MedPath

FaR-RMS: An Overarching Study for Children and Adults With Frontline and Relapsed RhabdoMyoSarcoma

Registration Number
NCT04625907
Lead Sponsor
University of Birmingham
Brief Summary

FaR-RMS is an over-arching study for children and adults with newly diagnosed and relapsed rhabdomyosarcoma (RMS)

Detailed Description

FaR-RMS is an over-arching study for children and adults with newly diagnosed and relapsed rhabdomyosarcoma (RMS). It is a multi-arm, multi-stage format, involving several different trial questions. FaR-RMS is intended to be a rolling programme of research with new treatment arms being introduced dependant on emerging data and innovation. This study has multiple aims. It aims to evaluate the impact of new agent regimens in both newly diagnosed and relapsed RMS; whether changing the duration of maintenance therapy affects outcome; and whether changes to dose, extent (in metastatic disease) and timing of radiotherapy improve outcome and quality of life. In addition the study will evaluate risk stratification through the use of PAX-FOXO1 fusion gene status instead of histological subtyping and explore the use of FDG PET-CT response assessment as a prognostic biomarker for outcome following induction chemotherapy.

Newly diagnosed patients should, where possible, be entered into the FaR-RMS study at the time of first diagnosis prior to receiving any chemotherapy. However, patients can enter at the point of radiotherapy or maintenance, and those with relapsed disease can enter the study even if not previously entered at initial diagnosis. Patients may be entered into more than one randomisation/registration, dependant on patient risk group and disease status.

