Targeting ALK in Advanced NSCLC A New Treatment Paradigm Alice T. Shaw, MD PhD Massachusetts General Hospital Cancer Center Harvard Medical School August 25, 2012 Oncogenic Drivers in NSCLC KRAS 24%1 EGFR 13%1 RET ~1%5 4 MET ~1% 1Sequist et al., Ann Oncol 22:2616, 2011 2Takeuchi et al., Nat Med, Feb 12 2012 NRAS 1%1 3Shaw et al., NEJM 365:158, 2011 4Kris et al., WCLC 2011 AKT ~1%3 5Takeuchi et al., Nat Med, Feb 12 2012 PIK3CA 4%1 ALK 3%2 HER2 2%3 BRAF 2%1 ROS ~1%2 The Promise of Genotype-Directed Therapy Treatment B Treatment A NSCLC Treatment C Treatment D The Promise of Genotype-Directed Therapy EGFR mutation ALK (or ROS) Erlotinib or gefitinib Crizotinib, other ALK TKIs NSCLC Treatment C Treatment D NCCN Guidelines for the Management of Stage IV NSCLC Crizotinib an update Crizotinib: A Dual MET/ALK Tyrosine Kinase Inhibitor Kinase IC50 (nM) mean* Selectivity ratio c-MET 8 – ALK 40-60 5-8X ROS 60 7X RON 80 10X 294 34X 322 37X Tie-2 448 52X Trk A 580 67X Trk B 399 46X Abl 1,159 166X IRK 2,887 334X Lck 2,741 283X Sky >10,000 >1,000X VEGFR2 >10,000 >1,000X PDGFR >10,000 >1,000X Axl Co-crystal structure of crizotinib (PF02341066) bound to c-MET Cui et al. J. Med. Chem. 2011;54:6342-63 and Pfizer data on file Marked Activity of Crizotinib in ALK+ NSCLC Update of the Phase 1 Study ORR = 60.8%* in 143 evaluable patients (133 evaluable patients shown) Median response duration = 49.1 wks Median PFS = 9.7 mos *Higher ORR in Asians vs non-Asians -30 Camidge et al., Lancet Onc, in press Phase 2 Study: PROFILE 1005 Key entry criteria ● Positive for ALK by central laboratory (local testing subsequently allowed on case-bycase basis) ● Progressive disease in Arm B of PROFILE 1007 study Crizotinib 250 mg BID N=250 >1000 administered PO on a continuous dosing schedule ● >1 prior chemotherapy ● ECOG PS 0-3 ● Stable/controlled brain mets allowed NCT00932451 Marked Activity of Crizotinib in ALK+ NSCLC Update of the Phase 2 Study Decrease or increase from baseline (%) 100 PD SD PR CR 80 60 40 20 0 –20 –40 –60 –80 –100 + + ++ –120 *n=240 response-evaluable patients from the mature population, and excludes patients with early death, indeterminate response and non-measurable disease +Per RECIST 1.1, percent change from baseline for subjects with best overall response of CR can be less than 100% when lymph nodes are included as target lesions Kim et al., ASCO 2012 Marked Activity of Crizotinib in ALK+ NSCLC Update of the Phase 2 Study Probability of survival without progression 1.0 Median PFS 8.1 months (95% CI: 6.8–9.7) 28% patients in follow-up for progression 0.8 + Censored 95% Hall-Wellner Band 0.6 0.4 0.2 0 n at risk 0 5 261 175 10 Time (months) 95 15 20 26 2 Kim et al., ASCO 2012 PROFILE 1005: Any-Grade TreatmentRelated AEs in ≥10% of Patients AE Mature population, n=261 n (%) Overall population, n=901 n (%) Any AE 245 (93.9) 827 (91.8) Vision disorder* 154 (59) 468 (51.9) Nausea 148 (56.7) 423 (46.9) Vomiting 116 (44.4) 352 (39.1) Diarrhea 106 (40.6) 369 (41.0) Constipation 86 (33.0) 249 (27.6) Peripheral edema 72 (27.6) 211 (23.4) Fatigue 64 (24.5) 163 (18.1) Decreased appetite 59 (22.6) 167 (18.5) Increased alanine aminotransferase 45 (17.2) 146 (16.2) Dysguesia 43 (16.5) 149 (16.5) Dizziness 40 (15.3) 95 (10.5) Neutropenia 36 (13.8) 84 (9.3) Increased aspartate aminotransferase 33 (12.6) 106 (11.8) *Includes visual impairment, photopsia, vision blurred, vitreous floaters, photophobia and diplopia Rare instances of fatal pneumonitis and fatal hepatotoxicity were reported in crizotinib clinical trial program Phase 3 Study: PROFILE 1007 Crizotinib 250 mg BID Key entry criteria ● Positive for ALK by central FISH testing ● Stage IIIB/IV NSCLC ● 1 prior chemotherapy (platinum-based) ● ECOG PS0-2 ● Measurable disease ● Treated brain metastases allowed1 R A N D O M I Z E N=318 administered PO on a continuous dosing schedule (N=159) Endpoints ● Primary - PFS (RECIST 1.1, independent review) ● Secondary - ORR, DR, DCR Pemetrexed 500 mg/m2 or Docetaxel 75 mg/m2 infused IV on day 1 of a 21-day cycle - OS - Safety - Patient reported outcomes (PROs) (N=159) NCT00932893 Next Generation ALK TKIs LDK378 Mechanisms of Crizotinib Resistance Target gene alteration (28%) ?? No loss of ALK (26/26) No EGFR or KRAS mutations (21/21) Bypass track activation (45%) * * ALK amplification ALK mutation EGFR activation CKIT amplification Unknown LDK378: A Potent and Selective ALK Inhibitor 2,4-diaminopyrimidine derivative. MW: 558.14 Potent, ATP-competitive inhibitor of ALK Assays LDK378 IC50 (μM) Crizotinib IC50 (μM) ALK (enzymatic) MET (enzymatic) 0.00015 3.2 0.003 0.008 0.027 