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OncoMatch/Clinical Trials/NCT06023641

Treatment of Newly Diagnosed Rhabdomyosarcoma Using Molecular Risk Stratification and Liposomal Irinotecan Based Therapy in Children With Intermediate and High Risk Disease

Is NCT06023641 recruiting? Yes, currently enrolling (May 2026). This Phase 2 trial studies multiple treatments for rhabdomyosarcoma.

Phase 2RecruitingSt. Jude Children's Research HospitalNCT06023641Data as of May 2026

Treatment: Vincristine · Dactinomycin · Cyclophosphamide · Liposomal irinotecan · Vinorelbine · Temozolomide · Filgrastim, peg-filgrastimThis is a phase II study to determine safety and efficacy of combining liposomal irinotecan with vincristine alternating with VAC in intermediate-risk patients, liposomal irinotecan with temozolomide and vincristine alternating with VAC in high-risk patients and the chemotherapy combinations when given with concomitant radiation therapy in intermediate and high risk patients. Primary Objective * Estimate event-free survival for intermediate-risk participants treated with VAC and vincristine and liposomal irinotecan (VLI) with the addition of maintenance therapy with vinorelbine and cyclophosphamide. * Estimate the event-free survival for high-risk patients treated with VAC and vincristine, liposomal irinotecan, and temozolomide with the addition of maintenance therapy with vinorelbine and cyclophosphamide. Secondary Objectives * To assess the relation between pharmacogenetic variation in CEP72 genotype and vinca alkaloid (vincristine; vinorelbine) disposition in children with rhabdomyosarcoma. * To assess the relation between the pharmacogenetic variation in drug metabolizing enzymes and drug transporters, and the pharmacokinetics of vinca alkaloids, liposomal irinotecan, and cyclophosphamide in children with rhabdomyosarcoma. * To assess the extent of inter-patient variability in the pharmacokinetics of vinca alkaloids, liposomal irinotecan, and cyclophosphamide in children with rhabdomyosarcoma, and explore possible associations between drug disposition and patient specific covariates (e.g., age, sex, race, weight). * Estimate the cumulative incidence of local recurrence and overall 3-year event-free survival in patients with low-risk disease, intermediate-risk disease or high-risk disease treated with either no adjuvant radiation or minimal volume radiation and compare these outcomes with the outcomes achieved on RMS13.

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Extracted eligibility criteria

Cancer type

Rhabdomyosarcoma

Biomarker criteria

Required: FOXO1 fusion

alveolar, spindle cell/sclerosing FOXO1 fusion positive histology

Required: TP53 mutation

High-risk: All MYOD1 and TP53 mutant tumors regardless of stage and Group

Required: MYOD1 mutation

High-risk: All MYOD1 and TP53 mutant tumors regardless of stage and Group

Excluded: TP53 mutation

Low-risk: TP53 and MYOD1 negative

Excluded: MYOD1 mutation

Low-risk: TP53 and MYOD1 negative

Excluded: TP53 mutation

Intermediate-risk: MYOD1 and TP53 negative

Excluded: MYOD1 mutation

Intermediate-risk: MYOD1 and TP53 negative

Excluded: FOXO1 fusion

embryonal, congenital/infantile spindle cell, or spindle cell/sclerosing FOXO1 fusion negative histology

Disease stage

Required: Stage LOW-RISK: STAGE 1 GROUP I, GROUP II, LOW-RISK: STAGE 1 GROUP III ORBITAL ONLY, LOW-RISK: STAGE 2 GROUP I, GROUP II, INTERMEDIATE-RISK: STAGE 1 GROUP III NON ORBIT, INTERMEDIATE-RISK: STAGE 3 GROUP I/II, INTERMEDIATE-RISK: STAGE 2/3 GROUP III, INTERMEDIATE-RISK: STAGE 4 GROUP IV AND OBERLIN 0-1, INTERMEDIATE-RISK: STAGE 1-3, GROUP I-III N0, HIGH-RISK: GROUP IV ≥ 10 YEAR OF AGE AND OBERLIN ≥ 2, HIGH-RISK: N1, HIGH-RISK: STAGE 4 GROUP IV

See Appendices I and II for Staging and Clinical Grouping.

Performance status

ECOG OR LANSKY 0–2

Performance level corresponding to ECOG score of 0, 1, or 2. The Lansky performance score should be used for participants < 16 years

Prior therapy

No prior treatment (treatment-naive required)
Max 0 prior lines

Cannot have received: chemotherapy

Exception: excluding steroids; emergency local tumor treatment allowed with PI discussion

Participant has received no prior radiotherapy or chemotherapy for rhabdomyosarcoma (excluding steroids) unless an emergency situation requires local tumor treatment (discuss with PI).

Cannot have received: radiation therapy

Exception: emergent radiation allowed

Patients who have received prior full course RT at the primary site of disease. This does not exclude patients that received emergent radiation.

Lab requirements

Blood counts

Peripheral ANC ≥ 750/μL; Platelet count ≥ 75,000/μL (transfusion independent)

Kidney function

Creatinine clearance or radioisotope GFR > 70 mL/min/1.732 or serum creatinine based on age and sex; participants with urinary tract obstruction by tumor must have unimpeded urinary flow established via decompression

Liver function

total bilirubin < 1.5 x ULN for age; participants with biliary or hepatic primaries with bilirubin > 1.5 x ULN may be enrolled if all other eligibility criteria are met

Adequate bone marrow function defined as: Peripheral absolute neutrophil count (ANC) ≥ 750/μL; Platelet count ≥ 75,000/μL (transfusion independent); Adequate liver function defined as total bilirubin < 1.5 x upper limit of normal (ULN) for age. Participants with biliary or hepatic primaries with bilirubin values greater than 1.5 x ULN may be enrolled on study if all other eligibility criteria are met. Adequate renal function defined as: Creatinine clearance or radioisotope GFR > 70 mL/min/1.732 or serum creatinine based on age as follows: ... Participants with urinary tract obstruction by tumor must meet the renal function criteria listed above AND must have unimpeded urinary flow established via decompression of the obstructed portion of the urinary tract.

Structured fields extracted by AI. May contain errors — verify against the official protocol.

US trial sites

  • Stanford University · Palo Alto, California
  • St. Jude Children's Research Hospital · Memphis, Tennessee
  • Cook Children's Medical Center · Fort Worth, Texas

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