Embryonal Tumor With Multilayered Rosettes

NCT ID: NCT06861244

Last Updated: 2026-01-07

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Clinical Phase

PHASE2

Total Enrollment

70 participants

Study Classification

INTERVENTIONAL

Study Start Date

2025-03-06

Study Completion Date

2032-03-31

Brief Summary

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This is an open-label, comprehensive, iterative investigation of evaluating the use of induction chemotherapy, high-dose chemotherapy, and focal radiation therapy in children with newly diagnosed Embryonal Tumor With Multilayered Rosettes (ETMR).

Detailed Description

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PRIMARY OBJECTIVES I. To determine the six-month progression-free survival (PFS6) of participants with newly diagnosed, gross-totally resected, non-metastatic ETMR, treated using a regimen of induction chemotherapy and early focal radiotherapy (Cohort 1)

SECONDARY OBJECTIVES I. To determine the two-year progression-free survival (PFS) and overall survival (OS) of participants with newly diagnosed, gross-totally resected, non-metastatic ETMR (Cohort 1).

II. To determine the two-year progression-free survival (PFS) and overall survival (OS) of participants with newly diagnosed, gross-totally resected, non-metastatic ETMR (Cohort 2).

III. To determine the two-year progression-free survival (PFS), overall survival (OS) and objective response rate of participants with newly diagnosed, incompletely resected and/or metastatic ETMR (Cohort 3A and 3B)

EXPLORATORY OBJECTIVES:

I. To validate the utility of a liquid miRNA biomarker in blood and Cerebral spinal fluid (CSF) as a correlative marker of a participant's disease status.

II. To better define the genomic landscape of ETMR.

OUTLINE:

Participants with newly diagnosed ETMR will obtain either gross total, or sub-total resection surgery prior to enrollment. After surgery, participants will be assigned to 1 of 4 possible cohorts:

Cohorts 1 and 2: Participants with newly diagnosed, gross-totally resected, non-metastatic ETMR.

Cohorts 3A and 3B: Participants with newly diagnosed, incompletely resected and/or metastatic ETMR.

Participants will be assessed for survival outcomes for up to 2 years. Follow-up procedures are to be captured under the PNOC COMP protocol. Participants will be followed under the Pediatric Neuro-Oncology Consortium (PNOC) COMP protocol until death or withdrawal from study.

Conditions

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Embryonal Tumor With Multilayered Rosettes Embryonal Tumor With Multilayered Rosettes, Nos

Study Design

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Allocation Method

NON_RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Cohort 1: Gross-total resection, non-metastatic, early radiotherapy

Participants will undergo gross total resection of the tumor prior to enrollment into this cohort. Standard dose induction chemotherapy and 6-weeks of early focal radiotherapy, followed by and a second standard dose induction chemotherapy for a total of 12 weeks of chemotherapy; 18 weeks of treatments in all. Participants will be followed for up to 2 years.

Group Type EXPERIMENTAL

Non-Investigational Surgical Resection

Intervention Type PROCEDURE

Undergo surgery directly before study enrollment as part of planned care.

Temozolomide

Intervention Type DRUG

Participants assigned to or whom receive optional RT will receive concurrent temozolomide

Tumor Tissue Sample

Intervention Type PROCEDURE

Tumor tissue will be collected for correlative studies

Blood Sample

Intervention Type PROCEDURE

Blood samples will be collected for correlative studies

Cerebrospinal Fluid (CSF) Sample

Intervention Type PROCEDURE

CSF samples will be collected for correlative studies

Radiotherapy (RT)

Intervention Type RADIATION

Undergo RT

Chemotherapy Drug, Cancer - Physician's Choice

Intervention Type DRUG

One or more of the following may be assigned by the physician (physician's choice) per standard of care guidelines upon study enrollment following surgery: Cytarabine, Carboplatin, Cisplatin, Vincristine Sulfate injection (Vincristine PFS), Topotecan Hydrochloride, Dactinomycin, Thiotepa, Filgrastim, Cyclophosphamide, or Doxorubicin Hydrochloride. Not all participants will receive all possible drug regimens.

