Actively Recruiting

Phase 2
All Genders
ID06861244

PNOC031: Protocol for Embryonal Tumor With Multilayered Rosettes (ETMR)

Led by University of California, San Francisco · Updated on 2026-04-23

70

Participants Needed

7

Research Sites

78 weeks

Total Duration

On this page

Sponsors

U

University of California, San Francisco

Lead Sponsor

P

Pediatric Neuro-Oncology Consortium

Collaborating Sponsor

AI-Summary

What this Trial Is About

Researchers are evaluating a treatment approach for children with newly diagnosed Embryonal Tumor With Multilayered Rosettes (ETMR). This open-label study investigates the use of induction chemotherapy, high-dose chemotherapy, and focal radiation therapy to improve outcomes. The study focuses on participants with both gross-totally resected, non-metastatic tumors and those with incompletely resected or metastatic disease, aiming to better understand survival rates and disease progression. Participants will be assigned to one of four groups based on their surgical results and metastatic status. Those with gross total resection and no metastasis receive either standard induction chemotherapy with early focal radiotherapy or high-dose chemotherapy with stem cell rescue, optionally followed by radiotherapy. Participants with metastatic or residual disease undergo similar chemotherapy regimens with or without early radiotherapy. Treatments last between 12 to 24 weeks depending on the group, with follow-up periods ranging from two to five years. Throughout the study, participants will have tumor tissue, blood, and cerebrospinal fluid collected for research. They will be monitored for progression-free survival and overall survival, with assessments continuing up to two years for some groups and up to five years for others. The study also aims to validate blood and cerebrospinal fluid markers and explore the genetic makeup of ETMR. Safety and treatment response are tracked according to a Pediatric Neuro-Oncology Consortium protocol until withdrawal or death.

CONDITIONS

Brief Title

Embryonal Tumor With Multilayered Rosettes

Who Can Participate

All Genders

Eligibility Criteria

Eligible

You may qualify if you...

  • Participants must have a molecularly or histologically confirmed embryonal tumor with multilayered rosettes.
  • Tumor tissue for enrollment requires at least 10-20 unstained slides or one block with at least 40% tumor content.
  • Cohort 1 participants must not have received any prior tumor-directed therapy other than surgery.
  • Cohort 2 and 3 participants may have had prior therapy but need approval from study chairs.
  • No prior radiation treatment for tumor is allowed.
  • Participants of any age are eligible.
  • Participants should start induction chemotherapy within 28 days of their most recent surgery.
  • Cohort 1: gross-total resection, eligible for early radiotherapy, no metastatic disease.
  • Cohort 2: gross-total resection, eligible for high-dose chemotherapy, no metastatic disease.
  • Cohort 3A: metastatic or residual disease, early radiotherapy.
  • Cohort 3B: metastatic or residual disease, high-dose chemotherapy.
  • Radiotherapy age criteria: over 12 months for infratentorial tumor or over 15 months for supratentorial tumor.
  • Karnofsky performance score 50 or higher for participants over 16 years old; Lansky score 50 or higher for participants 16 or younger.
  • Adequate organ function with specified blood counts, renal, liver, and neurologic function.
  • Participants with well-controlled seizure disorders may be eligible.
  • Women and men of child-bearing potential must use effective contraception during and 4 months after study therapy.
  • Participants must enroll in PNOC COMP if available before enrolling in this study.
  • Legal guardian or participant must be able to provide informed consent or assent.
Not Eligible

You will not qualify if you...

  • Cohort 1 participants who have received prior tumor-directed therapy other than surgery.
  • Participants receiving any other investigational tumor therapies.
  • History of allergic reactions to similar drugs used in the study.
  • Uncontrolled illnesses that could interfere with participation.
  • Women who are pregnant or breastfeeding and of childbearing potential.

AI-Screening

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Your Study Journey

Screening

Duration - 2 to 4 weeks

Participants are screened for eligibility to participate in the trial.

1 visit (in-person)

Surgery

Duration - Variable depending on recovery

Participants undergo tumor resection surgery as part of planned care before enrollment.

1 surgical hospitalization

Treatment

Duration - 12 to 24 weeks depending on cohort

Participants receive induction chemotherapy and may receive early focal radiotherapy or high-dose chemotherapy depending on cohort assignment.

Multiple visits weekly during chemotherapy and radiotherapy

Follow-up

Duration - Up to 5 years

Participants are followed for up to 2 years (Cohorts 1 and 2) or up to 5 years (Cohorts 3A and 3B) to assess progression-free and overall survival.

Regular follow-up visits as per Pediatric Neuro-Oncology Consortium (PNOC) COMP protocol

Trial Site Locations

Total: 7 locations

1

University of Alabama at Birmingham

Birmingham, Alabama, United States, 35233

Actively Recruiting

2

University of California, San Francisco

San Francisco, California, United States, 94143

Actively Recruiting

3

Riley Hospital for Children at Indiana University Health

Indianapolis, Indiana, United States, 46202

Actively Recruiting

4

Johns Hopkins University

Baltimore, Maryland, United States, 21218

Actively Recruiting

5

Washington University in St. Louis

St Louis, Missouri, United States, 63130

Actively Recruiting

6

Hackensack University Medical Center

Hackensack, New Jersey, United States, 07601

Actively Recruiting

7

St. Jude Children's Research Hospital

Memphis, Tennessee, United States, 38105

Actively Recruiting

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Research Team

P

PNOC Operations Office

How is the study designed?

Study Type

INTERVENTIONAL

Masking

NONE

Allocation

NON_RANDOMIZED

Model

PARALLEL

Primary Purpose

TREATMENT

Number of Arms

4

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