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Study of a DNAJB1-PRKACA Fusion Kinase Peptide Vaccine with DRP-104, Nivolumab, and Ipilimumab for Fibrolamellar Carcinoma

This study is testing a new combination treatment for advanced fibrolamellar carcinoma (a type of liver cancer). It combines a special vaccine (DNAJB1-PRKACA Peptide Vaccine) with DRP-104, Nivolumab, and Ipilimumab. Researchers want to see if this combination is safe and effective in shrinking tumors. To join, you must have fibrolamellar carcinoma that has spread or cannot be removed by surgery, and your cancer must have a specific genetic change called the DNAJB1-PRKACA fusion. You also need to have shown progression on previous immunotherapy. The study will measure how many patients experience serious side effects and how many see their tumors shrink. The current recruitment status is unclear.

Study design
This is an interventional study planning to enroll 27 participants aged 12 and older. It is not specified if it is randomized or blinded.
What's involved
You would receive the DNAJB1-PRKACA Peptide Vaccine, DRP-104, Nivolumab, and Ipilimumab on specific schedules over several cycles, including intravenous (IV) infusions and subcutaneous injections.
Compensation
Not stated in the trial record.
Follow-up
The study will track side effects and tumor response for up to 4 years.

AI-generated from the public study record. Only the study team can confirm whether you're eligible — confirm details with them before making decisions.

NCT07430202

A DNAJB1-PRKACA Fusion Kinase Peptide Vaccine Combined With Glutamine Antagonist DRP-104, Nivolumab, and Ipilimumab in Patients With Advanced Stage Fibrolamellar Carcinoma (FLC)

Not Yet Recruiting
PHASE1Ages 12+InterventionalTreatment
Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins
~27 participants
Updated 2026-08-25 on ClinicalTrials.gov
What's tested:DNAJB1-PRKACA Peptide VaccineDRP-104NivolumabIpilimumab

At a glance

Recruiting sites
0 of 1 listed site is recruiting right now
RecruitingSuspended, closed, or not yet open
What they're measuring
Number of participants experiencing grade 3 or above drug-related toxicities
Measured over 4 years
+1 more outcome measured

Conditions

Liver Cancer (Fibrolamellar Hepatocellular Cancer (FLC))

Explore related trials

Fibrolamellar CarcinomaDNAJB1-PRKACA

NCT07430202

Where you'd take part

This study runs at 1 site. They're the same protocol — you choose where, and that choice sets who your contact draft is addressed to.

  • Johns Hopkins SKCCC

    Baltimore, Marylandstudy coordinator listed

Sites open and close at different times, so the status above is per site — it can differ from the study's overall status.

Study leadership

  • Marina Baretti, MD · PRINCIPAL_INVESTIGATOR · SKCCC • Johns Hopkins Medical Institution

Who to contact

Colleen Apostol, RN
[email protected]
410-614-3644
Email the study team

Opens a ready-to-send draft in your own email app — review before sending.

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Eligibility criteria

Inclusion

Must have histologically confirmed FLC that is metastatic or unresectable.
Presence of DNAJB1-PRKACA fusion transcript, assessed by RNA-sequencing, DNA-sequencing, or in situ hybridization in the archival tissue.
Must have demonstrated radiographic progression on prior or current immunotherapy.
Age ≥ 12 years.
Patients \< 18 years old must have a body weight ≥ 40 kg.
ECOG (Eastern Cooperative Oncology Group) performance status of ≤2
Patients must have adequate organ and marrow function defined by study-specified laboratory tests.
Patients must have adequate kidney and liver function defined by study-specified laboratory tests.
Must have measurable disease per RECIST 1.1
Willingness to provide tissue and blood samples for mandatory translational research.
Women of childbearing potential (WOCBP) must have a negative urine or serum pregnancy test.
For both Women and Men, must use acceptable form of birth control while on study.
Ability to understand and willingness to sign a written informed consent document.

