | Renal Cell CarcinomaCURATED_BROADER | Predictive | C | Supports Sensitivity Response | 1 | accepted | EID10137A case study showed that the mTOR inhibitor everolimus exhibited a relatively long-term effect in a patient with renal cell carcinoma and a FLCN germline mutation (c. 1285dupC, likely loss of function… (full text at CIViC) PMID 23995526 · Nakamura et al., 2013 · Open in CIViC | civic |
| FLCN Mutation1 | ||||||||
| Everolimus | Papillary Renal Cell Carcinoma | Predictive | C | Supports Sensitivity Response | 3 | submitted | EID5990In a patient with Birt-Hogg-Dubé syndrome-associated papillary renal cell carcinoma use of everolimus as sixth-line systematic treatment exhibited a relatively long-term effect as compared with the pr… (full text at CIViC) PMID 23995526 · Nakamura et al., 2013 · Open in CIViC | civic |
| FLT3 T227M1 | ||||||||
| Sunitinib | Renal Cell CarcinomaCURATED_BROADER | Predictive | B | Supports Adverse Response | 3 | accepted | EID1317In 219 patients with mostly Renal Cell Carcinoma and some Gastrointestinal Stromal tumors given sunitinib treatment, FLT3 T227M (rs1933437) was associated with the development of leukopenia. 95% confi… (full text at CIViC) PMID 19667267 · van Erp et al., 2009 · Open in CIViC | civic |
| GNAS c.393T>C1 | ||||||||
| (prognostic) | Clear Cell Renal Cell Carcinoma | Prognostic | B | Supports Better Outcome | 4 | accepted | EID2896In this study, Kaplan-Meier curves for tumor progression, development of metastasis, and tumor-related death showed a significant association of the T393C polymorphism with outcome (5-year cancer-spec… (full text at CIViC) PMID 16467086 · Frey et al., 2006 · Open in CIViC | civic |
| HDAC2 Expression1 | ||||||||
| Abexinostat + PazopanibCombination | Renal Cell CarcinomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 5 | submitted | EID7057In this phase Ib study (NCT01543763), the histone deacetylase (HDAC) inhibitor abexinostat was used in combination with the multi-tyrosine kinase inhibitor pazopanib to evaluate the effect of abexinos… (full text at CIViC) PMID 28221861 · Aggarwal et al., 2017 · Open in CIViC | civic |
| HIF1A EXPRESSION1 | ||||||||
| Pazopanib | Renal Cell CarcinomaCURATED_BROADER | Predictive | B | Does Not Support Sensitivity Response | 2 | accepted | EID1674Immunohistochemical staining for HIF1A was performed on tumor samples from 65 patients that received pazopanib as part of VEG102616, of which 17 were classified as low expression and 48 were classifie… (full text at CIViC) PMID 23881929 · Choueiri et al., 2013 · Open in CIViC | civic |
| HMOX1 EXPRESSION1 | ||||||||
| Sorafenib + SunitinibSubstitutes | Renal Cell CarcinomaCURATED_BROADER | Predictive | B | Supports Resistance | 3 | accepted | EID829In 66 patients with advanced renal cell carcinoma, high expression of heme oxygenase-1 (HO-1) was associated with poor overall response rate (2.6% versus 53.6%, P<0.01), clinical benefit rate (47.4% v… (full text at CIViC) PMID 26309414 · Zheng et al., 2015 · Open in CIViC | civic |
| KDM5C Mutation1 | ||||||||
| Everolimus + SunitinibSequential | Renal Cell CarcinomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID5997This study evaluated the relationship between tumor mutations and treatment outcomes in the RECORD-3 clinical trial. With first-line everolimus, PBRM1 and BAP1 mutations were associated with longer (m… (full text at CIViC) PMID 27751729 · Hsieh et al., 2017 · Open in CIViC | civic |
| KLLN PROMOTER METHYLATION1 | ||||||||
| (predisposing) | Renal Cell CarcinomaCURATED_BROADER | Predisposing | B | Supports Predisposition | 3 | accepted | EID1762123 patients with Cowden or Cowden-like syndrome (from the IRB8458-PTEN series) and 50 unaffected individuals without PTEN variants were analyzed for germline methylation and expression of PTEN and KI… (full text at CIViC) PMID 21177507 · Bennett et al., 2010 · Open in CIViC | civic |
| MET Mutation1 | ||||||||
| Foretinib | Papillary Renal Cell Carcinoma | Predictive | B | Supports Sensitivity Response | 3 | accepted | EID796In a retrospective analysis of 74 patients with papillary renal cell carcinoma, treated with foretinib, MET germline mutation was associated with improved response rate. A response was observed in 5 … (full text at CIViC) PMID 23213094 · Choueiri et al., 2013 · Open in CIViC | civic |
| MTOR Gain-of-Function1 | ||||||||
| MTOR Inhibitor | Renal Cell CarcinomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID5839In a retrospective study of 79 RCC patients treated with diverse rapalogs (mTOR inhibitors) inactivating TSC1 and TSC2-mutations and activating MTOR-mutations were significantly more common in respons… (full text at CIViC) PMID 26831717 · Kwiatkowski et al., 2016 · Open in CIViC | civic |