1.3 0.11 0.028 BaF3/ALK BaF3/MET Li et al., AACR-NCI-EORTC, 2011; Mehra et al., ASCO 2012 Preclinical Evaluation of LDK378 Activity NCI-H2228 sensitive xenograft models • LDK378 caused complete tumor regression after 14 days of treatment • No growth of tumors for >4 months in mice treated with LDK378 50 mg/kg/day Li et al., AACR-NCI-EORTC, 2011 Preclinical Evaluation of LDK378 Activity Crizotinib-resistant xenograft models ALK C1156Y mutation No ALK mutation • LDK378 at 50-100mg/kg/day has antitumor activity in different crizotinib-resistant models Li et al., AACR-NCI-EORTC, 2011 Phase I study of LDK378 ALK+ lung cancer Resistant to prior ALKi Any ALK+ cancer ALK+ lung cancer Naive to prior ALKi Non-lung ALK+ tumors LDK378 continuous oral dosing Primary objective: Determine the MTD Secondary objectives: Safety, pharmacokinetics, and antitumor activity NCT01283516 Baseline Patient Characteristics Characteristic Age (median), yrs (range) All patients, n (%) N=56 53 (22–78) Sex (male) 19 (34) ECOG Performance Status 0 1 2 10 (18) 39 (70) 7 (12) Cancer type NSCLC Breast Rectal Alveolar rhabdomyosarcoma 50 (89) 4 (7) 1 (2) 1 (2) Prior crizotinib Crizotinib naive 37 (66) 19 (34) Mehra et al., ASCO 2012 Adverse events associated with LDK378 • SAEs related to LDK378 have occurred in 5 patients – Transaminase elevation (400 mg), vomiting (500 mg), dehydration (600 mg), and interstitial lung disease (750 mg) • All SAEs were reversible upon cessation of LDK378 – Two patients resumed treatment with LDK378 a lower dose level – Two had simultaneous progressive disease • Common AEs included – Nausea (59%), vomiting (54%), and diarrhea (48%), fatigue (21%), and dyspnea (16%) • Only one patient has discontinued treatment because of an AE Mehra et al., ASCO 2012 Antitumor Activity of LDK378 in ALK+ NSCLC NSCLC Other diseases Initial dose (mg) Patients (n) Responses <400 8 2 (25) ≥400 33 22 (67) 50–600 6 0 • Response rate 81% (21/26) in NSCLC patients treated at ≥400 mg who progressed following crizotinib – Responses include confirmed + unconfirmed per RECIST 1.0 (6 patients with PR awaiting confirmatory scans) Mehra et al., ASCO 2012 Typical Responses to LDK378 Baseline After 6 weeks on LDK378 Mehra et al., ASCO 2012 Typical Responses to LDK378 Baseline After 6 weeks on LDK378 Mehra et al., ASCO 2012 CNS Activity of LDK378 Baseline After 6 weeks on LDK378 Mehra et al., ASCO 2012 Summary of LDK378 • The MTD of LDK378 is 750 mg PO qd • The most common AEs are nausea, vomiting and diarrhea • LDK378 exhibits potent antitumor activity in patients with ALK+ NSCLC, particularly in those who have progressed following crizotinib • LDK378 is active in brain metastases Other Next Generation ALK TKIs CH5424802 AP26113 CH5424802 is a Potent and Selective ALK TKI A C B Sakamoto et al., Cancer Cell 2011;19:679-90 AP26113 is a Potent and Selective ALK TKI Relative Cell Viability Crizotinib 100 AP26113 EML4-ALK -L1196M 75 50 75 50 EML4-ALK 25 0 ALK-negative cells 100 1 10 100 25 1000 10000 0 1 10 100 1000 10000 Drug Concentration (nM) > > > Ba/F3 cells (ALK-negative) engineered to express EML4-ALK or the L1196M mutant AP26113, but not crizotinib, inhibits viability of the L1196M mutant with high selectivity Similar results obtained with multiple other mutants, including G1269S, S1206R, C1156Y, F1174C, F1245C, I1174T and L1152V Zhang et al, AACR 2010 Phase I/ II Schema Phase 1/2 Study of AP26113 Cohort 1: N=20 NSCLC: ALK rearrangement and ALK inhibitor naïve Dose Escalation Cohorts N=30 to 50 Advanced malignancies All histologies except leukemia ECOG 0-2 CNS mets allowed if stable for 8 wks Oral AP26113, 30 mg once daily (QD) starting dose → Dose level cohorts escalating until RP2D is established * Tissue must be obtained after development of resistance to ALK (cohort 2) or EGFR (cohort 3) TKI therapy * Cohort 2: N=20 NSCLC: ALK rearrangement and resistant to at least 1 prior ALK inhibitor * Cohort 3: N=20 NSCLC: EGFR activating mutation and resistant to at least 1 prior EGFR inhibitor Cohort 4: N=20 Other cancers with abnormal targets eg, ALK, ROS, and others Summary • Crizotinib is now a standard therapy for patients with advanced, ALK+ NSCLC - ORR 60% - Median PFS 8-9 months - Phase 3 studies • More potent ALK TKIs like LDK378 may be effective salvage therapies for the majority of patients who relapse on crizotinib Future Directions • Timing of next generation ALK TKIs - Front-line use of next generation ALK TKIs • Mechanism of action - Overcoming resistance vs more potent target inhibition • Resistance to next generation ALK TKIs • Developing combination strategies