Clinical Trials in non-clear cell RCC: ESPN, ASPEN and beyond

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Clinical Trials in non-clear cell RCC: ESPN, ASPEN and beyond Toni K. Choueiri, MD Clinical Director and Kidney Cancer Center Director The Lank Center for Genitourinary Oncology Dana-Farber Cancer Institute Brigham and Women s Hospital Associate Professor of Medicine Harvard Medical School

NCCN & ESMO Guidelines - US and EU guidelines for kidney cancer list clinical trial as the preferred option for patients with advanced non-clear cell RCC tfirst-line Non-clear cell NCCN guidelines Version 1. 2015 Clinical Trial ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2012

Non-clear cell RCC clinical trials: VEGF and mtor inhibitors: single agent Phase II data Tannir Lee Koh Ravaud (SUPAP) Escudier (RAPTOR) N 57 31 49 60 92 Histology Non-clear cell Non-clear cell Non-clear cell Papillary (I:15/II:45) Papillary 16/58 (25?) Agent sunitinib sunitinib everolimus sunitinib everolimus Previously treated ORR (RECIST) PFS (months) OS (months) Yes (no prior VEGF TT) Yes (46% chemo/imm) Yes (47% prior VEGF TT) 5% 36% 10% 12% NA 2.7 6.4 5.2 5.6 7.3 3.7 (central) 16.8 25.6 14 12.5 21-28 (type I) -20.3 (type II) No No 1. Tannir et al, Eur Urol 2012. 2. Lee et al, Ann Oncol 2012. 3. Koh et al, Ann Oncol 2013. 4. Ravaud et al, ESMO 2012. 5. Escudier et al, GU Cancer Symposium 2014

Cumulative event-free probability (%) Cumulative event-free probability (%) First-line PFS by Histology (RECORD-3) ASCO 2013 and JCO 2013 Clear Cell mrcc (N=290) Non-Clear Cell mrcc (N=66) 100 90 80 70 60 50 40 30 20 10 0 Everolimus 205 Sunitinib 197 0 3 6 9 12 15 18 21 24 27 30 33 Number of patients still at risk 145 153 107 127 80 94 62 72 Time (months) 38 49 26 38 Everolimus (n/n = 156/205) Sunitinib (n/n = 134/197) 100 90 80 70 60 50 40 30 20 10 0 Everolimus Sunitinib *Please note, this is a post-hoc exploratory sub-group analysis 19 26 10 14 3 8 0 3 0 0 Everolimus (n/n = 24/31) Sunitinib (n/n = 23/35) 0 3 6 9 12 15 18 21 24 27 30 33 Time (months) Number of patients still at risk 31 35 18 27 10 17 7 14 5 12 5 6 5 4 4 2 2 1 2 1 0 0 0 0 Kaplan-Meier Median PFS (mo) Everolimus Sunitinib 8.08 10.84 Hazard Ratio = 1.39 Two-sided 95% CI [1.10, 1.75] Kaplan-Meier Median PFS (mo) Everolimus Sunitinib 5.09 7.23 Hazard Ratio = 1.54 Two-sided 95% CI [0.86, 2.75 ]

ASCO 2014 Everolimus versus Sunitinib Prospective Evaluation in Metastatic Non-clear Cell Renal Cell Carcinoma (The ESPN Trial): A Randomized Multicenter Phase 2 Trial Nizar M. Tannir, Eric Jonasch, Emre Altinmakas, Chaan S. Ng, Wei Qiao, Pheroze Tamboli, Priya Rao, David F. McDermott, Christopher G. Wood, and Toni K. Choueiri The University of Texas MD Anderson Cancer Center, Houston, TX; Beth Israel Deaconess Medical Center, Boston, MA; Dana-Farber Cancer Institute, Boston, MA

