Do not make unauthorized copies. APPENDIX A KARNOFSKY AND LANSKY PERFORMANCE SCALES Karnofsky Lansky Score Description Score Description 100% No symptoms. 100% fully active, normal 90% Able to carry on normal activity; minor signs or symptoms of disease. 90% minor restrictions in strenuous physical activity 80% Able to carry on normal activity with effort; some signs or symptoms of disease. 80% active, but tired more quickly 70% Cares for self, unable to carry on normal activity or do active work. 70% greater restriction of play and less time spent in play activity 60% Requires occasional assistance but is able to care for most of own needs. 60% up and around, but active play minimal; keeps busy by being involved in quieter activities 50% Requires considerable assistance and frequent medical care. 50% lying around much of the day, but gets dressed; no active playing participates in all quiet play and activities 40% Disabled; requires special care and assistance. 40% mainly in bed; participates in quiet activities 30% Severely disabled; hospitalization indicated, although death not imminent. 30% bed-bound; needing assistance even for quiet play 20% Very ill; hospitalization necessary; active supportive treatment required. 20% sleeping often; play entirely limited to very passive activities 10% Moribund, fatal processes progressing rapidly. 10% doesn't play; does not get out of bed 0 Patient expired. 0 unresponsive Ph III Ewing’s Sarcoma Vigil ± Irinotecan +Temozolomide IND 014205 CL-PTL-130 Version: 03 Page 68 of 74 Confidential and proprietary information of Gradalis, Inc. Effective: 05-DEC-2018 It is the user’s responsibility to verify adequate training before executing a document. Do not make unauthorized copies. APPENDIX B SCHEDULE OF ASSESSMENTS Procedure Prestudy / Surgery Pre- Screening Protocol Screening Day 1 of each Cycle1 (unless otherwise noted) End of TX Response Follow -Up (q 3mo±7 days) Until Progression Informed consent X X Medical History X Physical Examination X X X X X Toxicity (adverse events) from time of procurement X X Concomitant medications X X X X Performance Status X X X X Radiological Tumor Assessment (chest/abdomen/pelvis) within 4 weeks (must be post- procurement) every 6 weeks ± 7 days from Cycle 1 Within 45 days X CBC with differential X2 X X X X HIV testing, if applicable X Hepatitis testing, if applicable X Serum Chemistry X3 X X X X PBMC collection for Immune Function Analysis ≤ 1 week of tumor procurement X Cycles 2, 4, and 6 Day 1 prior to chemotherapy administration X 3 months post EOT and q 6 months thereafter Plasma collection for ctDNA ≤ 1 week of tumor procurement X Cycles 2, 3, 4, and 6 Day 1 prior to chemotherapy administration X Pregnancy Test (if applicable) X Tissue Procurement X Vigil administration depending on randomization Day 15 q 21±3 days 4 Injection Site Assessment Day 16 only (may be conducted at home)5 Temozolomide Days 1 – 5, q21 days Irinotecan Days 1 – 5, q21 days Survival Status X Long Term Follow Up6 Immunohistochemistry X7 1 Subjects who are stable at the end of Cycle 4, with prior approval by the Sponsor may conduct evaluations on Day 15 of each c ycle (1 Cycle = 21 days ±3 days) (See Section 9.0). 2 Obtain from medical records from standard preoperative hematology and chemistry panels. 3 Obtain from medical records from standard preoperative hematology and chemistry panels. 4 Cross- over subjects will receive Vigil on Day 1 . 5 Cross- over subjects will have assessment on Day 2. 6 After progression, subjects will be contacted by phone quarterly for documentation of post study therapies and survival status. 7 These slides should correlate with tumor procured for vaccine manufacture. Ph III Ewing’s Sarcoma Vigil ± Irinotecan +Temozolomide IND 014205 CL-PTL-130 Version: 03 Page 69 of 74 Confidential and proprietary information of Gradalis, Inc. Effective: 05-DEC-2018 It is the user’s responsibility to verify adequate training before executing a document. Do not make unauthorized copies. REFERENCES 1.