Recruitment & Eligibility

Status
RECRUITING
Sex
All
Target Recruitment
1672
Inclusion Criteria

Not provided

Exclusion Criteria

Not provided

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
RT1B: Radiotherapy for resectable disease: standard doseradiotherapyTo receive 41.4 Gy
RT1A: Post operative radiotherapyradiotherapyTo be given either 41.4 Gy or 50.4 Gy following surgery
RT1B: Radiotherapy for resectable disease: dose escalatedradiotherapyTo receive 50.4 Gy
RT1C: Radiotherapy for unresectable disease: standard doseradiotherapyTo receive 50.4 Gy
RT2: Radiotherapy to primary tumour and involved lymph nodesradiotherapyRadiotherapy to the primary tumour and involved regional lymph nodes only
RT2: Radiotherapy to all metastatic sitesradiotherapyRadiotherapy given to all metastatic sites
RT1A: Preoperative RadiotherapyradiotherapyTo be given either 41.4 Gy or 50.4 Gy prior to surgery
RT1C: Radiotherapy for unresectable disease: dose escalatedradiotherapyTo receive 59.4 Gy
CT3: Relpased Chemotherapy - VIRTIrinotecanVincristine: 1.5 mg/m2 As per local practice: recommended as a short infusion (maximum dose 2mg) on days 1 and 8 Irinotecan: 50 mg/m2 as an i.v. infusion over 1 hour on days 1-5 Temozolomide: 125 mg/m2 (Escalate to 150mg/m2/day in Cycle 2 if no toxicity \> grade 3) as an oral tablets prior to vincristine and irinotecan on days 1-5
Phase 1b Dose finding: VHR induction - IRIVAIrinotecanIrinotecan: an i.v. infusion over 1 hour on days 8,9,10,11 and 12 . For the phase 1b registration, starting dose of 20 mg/m2. Ifosfamide: 3g/m2 as an i.v. infusion over 3 hours on days 1 and 2 Vincristine: 1.5 mg/m2 as an As per local practice: recommended as a short infusion (maximum dose 2mg). Administered days 1,8,15 on cycles 1-2 and on days 1 and 8 on cycles 3-9. Actinomycin: 1.5 mg/m2 as an i.v. bolus injection (maximum dose 2mg) on day 1.
Phase 1b Dose finding: VHR induction - IRIVAActinomycin DIrinotecan: an i.v. infusion over 1 hour on days 8,9,10,11 and 12 . For the phase 1b registration, starting dose of 20 mg/m2. Ifosfamide: 3g/m2 as an i.v. infusion over 3 hours on days 1 and 2 Vincristine: 1.5 mg/m2 as an As per local practice: recommended as a short infusion (maximum dose 2mg). Administered days 1,8,15 on cycles 1-2 and on days 1 and 8 on cycles 3-9. Actinomycin: 1.5 mg/m2 as an i.v. bolus injection (maximum dose 2mg) on day 1.
Phase 1b Dose finding: VHR induction - IRIVAIfosfamideIrinotecan: an i.v. infusion over 1 hour on days 8,9,10,11 and 12 . For the phase 1b registration, starting dose of 20 mg/m2. Ifosfamide: 3g/m2 as an i.v. infusion over 3 hours on days 1 and 2 Vincristine: 1.5 mg/m2 as an As per local practice: recommended as a short infusion (maximum dose 2mg). Administered days 1,8,15 on cycles 1-2 and on days 1 and 8 on cycles 3-9. Actinomycin: 1.5 mg/m2 as an i.v. bolus injection (maximum dose 2mg) on day 1.
Phase 1b Dose finding: VHR induction - IRIVAVincristineIrinotecan: an i.v. infusion over 1 hour on days 8,9,10,11 and 12 . For the phase 1b registration, starting dose of 20 mg/m2. Ifosfamide: 3g/m2 as an i.v. infusion over 3 hours on days 1 and 2 Vincristine: 1.5 mg/m2 as an As per local practice: recommended as a short infusion (maximum dose 2mg). Administered days 1,8,15 on cycles 1-2 and on days 1 and 8 on cycles 3-9. Actinomycin: 1.5 mg/m2 as an i.v. bolus injection (maximum dose 2mg) on day 1.
CT1A: VHR induction - IVADOActinomycin DIfosfamide: 3g/m2 as an i.v. infusion over 3 hours on days 1 and 2 Vincristine: 1.5 mg/m2 As per local practice: recommended as a short infusion (maximum dose 2mg). Administered days 1,8,15 on cycles 1-2 and on day 1 on cycles 3-9. Actinomycin: 1.5 mg/m2 as an i.v. bolus injection (maximum dose 2mg) on day 1. Doxorubicin: 30 mg/m2 as an i.v infusion over 1 hour on days 1 and 2 on cycles 1-4
CT1A: VHR induction - IVADODoxorubicinIfosfamide: 3g/m2 as an i.v. infusion over 3 hours on days 1 and 2 Vincristine: 1.5 mg/m2 As per local practice: recommended as a short infusion (maximum dose 2mg). Administered days 1,8,15 on cycles 1-2 and on day 1 on cycles 3-9. Actinomycin: 1.5 mg/m2 as an i.v. bolus injection (maximum dose 2mg) on day 1. Doxorubicin: 30 mg/m2 as an i.v infusion over 1 hour on days 1 and 2 on cycles 1-4