Renal Cell Carcinoma #4505 Tannir Everolimus vs. sunitinib in metastatic non-clear cell RCC Advanced nccrcc, including all histologies -ECOG PS 0-1 -No prior systemic therapy Primary Endpoint : PFS in first-line (1L) N = 108 planned, 73 enrolled, stopped early by DSMC (at first interim analysis) due to OS favoring sunitinib R A N D O M I Z E Everolimus 10 mg PO QD Crossover at progression Sunitinib 50 mg QD (4 wk on/2 wk off) Everolimus, n=35 Sunitinib, n=33 P-value ORR 1 st line 2.8% 6% mpfs 1 st line (months) 4.1 6.1 0.6 Nb of pts receiving 2 nd line 20 24 mos (months) -mos (non-sarcomatoid), n=49 14.9 10.5 16.2 31.6 0.18 0.07 Highlights of the Day - Genitourinary (Non prostate) Cancer T.K. Choueiri

OS (Exploratory Analysis) by Histology Subtype Everolimus Median, mos (95% CI) Sunitinib Median, mos (95% CI) Papillary 14.9 (7.1, 22.7) n=13 16.6 (5.9, NA) n=14 Chromophobe 25.1(4.7, NA) n=6 31.6 (14.2, NA) n=6 Unclassified NA n=6 15.4 (NA) n=4 Translocation 8.1 (5.5, 23) n=4 16.2 (8.8, NA) n=3 Sarcomatoid w/ clear-cell 11.1 (2.0, NA) n=6 7.0 (5.4, 10.4) n=6

ps6k pvefr2 pmtor AURKA PTEN

Phase II Study of Everolimus vs Sunitinib: Mixed Sub-type nccrcc (ASPEN) n = 108 Key inclusion criteria Papillary or chromophobe nccrcc KPS 60 Key exclusion criteria Collecting duct, medullary, small cell, oncocytoma, or lymphoma-type pathology Prior systemic therapy for mrcc R A N D O M I Z E Everolimus 10 mg QD on days 1 42 of each 42-day cycle Sunitinib 50 mg QD on days 1 28 of each 42-day cycle Treat until disease progression, unacceptable toxicity, or withdrawal of consent This study is ongoing, but not recruiting participants. Patients will be stratified by histology and MSKCC risk criteria Primary end point: PFS Secondary end points: tolerability, QoL ClinicalTrials.gov identifier: NCT01108445.

MET INHIBITORS?

Best percentage change from baseline Foretinib (VEGFR/MET inhibitor) Tumor Shrinkage in 50 out of 68 patients Germline Mutations 100 90 80 70 60 50 40 30 20 10 0-10 -20-30 -40-50 -60-70 -80-90 * MET aberration (MET amplification, trisomy 7 or somatic mutation; excluding germline mutations) Tested, but no germline mutation or MET aberrations Confirmed PR=13.5% (all cohort) * ** ** **** * Choueiri et al, JCO 2013

AZD6094 (Volitinib) Scientific Background Papillary renal cell cancer (PRCC) AZD6094 is a selective small molecule, highly potent inhibitor of cmet AZD6094 has an IC50 of 4 nm & >650 fold selectivity demonstrated vs 265 other kinases AZD6094 has shown preclinical activity as monotherapy in cell lines & xenograft models (e.g. PRCC, gastrointestinal and lung cancers) Ph1 monotherapy clinical activity has been reported in PRCC patients cmet receptor HGF rilotumumab onartuzumab RAF MEK MAPK R as Cell proliferation S O S AZD6094 crizotinib cabozantinib GAB1 F a k Cell invasion PI 3K AKT mtor Cell survival

GU111 Trial Design (accruing) Phase II, open-label, single-arm Patients will take AZD6094 600mg PO QD (21 day= 1cycle) Biopsy distant metastatic site highly encouraged MET pathways analyses (Tumor/germline) Stage 1: Approximately 20 patients Stage 2: (will occur if 6 tumor responses are observed in Stage 1) Approximately 55 patients Cancer.gov/NCT 02127710

Planned cooperative Group Trials with papillary RCC SWOG 14XX (PI: PAL/LARA) Courtesy of Monty Pal (SWOG)

Combo EGFR/MET Inhibitor--SWOG 1107 Erlotinib: ORR 11% (Gordon, JCO 2009) mrcc Papillary mrcc ECOG 0-1 0-1 prior therapies No prior MET inhibitor or erlotinib Erlotinib + ARQ197 ARQ197 Primary Endpoint RR Secondary Endpoint PFS OS Toxicity Courtesy of Monty Pal (SWOG)

