Welcome to the Jungle - Targeted Therapies, Immunomodulatory Approaches, and the New Drugs in HNSCC Ezra E. W. Cohen Moores Cancer Center University of California San Diego “Welcome to the jungle We've got fun 'n' games We got everything you want...” GNR Incidence • 2014, US: – ~50000 new cases – ~8000 deaths • Worldwide: – 500000 new cases – 6th leading cause of death • M:F = 3:1 Ten Leading Cancer Types, United States, 2012 CA Cancer J Clin 2012 Jan-Feb;62(1):10-29. Risk Factors • • • • Age (majority of patients >50 years old) Tobacco Alcohol Viral – Epstein-Barr virus (NPC) – HPV (oropharynx) • Hereditary (rare): – Family history increases risk (3.5X) – Rare genetic syndromes, e.g. Fanconi’s Anemia HPV Cancers with Increasing Incidence Trends in the US •CA Cancer J Clin. 2012 Jan 4. Three-dose HPV vaccination coverage among girls (13 to 17 years), by state, 2010. New (HPV)–associated cancers overall, and by sex, in the United States, 2009 Sexual Behaviors and HNC Diagnosis of HPV-SCCHN (case-case) Risk of HPV-SCCH (case-control) Number of sexual partners + + History of oral-genital sex + + History of anal cancer + + Spouse with tonsil cancer + + Assoc Assoc Behavior Cervical CIS or cancer HNC HPV • Strong association with oropharynx, especially tonsil, cancers • Non-smokers, non-drinkers • Younger age • M:F = 3:1 • 90% of HNC cases with HPV are sub-type 16 Prognostic effect of HPV •Langer CJ. Exploring biomarkers in head and neck cancer. Cancer. 2012 Aug 15;118(16):3882-92. 13 Human Papillomavirus and Overall Survival After Progression of Oropharyngeal Squamous Cell Carcinoma C Fakhry et al Overall survival after disease progression 100 Survival (%) 75 p16-positive 54.6% 50 p<0.001 p16-negative 25 27.6% 0 0 No. at Risk p16-positive 105 p16-negative 76 1 Years after Progression 61 27 2 51 19 15 DE-INTENSIFICATION …as an approach to address HPV related disease and good prognosis E1308 Induction followed by IMRT/Cetuximab INDUCTION (3 cycles) ELIGIBILITY Stage III/IVa,b Resectable HPV+ Oropharynx S I M U L A T I O N CONCURRENT Paclitaxel 90 mg/m2 q 7d CDDP 75mg/m2 q21d IMRT 54Gy/30 fxs CR* Cetuximab 250mg/m2 qwk Cetuximab 250mg/m2 qwk CONCURRENT <CR IMRT 69.3Gy/33fxs Cetuximab 250mg/m2 qwk Cetuximab loading dose = 400mg/m2 on Day1 of Cycle1 with Induction * CR indicates clinical CR and in patients with near CR will undergo biopsies of primary site to confirm pathological CR Phase II Randomized Trial of Transoral Surgical Resection followed by Low-dose or Standard-dose IMRT in Resectable p16+ Locally Advanced Oropharynx Cancer (E3311) p16+, Stage III/IV (cT1-2N1-N2b) OPSCC Credentialing of surgeon required as part of site participation in the trial Stratify by stage and smoking status Trial Schema Risk-Stratified IMRT Arm 1 TORS Nmax = 54 T1-4a, N1-3 HPV+ OPSCC Amenable to TORS B I O P S Y Induction Chemotherapy 3 cycles pCR or pT1-2 N0-1 (-) margin, no ECE Observe Arm 2 Cisplatin 75 mg/m2/q3wk TORS, SLND Paclitaxel 90 mg/m2/week BYL719 daily Close margin, ≥pN2, or PNI/LVI 60 Gy IMRT 9 weeks Arm 3 * *FDG/PET-CT scan * (+) margin, ECE 66 Gy IMRT + Weekly Cisplatin Treatment Overview • Early stage (I, II): single modality – RT or surgery – 80-90% long-term survival • Advanced