| MTOR Q2223K1 | ||||||||
| Temsirolimus | Clear Cell Renal Cell Carcinoma | Predictive | D | Supports Sensitivity Response | — | submitted | EID4522In an in vitro study, HEK293T cells expressing MTOR Q2223K, demonstrated sensitivity to temsirolimus treatment, comparable to HEK293T cells expressing wild-type MTOR. Sensitivity was determined by ass… (full text at CIViC) PMID 24622468 · Voss et al., 2014 · Open in CIViC | civic |
| PBRM1 Loss-of-function4 | ||||||||
| Atezolizumab + DurvalumabSubstitutes | Renal Cell CarcinomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID7537Among 35 patients with clear cell RCC, loss-of-function mutation of PBRM1 were correlated with response to anti-PD-1 monotherapy (P = 0.012). It was confirmed in an independent validation cohort of 63… (full text at CIViC) PMID 29301960 · Miao et al., 2018 · Open in CIViC | civic |
| Ipilimumab | Renal Cell CarcinomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID7301Among 35 patients with clear cell RCC, loss-of-function mutation of PBRM1 were correlated with response to anti-PD-1 monotherapy (P = 0.012). It was confirmed in an independent validation cohort of 6… (full text at CIViC) PMID 29301960 · Miao et al., 2018 · Open in CIViC | civic |
| Nivolumab | ||||||||
| PBRM1 Mutation4 | ||||||||
| (prognostic) | Clear Cell Renal Cell Carcinoma | Prognostic | B | Supports N/A | 3 | accepted | EID420In renal clear cell carcinoma, patients with PBRM1 mutations did not have a significantly different cancer specific survival. PMID 23620406 · Hakimi et al., 2013 · Open in CIViC | civic |
| 〃 | Renal Cell CarcinomaCURATED_BROADER | Prognostic | B | Supports Better Outcome | 3 | accepted | EID499In patients with sporatic clear cell renal cell carcinoma, those with mutations in PBRM1 had better overall survival than patients with BAP1 mutations. PMID 23333114 · Kapur et al., 2013 · Open in CIViC | civic |
| Anti-PD-L1 Monoclonal Antibody + CTLA-4 InhibitorSubstitutes | Clear Cell Renal Cell Carcinoma | Predictive | B | Supports | ||||
| SDHB Mutation1 | ||||||||
| Metformin + VandetanibCombination | Hereditary Renal Cell Carcinoma | Predictive | B | Supports Sensitivity Response | 2 | submitted | EID7959Vandetanib in Combination With Metformin is tested in Phase II trial in People With HLRCC or SDH-Associated Kidney Cancer or Sporadic Papillary Renal Cell Carcinoma PMID 27325049 · Sidana et al., 2016 · Open in CIViC | civic |
| SETD2 Loss2 | ||||||||
| Anti-PD1 Monoclonal Antibody + ATR InhibitorCombination | Clear Cell Renal Cell Carcinoma | Predictive | D | Supports Sensitivity Response | 2 | submitted | EID12580In preclinical RCC models (n ≈ 5/group), Setd2 loss enhanced sensitivity to the ATR inhibitor VE822 (60 mg/kg orally, 5 days/week) and anti–PD-1 antibody (100 µg intraperitoneally, every 3 days) reduc… (full text at CIViC) PMID 37527013 · Liu et al., 2023 · Open in CIViC | civic |
| Akt Inhibitor MK2206 + PI3Kbeta Inhibitor AZD8186 + TGX221Substitutes | Clear Cell Renal Cell Carcinoma | Predictive | D | Supports Sensitivity Response | 2 | submitted | EID12581In clear cell RCC, SETD2 loss predicts sensitivity to PI3Kβ pathway inhibition: SETD2-deficient 786-0 and SETD2-mutant A498 cells treated with PI3Kβ inhibitors TGX221 or AZD8186 showed reduced viabili… (full text at CIViC) PMID 31695846 · Terzo et al., 2019 · Open in CIViC | civic |
| SMARCB1 Loss2 | ||||||||
| (diagnostic) | Renal Medullary Carcinoma | Diagnostic | B | Supports Positive | 2 | accepted | EID11165In a retrospective analysis of 20 patients with renal medullary carcinoma, all patients had loss of SMARCB1 staining by IHC. FISH revealed 11 (55%) had a SMARCB1 translocation with loss of the second… (full text at CIViC) PMID 30980040 · Jia et al., 2019 · Open in CIViC | civic |
| 〃 | Renal Medullary Carcinoma | Diagnostic | B | Supports Positive | 2 | accepted | EID11167In a comparison of 6 renal medullary carcinomas (RMC) and 22 collecting duct carcinomas (CDC), all 6 RMC were negative for SMARCB1 by IHC whereas only 1 of 22 CDC lost SMARCB1 expressing, supporting S… (full text at CIViC) PMID 22686875 · Calderaro et al., 2012 · Open in CIViC | civic |
| TERT Overexpression1 | ||||||||
| UO126 | Clear Cell Renal Cell Carcinoma | Predictive | D | Supports Sensitivity Response | 3 | submitted | EID2900A total of 96 histopathologically confirmed RCC cases and corresponding normal tissues were subjected to hTERT gene expression using real-time PCR. Two RCC cell lines viz. ACHN and A498 were treated w… (full text at CIViC) PMID 28215740 · Pal et al., 2017 · Open in CIViC | civic |
