About this trial
This phase I/II trial tests the safety, side effects and best dose of DT2216 in combination with irinotecan and how well it works in treating children, adolescents and young adults with solid tumors and fibrolamellar cancer that has come back after a period of improvement (relapsed) or that has not responded to previous treatment (refractory). DT2216 is an anti-apoptotic protein B-cell lymphoma-extra large targeted protein degrader. It may stop the growth of tumor cells by blocking Bcl-xL, a protein needed for tumor cell survival. Irinotecan is in a class of antineoplastic medications called topoisomerase I inhibitors. It blocks a certain enzyme needed for cell division and deoxyribonucleic acid repair and may kill tumor cells. Giving DT2216 in combination with irinotecan may be safe, tolerable, and/or effective in treating children, adolescents and young adults with relapsed or refractory solid tumors or fibrolamellar cancer.
Eligibility criteria
This trial does not accept healthy volunteersQualifiers
PHASE 1: Patients between ≥ 1 year and ≤ 21 years of age at the time of study enrollment
PHASE 2: Patients between ≥ 1 year and ≤ 39 years of age at the time of study enrollment
PHASE 1: Patients with recurrent/refractory solid tumors excluding primary central nervous system tumors
PHASE 2: Patients with (FLC), which must include genomic confirmation of the DNAJB1:PRKACA fusion performed at a Clinical Laboratory Improvement Act (CLIA)-certified laboratory
Disqualifiers
Pregnant or breast-feeding women will not be entered on this study due to risks of fetal and teratogenic adverse events as seen in animal/human studies, OR because there is yet no available information regarding human fetal or teratogenic toxicities. Pregnancy tests must be obtained in girls who are post-menarchal. Males or females of reproductive potential may not participate unless they have agreed to use two effective methods of birth control, including a medically accepted barrier or contraceptive method (eg, male or female condom) for the duration of the study. Abstinence is an acceptable method of birth control. Patients who could become pregnant should use highly effective contraception during therapy and for 6 months after the last irinotecan dose or 120 days after the last dose of DT2216, whichever is longer. Patients with partners who could become pregnant should use condoms during therapy and for 3 months after the last dose irinotecan or 120 days after the last dose of DT2216, whichever is longer
Patients receiving corticosteroids who have not been on a stable or decreasing dose of corticosteroid for at least 7 days prior to enrollment are not eligible. If used to modify immune adverse events related to prior therapy, ≥ 14 days must have elapsed since last dose of corticosteroid
Patients who are currently receiving another investigational drug are not eligible
Patients who are currently receiving other anti-cancer agents are not eligible
Trial design
Single group
Treatments tested in this trial
Bcl-XL Proteolysis Targeting Chimera DT2216
Biological/VaccineGiven IV
Biospecimen Collection
Procedure/SurgeryUndergo blood sample collection
Irinotecan
DrugGiven IV
Treatment groups
Trial outcomes
Primary outcomes
Incidence of adverse events (AEs)
AEs will be assessed and graded using National Cancer Institute (NCI) Common Terminology Criteria for Adverse Events (CTCAE) version (v)5.0. A descriptive summary of all toxicities will be reported.
Dose-limiting toxicity (DLT)
AEs will be assessed and graded using NCI CTCAE v5.0. A descriptive summary of all toxicities will be reported.
Maximum tolerated dose (MTD)/ recommended phase 2 dose
MTD will be defined as the maximum dose at which fewer than one-third of patients experience DLT during cycle 1 of therapy.
Area under the dose concentration curve of DT2216
Median (minimum \[min\], maximum \[max\]) area under the dose concentration curve of DT2216 during cycle 1 assessed pre-dose, 5, 30, 60, 180, and 420 minutes post-dose.
Secondary outcomes
Pharmacodynamic activity
Western blotting will be performed to assess the pharmacodynamic activity by measuring peripheral mononuclear cell Bcl-xL levels.
Other outcomes
Correlation of peripheral blood levels of the DNAJB1-PRKACA chimera
A descriptive analysis of biomarkers will assess associations with disease response. The parameters will be summarized with simple summary statistics, including means, medians, ranges, and standard deviations.
Vitamin B12 levels
A descriptive analysis of biomarkers will assess associations with disease response. The parameters will be summarized with simple summary statistics, including means, medians, ranges, and standard deviations.
Genomic markers panel
Median (interquartile range) ratio of mutant droplet count to total droplet count stratified by study time point.
Intratumoral patterns of infiltrating immune cells
A descriptive analysis of biomarkers will assess associations with disease response. The parameters will be summarized with simple summary statistics, including means, medians, ranges, and standard deviations.
Sponsors and contacts
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