Recruiting
Study Start: September, 2020
Study Completion: June, 2030
NCT04625907

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

Keywords:
Sarcoma
Cancer

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.

Intervention / Treatment

01
Irinotecan
Drug
02
Actinomycin D
Drug
03
Doxorubicin
Drug
04
Ifosfamide
Drug
05
Vincristine
Drug
06
Vinorelbine
Drug
07
Cyclophosphamide
Drug
08
Temozolomide
Drug
09
Radiotherapy
Radiation
10
Regorafenib
Drug

Study Design

Primary Purpose

Treatment

Allocation

Randomized

Interventional Model

Parallel Assignment

Masking

None (Open Label)

Arm Groups

Experimental: Phase 1b Dose finding: VHR induction - IRIVA

Irinotecan: 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.

Active Comparator: CT1A: VHR induction - IVADO

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 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

Experimental: CT1A: VHR Induction IRIVA

Irinotecan: 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.

Active Comparator: CT1B: HR Induction IVA

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 day 1 on cycles 3-9.

Actinomycin: 1.5 mg/m2 as an i.v. bolus injection (maximum dose 2mg) on day 1.

Experimental: CT1B: HR Induction IRIVA

Irinotecan: 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.

Experimental: RT1A: Preoperative Radiotherapy

To be given either 41.4 Gy or 50.4 Gy prior to surgery

Active Comparator: RT1A: Post operative radiotherapy

To be given either 41.4 Gy or 50.4 Gy following surgery

Experimental: RT1B: Radiotherapy for resectable disease: dose escalated

To receive 50.4 Gy

Active Comparator: RT1B: Radiotherapy for resectable disease: standard dose

To receive 41.4 Gy

Experimental: RT1C: Radiotherapy for unresectable disease: dose escalated

To receive 59.4 Gy

Active Comparator: RT1C: Radiotherapy for unresectable disease: standard dose

To receive 50.4 Gy

Experimental: RT2: Radiotherapy to primary tumour and involved lymph nodes

Radiotherapy to the primary tumour and involved regional lymph nodes only

Experimental: RT2: Radiotherapy to all metastatic sites

Radiotherapy given to all metastatic sites

Experimental: CT2A: VHR Maintenance - VC

Vinorelbine: 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

No Intervention: CT2A: Maintenance -Stop treatment

To stop treatment at the point of randomisation

Experimental: CT2B: HR Maintenance - VC

Vinorelbine: 25 mg/m2 i.v. on days 1,8 and 15 Cyclophosphamide 25 mg/m2 orally daily for 28 days

No Intervention: CT2B: HR Maintenance - Stop Treatment

To stop treatment at the point of randomisation

Active Comparator: CT3: Relpased Chemotherapy - VIRT

Vincristine: 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

Experimental: CT3: Relapsed Chemotherapy - VIRR

Vincristine: 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.

Eligibility Criteria

  • Histologically confirmed diagnosis of RMS (except pleomorphic RMS)
  • Written informed consent from the patient and/or the parent/legal guardian

  • Entered into the FaR-RMS study at diagnosis
  • Very High Risk disease
  • Age >12 months and ≤25 years
  • No prior treatment for RMS other than surgery
  • Medically fit to receive treatment
  • Adequate hepatic function:

    • Total bilirubin ≤ 1.5 times upper limit of normal (ULN) for age, unless the patient is known to have Gilbert's syndrome
    • ALT or AST < 2.5 x ULN for age

  • Absolute neutrophil count ≥1.0 x 10^9/L
  • Platelets ≥ 80 x 10^9/L
  • Adequate renal function: estimated or measured creatinine clearance ≥60 ml/min/1.73 m2
  • Documented negative pregnancy test for female patients of childbearing potential
  • Patient agrees to use contraception during therapy and for 12 months after last trial treatment (females) or 6 months after last trial treatment (males), where patient is sexually active
  • Written informed consent from the patient and/or the parent/legal guardian

  • Weight <10kg
  • Active > grade 2 diarrhoea
  • Prior allo- or autologous Stem Cell Transplant
  • Uncontrolled inter-current illness or active infection
  • Pre-existing medical condition precluding treatment
  • Urinary outflow obstruction that cannot be relieved prior to starting treatment
  • Active inflammation of the urinary bladder (cystitis)
  • Known hypersensitivity to any of the treatments or excipients
  • Second malignancy
  • Pregnant or breastfeeding women