Cohort 2: Gross-total resection, non-metastatic, high-dose chemotherapy

Participants will undergo gross total resection of the tumor prior to enrollment into this cohort. Participants will receive 6 weeks of induction chemotherapy and 3 cycles (approximately 4 weeks each) of high-dose chemotherapy with stem cell rescue and will have the option to receive radiotherapy at the completion of therapy, for a total of 18-24 weeks. Participants will be followed for up to 2 years.

Group Type EXPERIMENTAL

Non-Investigational Surgical Resection

Intervention Type PROCEDURE

Undergo surgery directly before study enrollment as part of planned care.

Temozolomide

Intervention Type DRUG

Participants assigned to or whom receive optional RT will receive concurrent temozolomide

Tumor Tissue Sample

Intervention Type PROCEDURE

Tumor tissue will be collected for correlative studies

Blood Sample

Intervention Type PROCEDURE

Blood samples will be collected for correlative studies

Cerebrospinal Fluid (CSF) Sample

Intervention Type PROCEDURE

CSF samples will be collected for correlative studies

Radiotherapy (RT)

Intervention Type RADIATION

Undergo RT

Chemotherapy Drug, Cancer - Physician's Choice

Intervention Type DRUG

One or more of the following may be assigned by the physician (physician's choice) per standard of care guidelines upon study enrollment following surgery: Cytarabine, Carboplatin, Cisplatin, Vincristine Sulfate injection (Vincristine PFS), Topotecan Hydrochloride, Dactinomycin, Thiotepa, Filgrastim, Cyclophosphamide, or Doxorubicin Hydrochloride. Not all participants will receive all possible drug regimens.

Cohort 3A: Metastatic or residual disease, early radiotherapy

Participants metastatic disease or residual disease following their initial surgical interventions prior to enrollment into this cohort will receive standard dose induction chemotherapy and 6-weeks of early focal radiotherapy, followed by and a second standard dose induction chemotherapy for a total of 12 weeks of chemotherapy; 18 weeks of treatments in all. Participants will be followed for up to 5 years.

Group Type EXPERIMENTAL

Non-Investigational Surgical Resection

Intervention Type PROCEDURE

Undergo surgery directly before study enrollment as part of planned care.

Temozolomide

Intervention Type DRUG

Participants assigned to or whom receive optional RT will receive concurrent temozolomide

Tumor Tissue Sample

Intervention Type PROCEDURE

Tumor tissue will be collected for correlative studies

Blood Sample

Intervention Type PROCEDURE

Blood samples will be collected for correlative studies

Cerebrospinal Fluid (CSF) Sample

Intervention Type PROCEDURE

CSF samples will be collected for correlative studies

Radiotherapy (RT)

Intervention Type RADIATION

Undergo RT

Chemotherapy Drug, Cancer - Physician's Choice

Intervention Type DRUG

One or more of the following may be assigned by the physician (physician's choice) per standard of care guidelines upon study enrollment following surgery: Cytarabine, Carboplatin, Cisplatin, Vincristine Sulfate injection (Vincristine PFS), Topotecan Hydrochloride, Dactinomycin, Thiotepa, Filgrastim, Cyclophosphamide, or Doxorubicin Hydrochloride. Not all participants will receive all possible drug regimens.

Cohort 3B: Metastatic or residual disease, high-dose chemotherapy

Participants metastatic disease or residual disease following their initial surgical interventions prior to enrollment into this cohort. Participants will receive 6 weeks of induction chemotherapy and 3 cycles (approximately 4 weeks each) of high-dose chemotherapy with stem cell rescue and will have the option to receive radiotherapy at the completion of therapy, for a total of 18-24 weeks. Participants will be followed for up to 5 years.

Group Type EXPERIMENTAL

Non-Investigational Surgical Resection

Intervention Type PROCEDURE

Undergo surgery directly before study enrollment as part of planned care.