Immune Checkpoint Blockade in nonclear cell RCC

17 MPDL3280A: mrcc Baseline Characteristics Safety-evaluable population with RCC in Phase I expansion Characteristics All Patients, N = 69 Median age (range), y 61 (33-81) Male 77% ECOG PS 0 / 1 54% / 46% Histologic subtypes, n (%) Clear cell 62 (90%) Non-clear cell 7 (10%) Fuhrman grade 4 or with sarcomatoid histology 20 (29%) MSKCC poor risk, n (%) 18 (26%) Prior nephrectomy, n (%) 66 (96%) Previous systemic therapies, n (%) 60 (87%) Cytokine-based 27 (39%) Tyrosine kinase inhibitor 40 (58%) mtor inhibitor 17 (25%) Lung / liver / bone / brain metastases at enrollment, n (%) 49 (71%) / 16 (23%) / 24 (35%) / 3 (4%) McDermott et al., 26-30 September 2014, Madrid, Spain esmo.org

18 MPDL3280A: Response in Patient with Sarcomatoid Variant mrcc Pre-treatment (9 Mar 2012) Post cycle 2 (20 Apr 2012) Pre-treatment tumor specimen H&E PD-L1 Carolina BioOncology Institute (Powderly) McDermott et al., 26-30 September 2014, Madrid, Spain 51 year old male with metastatic RCC (75% sarcomatoid variant) diagnosed Oct 2011 with T3aN2 disease s/p L nephrectomy now metastatic to lungs, skin and bone Prior sunitinib, temsirolimus and XRT to T9 Poor MSKCC risk and ECOG PS 1 PD-L1 IHC IC positive [IC IHC 3] Duration of response was 76 weeks esmo.org

19 MPDL3280A: irrc Partial Response in Patient With Metastatic Papillary RCC Pre-treatment (8 Sept 2012) Post Cycle 8 (26 Feb 2013) 72 year old female with metastatic RCC oncocytic papillary RCC s/p R nephrectomy and sunitinib PD-L1 [IC] IHC negative (IHC 0) irrc, immune-related response criteria. Yale School of Medicine (Sznol/Herbst) McDermott et al., 26-30 September 2014, Madrid, Spain esmo.org

POSITIVE PD-L1 by IHC Tumor Tissue TIMC* NON-CLEAR CELL RCC 11% 56% -Papillary (n=50) 10% 60% -Chromophobe (n=36) 5.6% 30% -Translocation Xp11 (n=10) 30% 90% -Collecting Duct (n=5) 20% 100% *TIMC: Tumor Infiltrating Mononuclear Cells

PD-L1 Expression in Non-ccRCC and Clinical Outcome Overall Survival Time to Recurrence Choueiri et al. Ann Oncol 2014

Conclusions VEGF and mtor inhibitors are options for non-clear cell RCC, although clinical trials are preferred, even for frontline therapy Everolimus is not superior to sunitinib Targeting MET remains a viable strategy in papillary RCC: MET pathway analysis may select for optimal candidates TCGA (KIRP) results coming soon Immune checkpoint blockers should be tested in all RCC subtypes

Acknowledgements (Partial) Kidney Cancer Research at DF/HCC BWH/DFCI/Broad/MIT: Signoretti, Freedman, Freeman, Meyerson, Garraway, Chang (Urology), Hirsch, Hodi, Kaelin BIDMC and MGH: McDermott, Bhatt, Mier, Michaelson Harvard School of Public Health DF/HCC Kidney SPORE Collaborators: National: CCF (Rini), Mayo (Ho), COH (Pal), MSKCC (Motzer, Hakimi, Hsieh), MDACC (Tannir), Georgetown (Atkins), IMDC Centers PIs International: Heng/Tom Baker, Canadian Centers, IMDC Centers, Gustave-Roussy Industry Partners

KIDNEY CANCER CENTER Dana-Farber Cancer Institute 24