stage (III, IVA, IVB): multi-modality therapy – surgery/RT/chemotherapy – 50% long term survival • Recurrent/Metastasis – 15% can be salvaged (surgery, re-RT) – Palliative systemic therapy EGFR Directed Therapy Cetuximab Phase III Study Stratify by Karnofsky score: 90-100 vs. 60-80 Regional Nodes: Negative vs. Positive Tumor stage: AJCC T1-3 vs. T4 RT fractionation: Concomitant boost vs. Once daily vs. Twice daily R A N D O I M I Z E Arm 1 Radiation therapy Arm 2 Radiation therapy Cetuximab, weekly Phase III: Cetuximab Plus RT for SCCHN: Results Locoregional Control 47% vs 34% at 3 years P<0.01 at 3 years OS 55% vs 45% at 3 years P=0.05 at 3 years Bonner. N Engl J Med. 2006;354:567. Copyright © [2006] Massachusetts Medical Society. All rights reserved.; Posner. N Engl J Med. 2006;354:634. EXTREME - Study design Randomized Group A Cetuximab 400 mg/m2 initial dose then 250 mg/m2 weekly + EITHER carboplatin (AUC 5, d1) OR cisplatin (100 mg/m2 IV, d1) + 5-FU (1000 mg/m2 IV, d1-4): 3-week cycles Group B EITHER carboplatin (AUC 5, d1) OR cisplatin (100 mg/m2 IV, d1) + 5-FU (1000 mg/m2 IV, d1-4): 3-week cycles 6 chemotherapy cycles maximum Cetuximab No treatment Progressive disease or unacceptable toxicity Overall Survival 1.0 | 0.9 | || | | CTX only CET + CTX | | | Survival Probability 0.8 || HR (95%CI): 0.797 (0.644, 0.986) Strat. log-rank test: 0.0362 | 0.7 | 0.6 0.5 10.1 mo | 7.4 mo 0.4 | 0.3 0.2 | | | | |||| | | | | | | | || ||| | || || || | | || || | ||| | ||| ||| | | | | || | |||| | | | || | | | | | || || 0.1 0.0 0 Patients at Risk CTX only 220 CET + CTX 222 3 6 9 12 15 18 21 24 Survival Time [Months] 173 184 127 153 83 118 65 82 47 57 19 30 8 15 1 3 Afatinib versus methotrexate as second-line treatment for patients with R/M HNSCC who progressed after platinum-based therapy: primary efficacy results of LUX-Head & Neck 1, a Phase III trial J-P. H. Machiels, R. I. Haddad, J. Fayette, L. F. Licitra, M. Tahara, J. B. Vermorken, P. M. Clement, T. Gauler, D. Cupissol, J. J. Grau, J. Guigay, F. Caponigro, G. de Castro Jr, L. de Souza Viana, U. Keilholz, J. M. del Campo, X. Cong, L. Svensson, E. Ehrnrooth, and E. E. W. Cohen on behalf of the LUX-H&N 1 investigators LUX-Head & Neck 1: study design Patients with incurable R/M HNSCC progressing on/after first-line platinum-based therapy (N=474) Randomisation (2:1) Stratified by: ECOG PS (0 vs 1) and prior use of EGFR mAb therapy (Yes/No) Afatinib 40 mg orally once daily (n=316) Methotrexate 40 mg/m2 IV weekly (n=158) Primary endpoint: PFS Key secondary endpoint: OS Secondary endpoints: ORR, patient-reported outcomes, safety Primary endpoint: PFS independent review Estimated PFS probability 1.0 Afatinib MTX (n=322) (n=161) 275 (85.4) 135 (83.9) 2.6 1.7 0.80 (0.65–0.98) 0.030 PFS event, n (%) Median PFS (months) HR (95% CI) Log-rank test p-value 0.8 0.6 42.8% 0.4 30.5% 0.2 0 0 No. of patients Afatinib 322 MTX 161 3 6 9 Time (months) 12 15 18 93 28 26 6 9 2 3 0 1 0 0 0 CI, confidence interval; MTX, methotrexate Overall tumour response Percentage of patients 60 60 50 50 † Afatinib Methotrexate 49.1 38.5 40 40 30 30 20 20 10 10 * 10.2 5.6 00 ORR afatinib *Odds DCR‡ mtx ratio: 1.9 (0.88–4.14); p-value = 0.101 ratio: 1.5 (1.03–2.26); p-value = 0.035 ‡Disease control rate (DCR): includes objective response and stable disease †Odds Overall survival Estimated OS probability 1.0 Afatinib MTX (n=322) (n=161) OS event, n (%) 237 (73.6) 121 (75.2) Median OS (months) 6.8 6.0 HR (95% CI) 0.96 (0.77–1.19) Log-rank test p-value 0.700 0.8 0.6 0.4 0.2 0 0 No. of patients Afatinib 322 MTX 161 3 6 9 255 115 172 76 89 48 12 15 Time (months) 53 29 28 16 18 21 24 27 14 9 6 7 1 3 0 0 Time to deterioration of pre-specified patient-reported outcomes* Global health status Estimated probability 1.0 0.74 (0.56–0.97); 0.027 HR (95% CI); p-value† 0.8 0.6 Afatinib Methotrexate 0.4 0.2 0 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 1.0 Pain 0.8 HR (95% CI); p-value† Swallowing 1.0 0.73 (0.55–0.96); 0.022 0.6 0.4 0.2 0 Estimated probability Estimated probability Time (months) HR (95% CI); p-value† 0.8 0.67 (0.50–0.89); 0.004 0.6 0.4 0.2 0 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 Time (months) 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 Time (months) *Assessed using European Organization for Research and Treatment of Cancer (EORTC) questionnaire QLQ-C30 and Head and Neck cancer-specific module (QLQ-H&N35) for pain (composite of items 31–34) and swallowing (composite of items 35–38). †Based on log-rank test. Molecular Phenotyping COMPREHENSIVE GENOMIC CHARACTERIZATION OF SQUAMOUS CELL CARCINOMA OF THE HEAD AND NECK Neil Hayes, MD, MPH UNC Chapel Hill Lineberger Comprehensive Cancer Center ASCO 6/3/2013 Significantly mutated genes in HNSCC by whole exome sequencing Analysis – Juok Cho, Peter Hammerman, Carrie Sougnez COSMIC CDKN2A 17% FAT1 na TP53 44% CASP8 na JUB na PIK3CA 6% NOTCH1 14% MLL2 7% NSD1 6% HLA-A na TGFBR2 na HRAS 8% EPHA2 na RB1 na NFE2L2 5% KEAP1 na B2M na RAC1 na 40 Deregulation of signalling pathways and transcription factors. MS Lawrence et al. Nature 517, 576-582 (2015) doi:10.1038/nature14129 Cancer Immunotherapy Immunotherapy Harnessing the body’s own immune system to fight cancer Surgery Radiotherapy Chemotherapy Removal of the tumor Energy delivery to specific anatomical site Drugs designed to exploit cancer mutations Pursuing Immunotherapy in Head and Neck Cancer KEYNOTE-012 – Study Design • Multi-center, non-randomized Phase Ib HNSCC expansion cohort • Multi-cohort trial* HNSCC cohort Recurrent or metastatic Head and Neck Cancer - PD-L1 positive - Investigator assessed HPV status HPV(-) cohort Pembrolizumab HPV(+) cohort Pembrolizumab 10mg/kg Q2 weeks 10mg/kg Q2 weeks *Additional cohorts included: Bladder Cancer, Triple Negative Breast Cancer, Gastric Cancer •Presented by: Tanguy Seiwert Treat until PD* Efficacy: Waterfall Plot* 100 HPV (-) HPV (+) Change From Baseline, % 80 60 40 20 0 –20 –40 –60 51% (26/51) of patients had decreased tumor burden –80 –100 Subjects Best percent change from baseline in target lesions (site assessment) delineated by HPV status *as of May 23, 2014; Includes only patients with RECIST measurable lesions at baseline and at least 1 follow-up scan (n=51) •Presented by: Tanguy Seiwert Best Overall Response* 56 pts evaluable for Total Head/neck HPV (+) HPV (-) N=56† N=20 N=36§ Response n (%) 1 (1.8) 95% CI† (0.0, 9.6) n (%) 95% CI† n (%) 95% CI† 1 (5.0) (0.1, 