  • Entered into the FaR-RMS study at diagnosis
  • Very High Risk disease
  • Age ≥ 6 months
  • Available for randomisation ≤60 days after diagnostic biopsy/surgery
  • No prior treatment for RMS other than surgery
  • Medically fit to receive treatment
  • Adequate hepatic function:

    • Total bilirubin ≤ 1.5 times upper limit of normal (ULN) for age, unless the patient is known to have Gilbert's syndrome

  • Absolute neutrophil count ≥1.0 x 10^9/L (except in patients with documented bone marrow disease)
  • Platelets ≥ 80 x 10^9/L (except in patients with documented bone marrow disease)
  • Fractional Shortening ≥ 28%
  • Documented negative pregnancy test for female patients of childbearing potential
  • Patient agrees to use contraception during therapy and for 12 months after last trial treatment (females) or 6 months after last trial treatment (males), where patient is sexually active
  • Written informed consent from the patient and/or the parent/legal guardian

  • Active > grade 2 diarrhoea
  • Prior allo- or autologous Stem Cell Transplant
  • Uncontrolled inter-current illness or active infection
  • Pre-existing medical condition precluding treatment
  • Urinary outflow obstruction that cannot be relieved prior to starting treatment
  • Active inflammation of the urinary bladder (cystitis)
  • Known hypersensitivity to any of the treatments or excipients
  • Second malignancy
  • Pregnant or breastfeeding women

  • Entered into the FaR-RMS study at diagnosis
  • High Risk disease
  • Age ≥ 6 months
  • Available for randomisation ≤60 days after diagnostic biopsy/surgery
  • No prior treatment for RMS other than surgery
  • Medically fit to receive treatment
  • Adequate hepatic function:

    • Total bilirubin ≤ 1.5 times upper limit of normal (ULN) for age, except if the patient is known to have Gilbert's syndrome

  • Absolute neutrophil count ≥1.0 x 10^9/L
  • Platelets ≥ 80 x 10^9/L
  • Documented negative pregnancy test for female patients of childbearing potential
  • Patient agrees to use contraception during therapy and for 12 months after last trial treatment (females) or 6 months after last trial treatment (males), where patient is sexually active
  • Written informed consent from the patient and/or the parent/legal guardian

  • Active > grade 2 diarrhoea
  • Prior allo- or autologous Stem Cell Transplant
  • Uncontrolled inter-current illness or active infection
  • Pre-existing medical condition precluding treatment
  • Urinary outflow obstruction that cannot be relieved prior to starting treatment
  • Active inflammation of the urinary bladder (cystitis)
  • Known hypersensitivity to any of the treatments or excipients
  • Second malignancy
  • Pregnant or breastfeeding women

Frontline Radiotherapy Note: eligible patients may enter multiple radiotherapy randomisations.

  • Entered in to the FaR-RMS study (at diagnosis or prior to radiotherapy randomisation)
  • Very High Risk, High Risk and Standard Risk disease
  • ≥ 2 years of age
  • Receiving frontline induction treatment as part of the FaR-RMS trial or with a IVA/IVADo based chemotherapy regimen patients for whom. Note that patients for whom ifosfamide has been replaced with cyclophosphamide will be eligible
  • Patient assessed as medically fit to receive the radiotherapy
  • Documented negative pregnancy test for female patients of childbearing potential
  • Patient agrees to use contraception during therapy and for 12 months after last trial treatment (females) or 6 months after last trial treatment (males), where patient is sexually active
  • Written informed consent from the patient and/or the parent/legal guardian

  • Prior allo- or autologous Stem Cell Transplant
  • Second malignancy
  • Pregnant or breastfeeding women
  • Receiving radiotherapy as brachytherapy

  • Primary tumour deemed resectable (predicted R0/ R1 resection feasible) after 3 cycles of induction chemotherapy (6 cycles for metastatic disease)
  • Adjuvant radiotherapy required in addition to surgical resection (local decision)
  • Available for randomisation after cycle 3 and prior to the start of cycle 6 of induction chemotherapy for localised disease, or after cycle 6 and prior to the start of cycle 9 for metastatic disease