Temozolomide

Intervention Type DRUG

Participants assigned to or whom receive optional RT will receive concurrent temozolomide

Tumor Tissue Sample

Intervention Type PROCEDURE

Tumor tissue will be collected for correlative studies

Blood Sample

Intervention Type PROCEDURE

Blood samples will be collected for correlative studies

Cerebrospinal Fluid (CSF) Sample

Intervention Type PROCEDURE

CSF samples will be collected for correlative studies

Radiotherapy (RT)

Intervention Type RADIATION

Undergo RT

Chemotherapy Drug, Cancer - Physician's Choice

Intervention Type DRUG

One or more of the following may be assigned by the physician (physician's choice) per standard of care guidelines upon study enrollment following surgery: Cytarabine, Carboplatin, Cisplatin, Vincristine Sulfate injection (Vincristine PFS), Topotecan Hydrochloride, Dactinomycin, Thiotepa, Filgrastim, Cyclophosphamide, or Doxorubicin Hydrochloride. Not all participants will receive all possible drug regimens.

Interventions

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Non-Investigational Surgical Resection

Undergo surgery directly before study enrollment as part of planned care.

Intervention Type PROCEDURE

Temozolomide

Participants assigned to or whom receive optional RT will receive concurrent temozolomide

Intervention Type DRUG

Tumor Tissue Sample

Tumor tissue will be collected for correlative studies

Intervention Type PROCEDURE

Blood Sample

Blood samples will be collected for correlative studies

Intervention Type PROCEDURE

Cerebrospinal Fluid (CSF) Sample

CSF samples will be collected for correlative studies

Intervention Type PROCEDURE

Radiotherapy (RT)

Undergo RT

Intervention Type RADIATION

Chemotherapy Drug, Cancer - Physician's Choice

One or more of the following may be assigned by the physician (physician's choice) per standard of care guidelines upon study enrollment following surgery: Cytarabine, Carboplatin, Cisplatin, Vincristine Sulfate injection (Vincristine PFS), Topotecan Hydrochloride, Dactinomycin, Thiotepa, Filgrastim, Cyclophosphamide, or Doxorubicin Hydrochloride. Not all participants will receive all possible drug regimens.

Intervention Type DRUG

Other Intervention Names

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Focal Radiotherapy Radiation Therapy Standard of Care, High dose Chemotherapy Standard of Care Surgical Resection Standard of Care Surgical Resection of Tumor Temodar Tumor Specimen Blood Specimen CSF Specimen

Eligibility Criteria

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Inclusion Criteria

1. Participants must have either a molecularly or histologically confirmed embryonal tumor with multilayered rosettes.
2. For enrollment, a confirmation of a minimum of 10-20 unstained formalin-fixed paraffin-embedded (FFPE) slides or 1 block (15-20 mg) with tumor content of 40% or greater is required. Anything less must be discussed and approved by the study chairs prior to enrollment.
3. Prior Therapy:

1. Cohort 1 participants must not have received any prior tumor-directed therapy other than surgical resection.
2. Cohort 2 and 3 participants may receive tumor-directed therapy prior to enrollment. These participants must be discussed with study chairs prior to enrollment.
4. Participants must not have received prior radiation for treatment of tumor.
5. Participants of any age are eligible.
6. Participants should begin induction chemotherapy within 28 days of the most recent definitive surgical procedure. Participants beginning therapy beyond 28 days from surgery, will need to discuss with study chairs.
7. Cohort specific eligibility

1. Cohort 1: Gross-total resection, Eligible for early radiotherapy (please see age criteria below), and no evidence of metastatic disease.
2. Cohort 2: Gross-total resection, high dose chemotherapy (please see age criteria below), and no evidence of metastatic disease.
3. Cohort 3A: Metastatic or residual disease, and early radiotherapy.
4. Cohort 3B: Metastatic or residual disease, and high dose chemotherapy.
5. Radiotherapy Age Criteria (at the time of planned radiation): \>12 months of age for participants with infratentorial tumor OR \>15 months of age for participants with supratentorial tumor. For participants being treated on radiotherapy-containing arms, the legal parent/guardian or patient and the physician must be willing to allow the use of radiotherapy for treatment.
8. Performance Score: Karnofsky \>= 50 for participants \> 16 years of age and Lansky \>= 50 for participants \<=16 years of age. Participants who are unable to walk because of paralysis, but who are up in a wheelchair, will be considered ambulatory for the purpose of assessing the performance score.
9. Organ Function Requirements:

