David S Shulman, MD
This research is being done to test a new drug called PEEL-224 in combination with two commercially available drugs, Vincristine and Temozolomide, and to determine how effective this combination of drugs is at treating Ewing Sarcoma (EWS) and Desmoplastic Small Round Cell Tumor (DSRCT), as well as multiple other kinds of sarcomas. The names of the study drugs and biological agents involved in this study are: * PEEL-224 (a type of Topoisomerase 1 inhibitor) * Vincristine (A type of vinca alkaloid) * Temozolomide (A type of alkylating agent) * Pegfilgrastim or Filgrastim (types of Myeloid growth factors)
This is an open-label, single-arm, non-randomized, phase I/II trial to test a new drug called PEEL-224 in combination with two commercially available drugs, Vincristine and Temozolomide, and to determine how effective this combination of drugs is at treating Ewing Sarcoma (EWS) and Desmoplastic Small Round Cell Tumor (DSRCT), as well as multiple other kinds of sarcomas. This is the first time that PEEL-224 will be given in combination with Vincristine and Temozolomide to humans. In Phase 1, the safety and tolerability of PEEL-224 in combination with Vincristine and Temozolomide will be assess…
Inclusion Criteria: -Patients in all cohorts must have relapsed or refractory disease after standard therapy. Inclusion Criteria Phase 1 (only) diagnosis requirements: -Patients must have: * Evaluable or measurable disease; and * Histologic diagnosis of sarcoma Inclusion Criteria Phase 2 (only) diagnosis requirements * EWS cohort: Patients must have: * RECIST measurable disease at study entry; * Histologic diagnosis consistent with Ewing sarcoma; and * Molecular evidence of a FET-ETS family translocation including but not limited to any of the following: * EWSR1::FLI1, EWSR1:…
Topoisomerase 1 inhibitor, 200mg amber vial, via intravenous (into the vein) infusion per protocol.
Alkylating agent, 5, 20, 100, 140, 180, or 250 mg capsule, taken orally per standard of care.
Vinca alkaloid, 1 or 2mL vials, via intravenous (into the vein) infusion per institutional policy.
Myeloid growth factor administered per institutional standards.
Myeloid growth factor administered per institutional standards.