CT1A: VHR induction - IVADOIfosfamideIfosfamide: 3g/m2 as an i.v. infusion over 3 hours on days 1 and 2 Vincristine: 1.5 mg/m2 As per local practice: recommended as a short infusion (maximum dose 2mg). Administered days 1,8,15 on cycles 1-2 and on day 1 on cycles 3-9. Actinomycin: 1.5 mg/m2 as an i.v. bolus injection (maximum dose 2mg) on day 1. Doxorubicin: 30 mg/m2 as an i.v infusion over 1 hour on days 1 and 2 on cycles 1-4
CT1A: VHR Induction IRIVAActinomycin DIrinotecan: an i.v. infusion over 1 hour on days 8,9,10,11 and 12 . Phase 2 recommended dose as determined by IRIVA dose finding arm Ifosfamide: 3g/m2 as an i.v. infusion over 3 hours on days 1 and 2 Vincristine: 1.5 mg/m2 As per local practice: recommended as a short infusion (maximum dose 2mg). Administered days 1,8,15 on cycles 1-2 and on days 1 and 8 on cycles 3-9. Actinomycin: 1.5 mg/m2 as an i.v. bolus injection (maximum dose 2mg) on day 1.
CT1A: VHR Induction IRIVAIrinotecanIrinotecan: an i.v. infusion over 1 hour on days 8,9,10,11 and 12 . Phase 2 recommended dose as determined by IRIVA dose finding arm Ifosfamide: 3g/m2 as an i.v. infusion over 3 hours on days 1 and 2 Vincristine: 1.5 mg/m2 As per local practice: recommended as a short infusion (maximum dose 2mg). Administered days 1,8,15 on cycles 1-2 and on days 1 and 8 on cycles 3-9. Actinomycin: 1.5 mg/m2 as an i.v. bolus injection (maximum dose 2mg) on day 1.
CT1A: VHR Induction IRIVAIfosfamideIrinotecan: an i.v. infusion over 1 hour on days 8,9,10,11 and 12 . Phase 2 recommended dose as determined by IRIVA dose finding arm Ifosfamide: 3g/m2 as an i.v. infusion over 3 hours on days 1 and 2 Vincristine: 1.5 mg/m2 As per local practice: recommended as a short infusion (maximum dose 2mg). Administered days 1,8,15 on cycles 1-2 and on days 1 and 8 on cycles 3-9. Actinomycin: 1.5 mg/m2 as an i.v. bolus injection (maximum dose 2mg) on day 1.
CT1A: VHR induction - IVADOVincristineIfosfamide: 3g/m2 as an i.v. infusion over 3 hours on days 1 and 2 Vincristine: 1.5 mg/m2 As per local practice: recommended as a short infusion (maximum dose 2mg). Administered days 1,8,15 on cycles 1-2 and on day 1 on cycles 3-9. Actinomycin: 1.5 mg/m2 as an i.v. bolus injection (maximum dose 2mg) on day 1. Doxorubicin: 30 mg/m2 as an i.v infusion over 1 hour on days 1 and 2 on cycles 1-4
CT1A: VHR Induction IRIVAVincristineIrinotecan: an i.v. infusion over 1 hour on days 8,9,10,11 and 12 . Phase 2 recommended dose as determined by IRIVA dose finding arm Ifosfamide: 3g/m2 as an i.v. infusion over 3 hours on days 1 and 2 Vincristine: 1.5 mg/m2 As per local practice: recommended as a short infusion (maximum dose 2mg). Administered days 1,8,15 on cycles 1-2 and on days 1 and 8 on cycles 3-9. Actinomycin: 1.5 mg/m2 as an i.v. bolus injection (maximum dose 2mg) on day 1.
CT1B: HR Induction IVAIfosfamideIfosfamide: 3g/m2 as an i.v. infusion over 3 hours on days 1 and 2 Vincristine: 1.5 mg/m2 As per local practice: recommended as a short infusion (maximum dose 2mg). Administered days 1,8,15 on cycles 1-2 and on day 1 on cycles 3-9. Actinomycin: 1.5 mg/m2 as an i.v. bolus injection (maximum dose 2mg) on day 1.
CT1B: HR Induction IVAActinomycin DIfosfamide: 3g/m2 as an i.v. infusion over 3 hours on days 1 and 2 Vincristine: 1.5 mg/m2 As per local practice: recommended as a short infusion (maximum dose 2mg). Administered days 1,8,15 on cycles 1-2 and on day 1 on cycles 3-9. Actinomycin: 1.5 mg/m2 as an i.v. bolus injection (maximum dose 2mg) on day 1.
CT1B: HR Induction IVAVincristineIfosfamide: 3g/m2 as an i.v. infusion over 3 hours on days 1 and 2 Vincristine: 1.5 mg/m2 As per local practice: recommended as a short infusion (maximum dose 2mg). Administered days 1,8,15 on cycles 1-2 and on day 1 on cycles 3-9. Actinomycin: 1.5 mg/m2 as an i.v. bolus injection (maximum dose 2mg) on day 1.
CT1B: HR Induction IRIVAIrinotecanIrinotecan: an i.v. infusion over 1 hour on days 8,9,10,11 and 12 . Phase 2 recommended dose as determined by IRIVA dose finding arm Ifosfamide: 3g/m2 as an i.v. infusion over 3 hours on days 1 and 2 Vincristine: 1.5 mg/m2 As per local practice: recommended as a short infusion (maximum dose 2mg). Administered days 1,8,15 on cycles 1-2 and on days 1 and 8 on cycles 3-9. Actinomycin: 1.5 mg/m2 as an i.v. bolus injection (maximum dose 2mg) on day 1.