Exclusion

Patients with a history of prior treatment with checkpoint inhibitors, such as anti-PD-1, anti-PD-L1, anti-PD-L2, anti-CD137, anti-OX-40, anti-CD40, anti-CTLA-4, or anti-LAG-3 antibodies.
Must have had chemotherapy or other systemic therapy or radiotherapy, as follows:
Patients who have had chemotherapy, biological cancer therapy, or radiation 14 days prior to the first dose of study drug.
Patients who have had surgery within 28 days of dosing of investigational agent, excluding minor procedures.
Patients who have received other approved or investigational agents or device within 28 days of the first dose of study drug.
Patients who have not recovered from acute adverse events to grade ≤1 or baseline due to agents administered, with exception of grade 2 fatigue, rash, and endocrinopathy successfully managed hormone replacement therapy, or alopecia or stable neuropathy, unless approved by the IND Sponsor.
Patients who have received any non-oncology live vaccine therapy used for prevention of infectious diseases within 28 days of study treatment.
Known sensitivity to or history of allergic reactions attributed to compounds of similar chemical or biologic composition of polyinosinic-polycytidylic acid (Poly-ICLC) and/or DRP-104 and/or nivolumab and/or ipilimumab.
History of severe hypersensitivity reaction to any monoclonal antibody.
Has an active autoimmune disease.
Prior allogeneic stem cell transplantation or organ transplantation.
Has a diagnosis of immunodeficiency.
Systemic corticosteroids at immunosuppressive doses.
Subjects with interstitial lung disease that is symptomatic or may interfere with the detection or management of suspected drug-related pulmonary toxicity.
Has a pulse oximetry of \<92% on room air or is on supplemental home oxygen.
Active or untreated brain metastases or leptomeningeal metastases.
Uncontrolled intercurrent active medical and/or psychiatric illness/social psychosocial problems that that would limit compliance with study requirements.
Uncontrolled intercurrent illness including, but not limited to, uncontrolled infection, symptomatic congestive heart failure, unstable angina, cardiac arrhythmia, metastatic cancer, or psychiatric illness/social situations that would limit compliance with study requirements.
Pregnant or breastfeeding.
Has a known history of Human Immunodeficiency Virus (HIV)/AIDS.
Has active hepatitis B. Patients with chronic or acute HBV infection.
Have had evidence of active or acute diverticulitis, intra-abdominal abscess, or GI obstruction which are known risk factors for bowel perforation should be evaluated for the potential need for additional treatment before coming on study.
Unwilling or unable to follow the study schedule for any reason.
Patient is at the time of signing informed consent a regular user (including "recreational use") of any illicit drugs or had a recent history (within the last year) of substance abuse (including alcohol).
Evidence of clinical ascites.
Patients with QTc prolongation \> 470 ms according to Fridericia formula.
Patients receiving potent inducers of CYP 3A4/5 (including but not limited to apalutamide, carbamazepine, enzalutamide, mitotane, phenytoin, rifampin and St. John's Wort) who cannot safely discontinue drug at least 14 days prior to Cycle 1 Day 1.
Have had an allergen hyposensitization therapy within 2 weeks prior to initiation of study treatment.

What this trial measures

  • Number of participants experiencing grade 3 or above drug-related toxicities4 years

    When calculating the incidence of AEs, each AE (as defined by NCI CTCAE v6.0) will be counted only once for a given subject.

  • Objective response rate (ORR) using immune Response Evaluation Criteria for Solid Tumors (RECIST 1.1)4 years

    ORR is defined as the percentage of patients achieving a complete response (CR) or partial response (PR) to DRP-104 in combination with DRP-104, Nivolumab and Ipilimumab, based on the immune Response Evaluation Criteria in Solid Tumors (RECIST 1.1) at any time during the study. CR = disappearance of all target lesions, PR is =\>30percent decrease in sum of diameters of target lesions, progressive disease (PD) is \>20percent increase in sum of diameters of target lesions, stable disease (SD) is \<30percent decrease or \<20percent increase in sum of diameters of target lesions.