1. Peripheral absolute neutrophil count (ANC) \> 75,000/mm3
2. Platelet count \> 75,000/mm3 (transfusion independent, defined as not receiving platelet transfusions for at least 72 hours prior to enrollment).
10. Adequate Renal Function defined as:

a. Serum creatinine \< 1.5 x upper limit normal (ULN) based on age and gender.
11. Adequate Liver Function defined as:

1. Total bilirubin \< 1.5 x upper limit of normal (ULN) for age; in presence of Gilbert's syndrome, total bilirubin \< 3 x ULN or direct bilirubin \< 1.5 x ULN,
2. alanine aminotransferase (ALT) \< 3 x ULN,
3. aspartate aminotransferase (AST) \< 3 x ULN,
12. Adequate Neurologic Function defined as:

a. Participants with seizure disorder may be enrolled if well controlled. Participants on enzyme inducing anticonvulsants may be excluded pending interaction(s) with study drugs.
13. As chemotherapeutic agents used in this trial are known to be teratogenic, women and men of child-bearing potential must agree to use adequate contraception (hormonal or barrier method of birth control; abstinence) prior to study entry, for the duration of study participation and 4 months after completion of study therapy. Should a woman become pregnant or suspect she is pregnant while she or her partner is participating in this study, she should inform her treating physician immediately.
14. Participants must be enrolled on PNOC COMP prior to enrollment on PNOC031 if PNOC COMP is open to accrual at the enrolling institution.
15. A legal parent/guardian or patient must be able to understand, and willing to sign, a written informed consent and assent document, as appropriate.

Exclusion Criteria

1. Cohort 1 only: Participants who have received any prior tumor-directed therapy other than surgical intervention
2. Participants who are receiving any other tumor directed investigational agents.
3. History of allergic reactions attributed to compounds of similar chemical or biologic composition to the agents used in study.
4. Uncontrolled intercurrent illness.
5. Women of childbearing potential must not be pregnant or breast-feeding.
Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Pediatric Neuro-Oncology Consortium

OTHER

Sponsor Role collaborator

Solving Kids' Cancer

OTHER

Sponsor Role collaborator

University of California, San Francisco

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Sabine Mueller, MD, PhD, MAS

Role: PRINCIPAL_INVESTIGATOR

University of California, San Francisco

Derek Hanson, MD

Role: STUDY_CHAIR

Hackensack Meridian Health

Locations

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University of Alabama at Birmingham

Birmingham, Alabama, United States

Site Status RECRUITING

University of California, San Francisco

San Francisco, California, United States

Site Status RECRUITING

Johns Hopkins University

Baltimore, Maryland, United States

Site Status RECRUITING

Washington University in St. Louis

St Louis, Missouri, United States

Site Status RECRUITING

Countries

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United States

Central Contacts

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PNOC Operations

Role: CONTACT

415-502-1600

Facility Contacts

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Girish Dhall, MD

Role: primary

205-638-6194

Laura K Metrock, MD

Role: backup

205-638-9285

Madina Halim

Role: primary

415-502-1600

Role: backup

877-827-3222

Kenneth Cohen, MD, MBA

Role: primary

410-614-5055

Robyn Gartrell, MD, MS

Role: backup

410-955-2548

Michael Angelo Huang, MD

Role: primary

314-362-4563

Mohamed AbdelBaki, MD

Role: backup

(314) 454-6018

Other Identifiers

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NCI-2025-01764

Identifier Type: REGISTRY

Identifier Source: secondary_id

240830

Identifier Type: -

Identifier Source: org_study_id

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