CT1B: HR Induction IRIVAActinomycin DIrinotecan: an i.v. infusion over 1 hour on days 8,9,10,11 and 12 . Phase 2 recommended dose as determined by IRIVA dose finding arm Ifosfamide: 3g/m2 as an i.v. infusion over 3 hours on days 1 and 2 Vincristine: 1.5 mg/m2 As per local practice: recommended as a short infusion (maximum dose 2mg). Administered days 1,8,15 on cycles 1-2 and on days 1 and 8 on cycles 3-9. Actinomycin: 1.5 mg/m2 as an i.v. bolus injection (maximum dose 2mg) on day 1.
CT1B: HR Induction IRIVAVincristineIrinotecan: an i.v. infusion over 1 hour on days 8,9,10,11 and 12 . Phase 2 recommended dose as determined by IRIVA dose finding arm Ifosfamide: 3g/m2 as an i.v. infusion over 3 hours on days 1 and 2 Vincristine: 1.5 mg/m2 As per local practice: recommended as a short infusion (maximum dose 2mg). Administered days 1,8,15 on cycles 1-2 and on days 1 and 8 on cycles 3-9. Actinomycin: 1.5 mg/m2 as an i.v. bolus injection (maximum dose 2mg) on day 1.
CT1B: HR Induction IRIVAIfosfamideIrinotecan: an i.v. infusion over 1 hour on days 8,9,10,11 and 12 . Phase 2 recommended dose as determined by IRIVA dose finding arm Ifosfamide: 3g/m2 as an i.v. infusion over 3 hours on days 1 and 2 Vincristine: 1.5 mg/m2 As per local practice: recommended as a short infusion (maximum dose 2mg). Administered days 1,8,15 on cycles 1-2 and on days 1 and 8 on cycles 3-9. Actinomycin: 1.5 mg/m2 as an i.v. bolus injection (maximum dose 2mg) on day 1.
CT2A: VHR Maintenance - VCVinorelbineVinorelbine: 25 mg/m2 i.v. or 60 mg/m2 orally on days 1,8 and 15 Cyclophosphamide 25 mg/m2 orally daily for 28 days
CT2A: VHR Maintenance - VCCyclophosphamideVinorelbine: 25 mg/m2 i.v. or 60 mg/m2 orally on days 1,8 and 15 Cyclophosphamide 25 mg/m2 orally daily for 28 days
CT3: Relpased Chemotherapy - VIRTVincristineVincristine: 1.5 mg/m2 As per local practice: recommended as a short infusion (maximum dose 2mg) on days 1 and 8 Irinotecan: 50 mg/m2 as an i.v. infusion over 1 hour on days 1-5 Temozolomide: 125 mg/m2 (Escalate to 150mg/m2/day in Cycle 2 if no toxicity \> grade 3) as an oral tablets prior to vincristine and irinotecan on days 1-5
CT2B: HR Maintenance - VCVinorelbineVinorelbine: 25 mg/m2 i.v. on days 1,8 and 15 Cyclophosphamide 25 mg/m2 orally daily for 28 days
CT2B: HR Maintenance - VCCyclophosphamideVinorelbine: 25 mg/m2 i.v. on days 1,8 and 15 Cyclophosphamide 25 mg/m2 orally daily for 28 days
CT3: Relpased Chemotherapy - VIRTTemozolomideVincristine: 1.5 mg/m2 As per local practice: recommended as a short infusion (maximum dose 2mg) on days 1 and 8 Irinotecan: 50 mg/m2 as an i.v. infusion over 1 hour on days 1-5 Temozolomide: 125 mg/m2 (Escalate to 150mg/m2/day in Cycle 2 if no toxicity \> grade 3) as an oral tablets prior to vincristine and irinotecan on days 1-5
CT3: Relapsed Chemotherapy - VIRRIrinotecanVincristine: 1.5 mg/m2 As per local practice: recommended as a short infusion (maximum dose 2mg) on days 1 and 8 Irinotecan: 50 mg/m2 as an i.v. infusion over 1 hour on days 1-5 Regorafenib: Children between 6 and 24 months = 65 mg/m2, children less than 12 and/or less than 40kg dose = 82 mg/m2 Maximum 120 mg, Fixed dose of 120 mg for patients over 12 years of age AND ≥ 40 kg, as an oral tablets on days 8 to 21.
CT3: Relapsed Chemotherapy - VIRRVincristineVincristine: 1.5 mg/m2 As per local practice: recommended as a short infusion (maximum dose 2mg) on days 1 and 8 Irinotecan: 50 mg/m2 as an i.v. infusion over 1 hour on days 1-5 Regorafenib: Children between 6 and 24 months = 65 mg/m2, children less than 12 and/or less than 40kg dose = 82 mg/m2 Maximum 120 mg, Fixed dose of 120 mg for patients over 12 years of age AND ≥ 40 kg, as an oral tablets on days 8 to 21.
CT3: Relapsed Chemotherapy - VIRRRegorafenibVincristine: 1.5 mg/m2 As per local practice: recommended as a short infusion (maximum dose 2mg) on days 1 and 8 Irinotecan: 50 mg/m2 as an i.v. infusion over 1 hour on days 1-5 Regorafenib: Children between 6 and 24 months = 65 mg/m2, children less than 12 and/or less than 40kg dose = 82 mg/m2 Maximum 120 mg, Fixed dose of 120 mg for patients over 12 years of age AND ≥ 40 kg, as an oral tablets on days 8 to 21.
Primary Outcome Measures
NameTimeMethod
Event Free Survival (RT2)From randomisation to first failure event, timeframe 36 months

