Actively Recruiting

Phase 2
Age: 0 - 22Years
All Genders
NCT06023641

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

Led by St. Jude Children's Research Hospital · Updated on 2025-08-19

135

Participants Needed

3

Research Sites

707 weeks

Total Duration

On this page

AI-Summary

What this Trial Is About

This 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.

CONDITIONS

Official Title

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

Who Can Participate

Age: 0 - 22Years
All Genders

Eligibility Criteria

Eligible

You may qualify if you...

  • Newly diagnosed with rhabdomyosarcoma of any subtype
  • Have low-, intermediate-, or high-risk disease as defined by specific genetic and staging criteria
  • Age less than 22 years old
  • Performance status ECOG 0, 1, or 2 (Lansky score used if younger than 16 years)
  • No prior radiotherapy or chemotherapy for rhabdomyosarcoma except emergency local treatment
  • Planned start of chemotherapy within 6 weeks of biopsy or surgery
  • Adequate bone marrow function (ANC  750/bcL, platelets  75,000/bcL)
  • Adequate liver function (total bilirubin less than 1.5 times upper limit of normal)
  • Adequate kidney function based on creatinine clearance or serum creatinine thresholds by age and sex
  • No evidence of dyspnea at rest and pulse oximetry over 94% if clinically indicated
  • No active, uncontrolled infection
  • Signed informed consent by participant or guardian
Not Eligible

You will not qualify if you...

  • Prior chemotherapy for rhabdomyosarcoma (excluding steroids)
  • Prior full course radiation at primary tumor site (except emergency radiation)
  • History or ongoing non-infectious interstitial lung disease requiring significant treatment
  • Sexually active patients of reproductive potential not agreeing to effective contraception during and 3 months after study
  • Pregnant females or those over 10 years/post-menarchal without negative pregnancy test
  • Lactating females who are breastfeeding or planning to breastfeed infants

AI-Screening

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Trial Site Locations

Total: 3 locations

1

Stanford University

Palo Alto, California, United States, 94304

Actively Recruiting

2

St. Jude Children's Research Hospital

Memphis, Tennessee, United States, 38105

Actively Recruiting

3

Cook Children's Medical Center

Fort Worth, Texas, United States, 76104-2796

Not Yet Recruiting

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Research Team

A

Alberto Pappo, MD

CONTACT

How is the study designed?

Study Type

INTERVENTIONAL

Masking

NONE

Allocation

NON_RANDOMIZED

Model

SINGLE_GROUP

Primary Purpose

TREATMENT

Number of Arms

3

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