24.9) 0 (0.0) (0.0, 9.7) Partial Response 10 (17.9) (8.9, 30.4) 3 (15.0) (3.2, 37.9) 7 (19.4) (8.2, 36.0) Best Overall Response (Complete + Partial)‡ 11 (19.6) (10.2, 32.4) 4 (20.0) (5.7, 43.7) 7 (19.4) (8.2, 36.0) Stable Disease 16 (28.6) (17.3, 42.2) 8 (40.0) (19.1, 63.9) 8 (22.2) (10.1, 39.2) Progressive Disease 25 (44.6) (31.3, 58.5) 7 (35.0) (15.4, 59.2) 18 (50.0) (32.9, 67.1) 4 (7.1) (2.0, 17.3) 1 (5.0) (0.1, 24.9) 3 (8.3) (1.8, 22.5) Response Evaluation Complete Response No Assessment Based on RECIST 1.1 Per site assessment; includes confirmed and unconfirmed responses †61 patients eligible for treatment; 60 patients dosed; 56 patients eligible for pre-defined full analysis set. ‡A single patient with PD followed by PR on treatment was classified as PR. §Includes † Based 2 patients for whom HPV data unavailable. on binomial exact confidence interval method. • PD-L1 expression correlates with Response • Using a Youden-Index derived, preliminary PD-L1 cut point: Above cutpoint: 45.5% (5/11) RR Below cutpoint: 11.4% (5/44) RR *as of May 23, 2014 •Presented by: Tanguy Seiwert Subjects Time on treatment and disposition* Complete Response Partial Response Treatment Ongoing 0 4 8 12 16 20 24 28 32 36 40 44 48 Treatment Exposure, weeks Swimmer plot of all patients who experienced CR or PR. 8 additional patients had SD >6 months, of which 7/8 remain on treatment. *as of May 23, 2014 •Presented by: Tanguy Seiwert Toll-like Receptor 8 (TLR8) Pathway is Important in Human Immune Responses • Activation induces potent Th1 immune response • Expressed on myeloid dendritic cells (CD11c+), monocytes (CD14+), and natural killer cells (CD56+) in humans • Induces significant IL-12 production in humans • Can be activated by small molecule agonists •58 A Randomized, Double-Blind, Placebo-Controlled Study of Chemotherapy Plus Cetuximab in Combination with VTX-2337 in Patients with Recurrent or Metastatic Squamous Cell Carcinoma of the Head and Neck Active8 Study Schema 175 patients with locally advanced or metastatic head/neck cancer Primary endpoint: progression-free survival Mutation frequency among cancers of different histotypes Mutational screen of 305 HNSCC exome sequences deposited in TCGA HNSCC tumors express missense mutations Mutational screen of 305 HNSCC exome sequences deposited in TCGA HNSCC tumors express missense mutations Project: Exome-guided neoantigen discovery in HNSCC HNSCC pt In office/clinic 1) Tumor: FNA, core, or excision biopsy 2) <1 ml PBL salvage surgery Immune monitoring of checkpoint blockade trials gDNA & mRNA Whole Exome Sequencing expand TIL with ‘antigenic mutanome’ peptides Establish PDX model & perform ACT with ‘improved” TIL “antigenic mutanome” Test peptides for recognition by autologous T cells w ELISPOT (use prediction algorithm as needed) Bioinformatics: Reassembly, alignment, QC mutation calling Confirm SNVs in expressed genes (RNAseq) CONCLUSIONS • EGFR inhibitors still the only proven “targeted therapy” in HNSCC • HPV is a recognized prognostic marker – Other stratification factors needed • Immunotherapy extremely promising – Phase 3 trials underway in R/M disease likely to complete accrual this year – PDL1 being explored as predictive biomarker