Failure events are:

* Relapse or progression of existing disease, or occurrence of disease at new sites,

* Death from any cause without disease progression,

* Second malignant neoplasm

Event Free Survival (CT2A)From randomisation to first failure event, timeframe 36 months

Failure events are:

* Relapse or progression of existing disease, or occurrence of disease at new sites,

* Death from any cause without disease progression,

* Second malignant neoplasm

Event Free Survival (CT1B)From randomisation to first failure event, timeframe 36 months

Failure events are:

* Relapse or progression of existing disease, or occurrence of disease at new sites,

* Death from any cause without disease progression,

* Second malignant neoplasm

Event Free Survival (CT2B)Time from randomisation to first failure event, timeframe 36 months

Failure events are:

* Relapse or progression of existing disease, or occurrence of disease at new sites,

* Death from any cause without disease progression,

* Second malignant neoplasm

Event Free Survival (CT3)Patients will be followed up for a minimum of 6 years from trial entry (or 5 years from end of relapsed trial treatment, whichever comes later). Patients will be followed up for progression and death until the end of trial definition has been met.

To determine whether new systemic therapy regimens improve event free survival in relapsed RMS compared to standard therapy (VIRT) (CT3):

Initial new systemic therapy combination to be tested:

o Regorafenib (R) added to vincristine and irinotecan (VIR) (VIRR)

Event Free Survival (CT1A)From randomisation to first failure event, timeframe 36 months

Failure events are:

* Relapse or progression of existing disease, or occurrence of disease at new sites,

* Death from any cause without disease progression,

* Second malignant neoplasm

Local Failure Free Survival (RT1C)Time from randomisation to first local failure event, timeframe 36 months

A local failure event is relapse or progression of tumour at the primary site at any time even if there has been a prior /concurrent, regional or distant failure

Local Failure Free Survival (RT1A and RT1B)Time from randomisation to first local failure event, timeframe 36 months

A local failure event is relapse or progression of tumour at the primary site at any time even if there has been a prior /concurrent, regional or distant failure

Secondary Outcome Measures
NameTimeMethod
Toxicity (All chemotherapy randomisations)From date of protocol defined treatment until 30 days after the administration of the last treatment

Categorised and graded using Common Terminology Criteria for Adverse Events

Overall Survival (CT1A)From randomisation to death from any cause, assessed for 36 months

Death from any cause

Overall Survival (CT2B)From randomisation to death from any cause, assessed for 36 months

Death from any cause

Acute wound complications and post-operative complications (RT1A and RT1B)Within 120 days from surgery

specific grade 3 and above complications according to CTCAE v 4 and Clavien Dindo scale. Specific wound complications within the same time frame will also be collected

Acute post-radiotherapy complications (All radiotherapy randomisations)Within 120 days from start of radiotherapy

any grade 3 and above event according to CTCAE v 4

Local Failure Free Survival (if participating in PET Sub-study)From date of randomisation/registration to first local failure event, assessed for 36 months

A local failure event is relapse or progression of tumour at the primary site at any time even if there has been a prior /concurrent, regional or distant failure

Overall Survival (CT1B)From randomisation to death from any cause, assessed for 36 months

Death from any cause

Overall Survival (RT2)From RT2 randomisation to death from any cause, as assessed for 36 months

Death from any cause

Overall Survival (CT3)Patients will be followed up for a minimum of 6 years from trial entry (or 5 years from end of relapsed trial treatment, whichever comes later). Patients will be followed up for progression and death until the end of trial definition has been met.

To evaluate the anti-tumour activity and effect on overall survival of VIRR when compared to standard therapy

Late complications (RT1A, RT1B. RT1C)After 120 days from last local therapy

specific grade 3 and above events according to CTCAE and Clavien-Dindo scale

Maximum Tolerated Dose (Phase 1b)From first patient first visit in dose finding study until appropriate dose level

Dose level at which no or one participant experiences a DLT when at least two of three to six participants experience a DLT at the next highest dose.

Dose Limiting Toxicity (Phase 1b)From commencement of treatment until 21 days after the start of cycle 2 (each cycle is 21 days)

Diarrhoea: Grade 3 for \>3 days despite loperamide therapy Diarrhoea: Grade 4 despite loperamide therapy. Enterocolitis: Grade 3 or above Ileus: Grade 3 or above for more than 3 days Oral mucositis: Grade 3 above for \>3 days despite optimal supportive care Persistent neutropenia or thrombocytopenia leading to delay of start of next course by \>7 days; i.e. starting \> day 28 Any grade 3 or 4 toxicity resulting in discontinuation of the new combination Any grade 5 toxicity

Response (Phase 1b, CT1A, CT1B)Response assessed after course 3 (63 days) and 6 (126 days)

defined as complete (CR) or partial response (PR) and is clinically defined. Patients who are not assessable for response - e.g. because of early stopping of treatment or death - will be assumed to be non-responders.

Tolerability (CT3)From registration/randomisation until death/study endpoint

To determine the tolerability of the regimens.

Overall Survival (RT1A and RT1B)From randomisation to death from any cause, assessed for 36 months

Death from any cause

Loco-regional failure-free survival (All radiotherapy randomisations)From randomisation to first local and/or regional failure event, assessed for 36 months

A local failure event is relapse or progression of tumour at the primary site at any time even if there has been a prior concurrent local, regional or distant failure. A regional event is relapse or progression of tumour at regional lymph nodes at any time even if there has been a prior distant failure.