| TFE3 VCP::TFE31 | ||||||||
| (prognostic) | Renal Cell CarcinomaCURATED_BROADER | Prognostic | B | Supports Poor Outcome | 4 | rejected | EID10148This study evaluates 200 nephrectomy cases. Within the 200 cases, there are four cases that are Xp11.2 translocation renal cell carcinomas (RCC). Previous studies identified two stages (pediatric and … (full text at CIViC) PMID 34917511 · Ge et al., 2021 · Open in CIViC | civic |
| TFEB Amplification1 | ||||||||
| (prognostic) | Renal Cell Carcinoma with MiT Translocations | Prognostic | B | Supports Poor Outcome | 4 | submitted | EID10625This manuscript is a study of 37 TFEB-altered tumors, 15 of which are associated with TFEB amplification at 6p21.1 and 22 of which are associated with TFEB-rearrangement. Previous literature had indic… (full text at CIViC) PMID 31600176 · Gupta et al., 2019 · Open in CIViC | civic |
| TSC1 Loss-of-function2 | ||||||||
| MTOR Inhibitor | Renal Cell CarcinomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID5837In a retrospective study of 79 RCC patients treated with diverse rapalogs (mTOR inhibitors) inactivating TSC1 and TSC2-mutations and activating MTOR-mutations were significantly more common in respons… (full text at CIViC) PMID 26831717 · Kwiatkowski et al., 2016 · Open in CIViC | civic |
| 〃 | Renal Cell CarcinomaCURATED_BROADER | Predictive | C | Supports Sensitivity Response | 2 | submitted | EID6053In 5 patients with renal cell carcinoma who achieved durable disease control with mTOR inhibitors (median duration, 28 months), Custom target capture and ultradeep sequencing was used to identify alt… (full text at CIViC) PMID 24622468 · Voss et al., 2014 · Open in CIViC | civic |
| TSC2 Loss-of-function1 | ||||||||
| MTOR Inhibitor | Renal Cell CarcinomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID5838In a retrospective study of 79 RCC patients treated with diverse rapalogs (mTOR inhibitors) inactivating TSC1 and TSC2-mutations and activating MTOR-mutations were significantly more common in respons… (full text at CIViC) PMID 26831717 · Kwiatkowski et al., 2016 · Open in CIViC | civic |
Data updated 21 hours agoSource updated unknownsource: civic (CC0)
Evidence levels, directions and ratings are those assigned by CIViC curators. "Submitted" items have not completed curation review. This is not treatment guidance.
EID461In patients with clear cell renal cell carcinoma, those with nonsilent mutations of BAP1 had worse overall survival than wild-type. PMID 23792563 · 2013, Nature · Open in CIViC |
| civic |
| Everolimus + SunitinibSequential | Clear Cell Renal Cell Carcinoma | Predictive | B | Supports Resistance | 4 | accepted | EID5339This study evaluated the effects of everolimus and sunitinib on patients with metastatic renal cell carcinoma. A total of 220 patients with RCC were evaluated for 341 genes at 540x coverage. Of all 22… (full text at CIViC) PMID 27751729 · Hsieh et al., 2017 · Open in CIViC | civic |
| Renal Cell CarcinomaCURATED_BROADER |
| Predictive |
| B |
| Supports Sensitivity Response |
| 4 |
| submitted |
EID7584In post hoc analysis in phase 3 trial, which compared nivolumab and everolimus in patients with clear cell renal cell carcinoma who received prior antiangiogenic therapy, PBRM1 mutations were identifi… (full text at CIViC) PMID 31486842 · Braun et al., 2019 · Open in CIViC |
| civic |
| Nivolumab + PembrolizumabSubstitutes | Renal Cell CarcinomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID7536Among 35 patients with clear cell RCC, loss-of-function mutation of PBRM1 were correlated with response to anti-PD-1 monotherapy (P = 0.012). It was confirmed in an independent validation cohort of 63… (full text at CIViC) PMID 29301960 · Miao et al., 2018 · Open in CIViC | civic |
| 4 |
| submitted |
EID7247Clinical benefit to immune checkpoint blockade was associated with loss-of-function mutations in the PBRM1 gene (P = 0.012), which encodes a subunit of the PBAF switch-sucrose nonfermentable (SWI/SNF)… (full text at CIViC) PMID 29301960 · Miao et al., 2018 · Open in CIViC |
| civic |
| Everolimus + SunitinibSequential | Renal Cell CarcinomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID5996This study evaluated the relationship between tumor mutations and treatment outcomes in the RECORD-3 clinical trial. With first-line everolimus, PBRM1 and BAP1 mutations were associated with longer (m… (full text at CIViC) PMID 27751729 · Hsieh et al., 2017 · Open in CIViC | civic |