FCD.evidencereview.092818.4x4.SUBMITTED Data from 3 studies and 2 physician surveys indicate that the 31-GEP test results significantly impact management decisions for approximately 1 of 2 patients10-14 Technical success studies demonstrate 99% inter- and 100% intra-assay concordance9, 15 Evidence supports consistent ability of the 31-GEP test to accurately identify recurrence, metastasis, and melanoma-specific mortality in CM patients1-8 Sample adequacy Literature review of a prognostic 31-gene expression profile (31-GEP) test for cutaneous melanoma (CM) risk prediction Robert W. Cook, PhD, Kristen M. Plasseraud, PhD, Sarah J. Kurley, PhD, Kyle R. Covington, PhD, Federico A. Monzon, MD, FCAP Castle Biosciences, Inc., Friendswood, TX SYNOPSIS & OBJECTIVE In several cancers, molecular testing has added prognostic value and utility in the clinical setting. A 31-gene expression profile (31-GEP) test has been developed and validated for determining metastatic risk in cutaneous melanoma, with Class 1 and 2 results indicating low and high risks, respectively. As melanoma staging and guideline recommendations continue to evolve, it is important to consider the evidence supporting the use of clinicopathologic and molecular factors in melanoma patient care. Herein, published evidence supporting the 31-GEP test, including clinical validity, analytical validity, and clinical utility, are reviewed. From clinical validity evidence spanning eight peer-reviewed articles (n=1268 total patients) including two prospective studies, the 31-GEP test consistently demonstrated accuracy to identify patients with CM at high risk for recurrence, metastasis, and melanoma-specific mortality. Published analytical validity data verified the reliability of 31-GEP testing with inter- and intra- assay concordance of 99% and 100%, respectively, and 98% technical success on specimens with sufficient tumor content. Clinical utility data from three studies (n=494 total patients) and two physician surveys indicate that the 31-GEP test results significantly impact management decisions for approximately 1 of 2 patients, consistent with the impact of genomic testing in other cancers. In contrast to other prognostic melanoma GEP tests that have been reported, the 31-GEP test has published evidence from multiple retrospective and prospective clinical validity studies beyond initial development, along with published analytical validity and clinical utility data, in support of its use for melanoma risk assessment and patient management decisions. BACKGROUND & METHODS •The 31-GEP test is performed in a CAP- accredited/CLIA-certified laboratory using high-throughput RT-PCR assays as previously described1-4. •Clinical validity, analytical validity, and clinical utility studies surrounding the 31-GEP are reviewed herein. This study was sponsored by Castle Biosciences, Inc., which provided funding to contributing centers for tissue and clinical data retrieval. RWC, KPM, SJK, KRC, & FAM are employees and options holders of Castle Biosciences, Inc. CLINICAL VALIDITY CLINICAL UTILITY ANALYTICAL VALIDITY REFERENCES FUNDING & DISCLOSURES CONCLUSION Case Clinical Features 1 0.6mm, 42y female, Stage IA 2 0.54 mm, 62y male, Stage IB, ulcerated, personal hx 3 0.76 mm, 69y male, Stage IB, ulcerated, personal hx 4 >0.5mm, 45y male, Stage IB/IIA 5 0.9 mm, 61y female, Stage IB, ulcerated, mitoses 6 1.2 mm, 38y female, Stage IIA, ulcerated Does 31-GEP testing alter management decisions and if so, for what modality?13 0 20 40 60 80 100 0.26 0.5 0.76 2.1 % R ec om m en di ng 3 1- G E P Breslow Thickness (mm) Percentage of dermatologists who would order the 31-GEP test in different clinical scenarios Baseline Ulcerated SLN Negative * ** * ** **p<0.05 *p<0.001 Adapted from Svoboda et al. What features prompt physicians to recommend testing?14 Adapted from Farberg et al. *p<0.05 Fisher’s exact test* * * Figure 6. Schematic representation of using AJCC staging with 31-GEP test result to guide clinical management High Risk Class 2 Consider increased surveillance intensity Consider decreased intensity Continue low intensity managementLow Risk Stage I-IIA Early detection of recurrence Melanoma Staging (per NCCN) Low Risk Class 1 High Risk Stage IIB-III High Risk Class 2 Low Risk Class 1 Continue high intensity management Appropriate treatment or clinical trial AJCC Staging 31-GEP Test Management Decision Recommendation changes with GEP result in patient vignettes Technical reliability Sufficient tumor content Insufficient content (<40%) 96.3% n=16,472 98.3% n=16,190 3.7% n=630 1.7% n=282 Successful GEP Multi-gene failure Figure 8. The 31-GEP test has high technical reliability on >17,000 clinical cases since July 201615 Figure 7. 