Health related quality of life (RT1A and RT2) self-reported questionnaire completed by the patient4 timepoints: 1) 1 day of start of radiotherapy, 2) at completion of radiotherapy, average 5 weeks after start of radiotherapy, 3) 3 months and 4) 24 months following radiotherapy

will be assessed using European Organisation for Research and Treatment of Cancer, Quality of Life Questionnaire 30 (EORTC QLQ-C30) for patients 18 years of age and over. The minimum score is 0 where quality of life is completely unaffected by the intervention to 4 where quality of life is severely affected.

Health related quality of life (CT3) self-reported questionnaire completed by the patient3 timepoints: Each Cycle is 28 days. Timepoint 1: Day 0 of cycle 1 (prior to starting treatment), Timepoint 2 day 0 cycle 3, Timepoint 3) day 0 cycle 5

will be assessed using European Organisation for Research and Treatment of Cancer, Quality of Life Questionnaire 30 (EORTC QLQ-C30) for patients 18 years of age and over. The minimum score is 0 where quality of life is completely unaffected by the intervention to 4 where quality of life is severely affected.

Recommended Phase II Dose (Phase 1b)From first patient first visit in dose finding study until appropriate dose level found, estimated 9 months

Based on tolerability, where tolerability is evaluated through the occurrence of dose limiting toxicity (DLT).

Overall Survival (CT2A)From randomisation to death from any cause, assessed for 36 months

Death from any cause

Overall Survival (all patients)From randomisation/registration to death from any cause, assessed for 36 months

Death from any cause

Health related quality of life (RT1A and RT2) self-reported questionnaire completed by patient4 timepoints: 1) 1 day of start of radiotherapy, 2) at completion of radiotherapy, average 5 weeks after start of radiotherapy, 3) 3 months and 4) 24 months following radiotherapy

will be assessed using Pediatric quality of life questionnaire (PedsQL) for the paediatric population (under 18 years). The minimum score is 0 where quality of life is completely unaffected by the intervention to 4 where quality of life is severely affected.

Event Free Survival (all patients)From date of randomisation/registration to death from any cause, assessed for 36 months

Failure events are:

* Relapse or progression of existing disease, or occurrence of disease at new sites,

* Death from any cause without disease progression,

* Second malignant neoplasm

Overall Survival (RT1C)From RT1C randomisation to death from any cause, assessed for 36 months

Death from any cause

Acceptability and Palatability of Regorafenib (CT3)1 timepoint: Day 8 of cycle 1 (Each Cycle is 28 days)

"Acceptability and Palatability Questionnaire" To evaluate the acceptability and palatability of regorafenib formulations

PET Response (if participating in PET Sub-study)After three cycles of chemotherapy (each cycle is 21 days)

assessed by PERCIST criteria and visual 'Deauville like' criteria

Event Free Survival (if participating in PET Sub-study)From date of randomisation/registration to death from any cause, assessed for 36 months

Failure events are:

* Relapse or progression of existing disease, or occurrence of disease at new sites,

* Death from any cause without disease progression,

* Second malignant neoplasm

Trial Locations

Locations (128)

University Unit of Pediatric Oncology-hematology - Children's Hospital Agia Sophia

🇬🇷

Athens, Greece

Rambam Health Care Campus

🇮🇱

Haifa, Israel

Hadassah University Medical Centre

🇮🇱

Jerusalem, Israel

Schneider Medical Centre

🇮🇱

Petah Tikva, Israel

Department of Pediatric Hematology-oncology - Aghia Sophia Children's Hospital

🇬🇷

Athens, Greece

Hospital Virgen Del Rocio

🇪🇸

Seville, Spain

Uppsala University Childrens Hospital

🇸🇪

Uppsala, Sweden

The Queen Elizabeth Hospital

🇬🇧

Birmingham, United Kingdom

Hospital Regional Universitario De Malaga

🇪🇸

Malaga, Spain

Hospital De Cruces

🇪🇸

Bilbao, Spain

Hospital Politecnico U La Fe

🇪🇸

Valencia, Spain

Royal Aberdeen Children's Hospital

🇬🇧

Aberdeen, United Kingdom

Royal Belfast Hospital for Sick Children

🇬🇧

Belfast, United Kingdom

Hospital Universitario Gregorio Maranon

🇪🇸

Madrid, Spain

Starship Children's Health

🇳🇿

Auckland, New Zealand

Hospital Sant Joan De Deu

🇪🇸

Barcelona, Spain

Inselspital Bern

🇨🇭

Bern, Switzerland

Belfast City Hospital

🇬🇧

Belfast, United Kingdom

Addenbrooke's Hospital

🇬🇧

Cambridge, United Kingdom

Hospital Universitari Vall D'hebron

🇪🇸

Barcelona, Spain

Birmingham Children's Hospital

🇬🇧

Birmingham, United Kingdom

Noah's Ark Children's Hospital for Wales

🇬🇧

Cardiff, United Kingdom

Hospital Universitario Miguel Servet Materno - infantil

🇪🇸

Zaragoza, Spain

Christchurch Hospital

🇳🇿

Christchurch, New Zealand

Universitaetsspital Zurich

🇨🇭

Zurich, Switzerland

Bristol Haematology And Oncology Centre

🇬🇧

Bristol, United Kingdom

Beatson West of Scotland Cancer Centre

🇬🇧

Glasgow, United Kingdom

St James's University Hospital

🇬🇧

Leeds, United Kingdom

Leicester Royal Infirmary

🇬🇧

Leicester, United Kingdom

Royal Hospital for Children and Young People

🇬🇧

Edinburgh, United Kingdom

Royal Marsden Hospital London

🇬🇧

London, United Kingdom

University College London Hospital

🇬🇧

London, United Kingdom

Nottingham City Hospital

🇬🇧

Nottingham, United Kingdom

John Radcliffe Hospital

🇬🇧

Oxford, United Kingdom

Queensland Children's Hospital

🇦🇺

Brisbane, Australia

Peter Maccallum Cancer Centre

🇦🇺

Melbourne, Australia

Royal Childrens Hospital Melbourne

🇦🇺

Melbourne, Australia

John Hunter Children's Hospital

🇦🇺

New Lambton Heights, Australia

Perth Children's Hospital

🇦🇺

Perth, Australia

Sydney Children's Hospital

🇦🇺

Sydney, Australia

The Childrens Hospital At Westmead

🇦🇺

Sydney, Australia

Westmead Hospital

🇦🇺

Westmead, Australia

Princess Alexandra Hospital

🇦🇺

Woolloongabba, Australia

Kepler University Clinic Linz

🇦🇹

Linz, Austria

St Anna Childrens Hospital

🇦🇹

Vienna, Austria

Hopital Universitaire Des Enfants Reine Fabiola

🇧🇪

Brussels, Belgium

Cliniques Universitaires Saint Luc

🇧🇪

Bruxelles, Belgium

Uz Leuven Campus Gasthuisberg

🇧🇪

Leuven, Belgium

Clinique Chc Montlegia

🇧🇪

Liège, Belgium

Masaryk University Hospital Brno

🇨🇿

Brno, Czechia

Aarhus University Hospital

🇩🇰

Aarhus, Denmark

Centre Hospitalier Universitaire D'angers

🇫🇷

Angers, France

Centre Hospitalier Regional Universitaire Besancon - Hopital Jean Minjoz

🇫🇷

Besançon, France

Centre Hospitalier Universitaire De Bordeaux - Hopital Pellegrin

🇫🇷

Bordeaux, France

Centre Francois Baclesse

🇫🇷

Caen, France

Centre Hospitalier Regional Universitaire Brest - Hopital Morvan

🇫🇷

Brest, France

Centre Hospitalier Universitaire De Caen

🇫🇷

Caen, France

Centre Hospitalier Universitaire Dijon Bourgogne - Hopital D'enfants

🇫🇷

Dijon, France

Centre Hospitalier Universitaire De Grenoble

🇫🇷

Grenoble, France

Centre Hospitalier Universitaire La Reunion

🇫🇷

La Réunion, France

Centre Oscar Lambret

🇫🇷

Lille, France

Centre Leon Berard

🇫🇷

Lyon, France

Hopital De La Timone (ap-hm)

🇫🇷

Marseille, France

Centre Hospitalier Universitaire De Nancy

🇫🇷

Nancy, France

Institut Curie

🇫🇷

Paris, France

Centre Hospitalier Universitaire De Nantes

🇫🇷

Nantes, France

Hopital Armand Trousseau

🇫🇷

Paris, France

Centre Hospitalier Universitaire Haut Levque

🇫🇷

Pessac, France

Chu De Reims

🇫🇷

Reims, France

Centre Eugne Marquis De Rennes

🇫🇷

Rennes, France

Centre Hospitalier Universitaire De Rennes - Hopital Pontchaillou

🇫🇷

Rennes, France

Centre Hospitalier Universitaire De Rouen

🇫🇷

Rouen, France

Centre Hospitalier Universitaire Saint-etienne

🇫🇷

Saint-Étienne, France

Centre Hospitalier Universitaire De Toulouse - Hopital Des Enfants

🇫🇷

Toulouse, France

Gustave Roussy

🇫🇷

Villejuif, France

Children's General Hospital P and A Kyriakou

🇬🇷

Athens, Greece

Clatterbridge Cancer Centre

🇬🇧

Wirral, United Kingdom

University Hospital Rigshospitalet

🇩🇰

Copenhagen, Denmark

University Hospital of Padova (azienda Ospedaliera of Padua)