31-GEP results are highly concordant between assays (n=168 cases)9 Experiment 1 Probability Score E xp er im en t 2 P ro ba bi lit y S co re y = 0.9568x + 0.0115 R2 = 0.962 Figure 2. The 31-GEP test is an independent predictor of risk in a multivariate analysis across a large retrospective cohort of Stage I-III cases 4 1. Gerami P, et al. Clin Cancer Res 2015;21:175-83. 2. Gerami P, et al. J Am Acad Dermatol 2015;72:780-5 e783. 3. Zager JS, et al. BMC Cancer 2018;18(1):130. 4. Gastman BR, et al. JAAD 2018; doi: 10.1016/j.jaad.2018.07.028. 5. Hsueh EC, et al. J Hematol Oncol 2017;10:152. 6. Greenhaw B, et al. Dermatol Surg 2018; DOI: 10.1097/DSS.0000000000001588. 7. Renzetti M, et al. Society of Surgical Oncology Annual Meeting 2017. 8. Hsueh EC, et al. J Clin Oncol 2016; 34(15_suppl):9565. 9. Cook RW et al. Diagn Pathol 2018;13(1):13. 10. Berger AC et al. Curr Res Med Opin 2016;32(9):1599-604. 11. Dillon LD et al. SKIN: J Cut Med 2018;2(2):111-21. 12. Schuitevoerder D et al. J Drugs Dermatol 2018;17(2):196-199. 13. Farberg AS et al. J Drugs Dermatol 2017;16(5):428-431. 14. Svoboda RM et al. J Drugs Dermatol 2018;17(5):544. 15. Berg AS et al. DERM2018 Conference 2018 Design (n) GEP Impact Prospectively tested patients, Retrospective chart review; (156 patients)10 53% Prospective documentation of pre and post test plans; (247 patients)11 49% Prospectively tested patients, Retrospective chart review; (90 patients)12 52% Physician survey of clinical decisions with or without test results; (169 physicians)13 47- 50% Physician survey of clinical factors that affect use of 31-GEP test; (181 physicians)14 * *overall GEP impact not assessed with study design Table 1. Comparison of Clinical Utility Studies Figure 5. Physician survey studies address key questions for 31-GEP use13,14 Cook et al. In review of the literature, the value of the 31-GEP test for use in prognosis and clinical management decision making is supported by evidence from the 3 pillars of molecular tests: clinical validity, clinical utility, and analytical validity. Cox Multivariate Analysis RFS DMFS MSS HR 95% CI P-value HR 95% CI P-value HR 95% CI P-value Breslow Depth 1.21 1.12- 1.3 <0.0001 1.19 1.09- 1.29 <0.0001 1.16 1.0- 1.34 0.05 Mitotic rate 1.01 0.99- 1.03 0.18 1.01 0.99- 1.03 0.24 0.97 0.92- 1.03 0.34 Ulceration 1.1 0.75- 1.59 0.64 1.57 1.02- 2.43 0.04 0.77 0.38- 1.57 0.47 Positive node 2.45 1.74- 3.46 <0.0001 3.02 2.0- 4.57 <0.0001 3.83 1.85- 7.95 0.0003 Class 1B 1.13 0.56- 2.29 0.73 1.35 0.58- 3.15 .48 4.37 0.84- 22.72 0.08 Class 2A 1.48 0.77- 2.84 0.24 1.53 0.68- 3.43 .30 2.52 0.42- 15.2 0.31 Class 2B 2.92 1.7- 5.00 <0.0001 2.89 1.49- 5.62 0.002 9.02 2.02- 40.24 0.004 HR, hazard ratio; RFS, recurrence-free survival; DMFS, distant metastasis-free survival; MSS, melanoma-specific survival; GEP, gene expression profile; CI, confidence interval; 147 recurrences, 107 distant metastases, 36 melanoma-specific deaths Subgroup analysis of this cohort also demonstrated independent ability of the 31-GEP test to detect patients at high risk for metastasis in low-risk populations of sentinel lymph node-negative, Stage I-IIA, and T1 tumors. Figure 1. Accuracy metrics of the 31-GEP test within a large retrospective cohort (n=690) •The 31-GEP test predicts a CM patient’s risk of recurrence, metastasis, or melanoma- specific mortality at 5 years after diagnosis Class 1 Low risk of melanoma recurrence within 5 years Class 2: High risk of melanoma recurrence within 5 years 1A Lowest risk 2B Highest risk 2A Increased risk 1B Low risk RNA isolation cDNA generation and amplification (14X) Open Array PCR gene card 28 discriminant gene targets and 3 control genes Patients with Stage I-III melanoma Primary CM tumor tissue Formalin Fixed, Paraffin Embedded (≥ 40% tumor content) Analysis of gene expression profile with a proprietary algorithm to determine Class and metastatic risk Change StudyBerger et al. Dillon et al. Class 1 Changed 37% 36% Changed Class 1 w/ decrease 94% 67% Class 2 Changed 77% 85% Changed Class 2 w/ increase 94% 92% Figure 4. 31-GEP result drives surveillance changes in multicenter studies10,11 Consistent specific modality changes across both studies: • Decreases in imaging, visits, and referrals with Class 1 result • Increases in labs, imaging, visits and referrals with Class 2 result Multi-center study5 n=322% R ec ur re nc e Fr ee Figure 3. 31-GEP Class divides patients into high and low-risk categories for recurrence, metastasis and death across multiple prospective studies5,6,8 Class 1 Class 2 p<0.0001 Single-center study6 p<0.0001 Time (years) P ro ba bi lit y A vo id in g M et as ta tic D is ea se % M et as ta si s Fr ee Hsueh et al. ASCO 2016 Time (months) n=159 p<0.00001S u rv iv a l P ro b a b il it y 1.0 0.8 0.6 0.4 0.2 0 Additional single-center study8 Class 1 Class 2 p<0.00001 % S ur vi va l -30 -20 -10 0 10 20 30 40 50 60 1 2 3 4 5 6 Class 1 Class 2 % o f p hy si ci an s re co m m en di ng (c om pa re d to w ith ou tG E P re su lt) Imaging SLNB -30 -20 -10 0 10 20 30 40 50 60 1 2 3 4 5 6 * * * * * * * * * * * * * * * * * * *