🇮🇹

Padova, Italy

Bristol Royal Hospital for Children

🇬🇧

Bristol, United Kingdom

Velindre Hospital

🇬🇧

Cardiff, United Kingdom

Royal Hospital for Children Glasgow

🇬🇧

Glasgow, United Kingdom

Leeds General Infirmary

🇬🇧

Leeds, United Kingdom

Alder Hey Children's Hospital

🇬🇧

Liverpool, United Kingdom

Great Ormond Street Hospital for Children

🇬🇧

London, United Kingdom

Royal Manchester Children's Hospital

🇬🇧

Manchester, United Kingdom

Christie Hospital

🇬🇧

Manchester, United Kingdom

Royal Victoria Infirmary

🇬🇧

Newcastle Upon Tyne, United Kingdom

Queen's Medical Centre, Nottingham

🇬🇧

Nottingham, United Kingdom

Sheffield Children's Hospital

🇬🇧

Sheffield, United Kingdom

Weston Park Hospital

🇬🇧

Sheffield, United Kingdom

Southampton General Hospital

🇬🇧

Southampton, United Kingdom

Centre Hospitalier Universitaire De Poitiers

🇫🇷

Poitiers, France

Strasbourg Hautepierre

🇫🇷

Strasbourg, France

Centre Hospitalier Regional Universitaire De Tours - Hopital Clocheville

🇫🇷

Tours, France

Chris O'brien Lifehouse

🇦🇺

Camperdown, Australia

Monash Children's Hospital

🇦🇺

Clayton, Australia

Hospital Universitario La Paz

🇪🇸

Madrid, Spain

Hospital Del Nino Jesus

🇪🇸

Madrid, Spain

Children's and Adolescent's Oncology Clinic, "MITERA" Children's Hospital

🇬🇷

Attikí, Greece

Ippokratio General Hospital of Thessaloniki

🇬🇷

Thessaloniki, Greece

Ahepa University General Hospital of Thessaloniki

🇬🇷

Thessaloníki, Greece

Prinses Maxima Centrum Voor Kinderoncologie

🇳🇱

Utrecht, Netherlands

Haukeland University Hospital - Paediatric

🇳🇴

Bergen, Norway

Oslo University Hospital - Radiumhospitalet

🇳🇴

Oslo, Norway

Hematology-oncology Children's Clinic, University General Hospital of Heraklion

🇬🇷

Iraklio, Greece

Dana Children's Hospital, Tel Aviv Sourasky Medical Center

🇮🇱

Tel Aviv, Israel

Instituto Portugues De Oncologia De Losbona Francisco Gentil, Epe

🇵🇹

Lisbon, Portugal

Universitair Ziekenhuis Gent

🇧🇪

Gent, Belgium

Hellenic Society of Pediatric Hematology- Oncology

🇬🇷

Athens, Greece

Oslo University Hospital - Paediatrics

🇳🇴

Oslo, Norway

University Hospital of North Norway - Paediatric

🇳🇴

Tromso, Norway

Chaim Sheba Medical Centre

🇮🇱

Tel HaShomer, Israel

Kantonsspital Aarau

🇨🇭

Aarau, Switzerland

Ospedale San Giovanni

🇨🇭

Bellinzona, Switzerland

Luzerner Kantonspital - Kinderspital Luzern

🇨🇭

Luzern, Switzerland

Royal Marsden Hospital

🇬🇧

Sutton, Surrey, United Kingdom

Centre Hospitalier Regional De La Citadelle

🇧🇪

Liège, Belgium

Our Lady's Children's Hospital

🇮🇪

Crumlin, Ireland

University Medical Centre Groningen

🇳🇱

Groningen, Netherlands

St Olavs Hospital - Paediatric

🇳🇴

Trondheim, Norway

Universitats-kinderspital Bieder Basel (UKBB)

🇨🇭

Basel, Switzerland

Bratislava, National Institute for Children's Diseases

🇸🇰

Bratislava, Slovakia

University Medical Centre Ljubjlana

🇸🇮

Ljubljana, Slovenia

University Childrens Hospital Ljubljana

🇸🇮

Ljubljana, Slovenia

Hug Hopitaux Universitaires De Geneve

🇨🇭

Geneva, Switzerland

Centre Hospitalier Universitaire Vaudois (CHUV), Lausanne

🇨🇭

Lausanne, Switzerland

Ostschweizer Kinderspital

🇨🇭

St Gallen, Switzerland

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