  • Primary tumour deemed resectable (predicted R0/R1 resection) after 3 cycles of induction chemotherapy (6 cycles for metastatic disease).
  • Adjuvant radiotherapy required in addition to surgical resection (local decision)
  • Higher Local Failure Risk (HLFR) based on presence of either of the following criteria:

    • Unfavourable site
    • Age ≥ 18yrs

  • Available for randomisation after cycle 3 and prior to the start of cycle 6 of induction chemotherapy for localised disease, or after cycle 6 and prior to the start of cycle 9 for metastatic disease

  • Primary radiotherapy indicated (local decision)
  • Higher Local Failure Risk (HLFR) based on either of the following criteria:

    • Unfavourable site
    • Age ≥ 18yrs

  • Available for randomisation after cycle 3 and prior to the start of cycle 6 of induction chemotherapy for localised disease, or after cycle 6 and prior to the start of cycle 9 for metastatic disease

  • Available for randomisation after cycle 6 and before the start of cycle 9 of induction chemotherapy.
  • Unfavourable metastatic disease, defined as Modified Oberlin Prognostic Score 2-4

    • Note: Definition of metastatic lesions for RT2 eligibility

Modified Oberlin Prognostic Score (1 point for each adverse factor):

  • Age ≥10y
  • Extremity, Other, Unidentified Primary Site
  • Bone and/or Bone Marrow involvement
  • ≥3 metastatic sites

Unfavourable metastatic disease: 2-4 adverse factors; Favourable metastatic disease: 0-1 adverse factors

Randomisation must take place during the 12th cycle of maintenance chemotherapy.

  • Entered in to the FaR-RMS study (at diagnosis or at any subsequent time point)
  • Very High Risk disease
  • Received frontline induction chemotherapy as part of the FaR-RMS trial or with a IVA/IVADo based chemotherapy regimen

    • Patients for whom ifosfamide has been replaced with cyclophosphamide will be eligible

  • Completed 11 cycles of VnC maintenance treatment (either oral or IV regimens)
  • No evidence of progressive disease
  • Absence of severe vincristine neuropathy - i.e requiring discontinuation of vincristine treatment)
  • Medically fit to continue to receive treatment
  • Patient agrees to use contraception during therapy and for 12 months after last trial treatment (females) or 6 months after last trial treatment (males), where patient is sexually active
  • Written informed consent from the patient and/or the parent/legal guardian

  • Prior allo- or autologous Stem Cell Transplant
  • Uncontrolled intercurrent illness or active infection
  • Urinary outflow obstruction that cannot be relieved prior to starting treatment
  • Active inflammation of the urinary bladder (cystitis)
  • Second malignancy
  • Pregnant or breastfeeding women

Randomisation must take place during the 6th cycle of maintenance chemotherapy.

  • Entered in to the FaR-RMS study (at diagnosis or at any subsequent time point)
  • High Risk disease
  • Received frontline induction chemotherapy as part of the FaR-RMS trial or with a IVA based chemotherapy regimen. Note that patients for whom ifosfamide has been replaced with cyclophosphamide will be eligible
  • Completed 5 cycles of VnC maintenance treatment
  • No evidence of progressive disease
  • Absence of severe vincristine neuropathy i.e. requiring discontinuation of vincristine treatment
  • Medically fit to continue to receive treatment
  • Patient agrees to use contraception during therapy and for 12 months after last trial treatment (females) or 6 months after last trial treatment (males), where patient is sexually active
  • Written informed consent from the patient and/or the parent/legal guardian

  • Prior allo- or autologous Stem Cell Transplant
  • Uncontrolled inter current illness or active infection
  • Urinary outflow obstruction that cannot be relieved prior to starting treatment
  • Active inflammation of the urinary bladder (cystitis)
  • Second malignancy
  • Pregnant or breastfeeding women

  • Entered in to the FaR-RMS study (at diagnosis or at any subsequent time point)
  • First or subsequent relapse of histologically verified RMS
  • Age ≥ 6 months
  • Measurable or evaluable disease
  • No cytotoxic chemotherapy or other investigational medicinal product (IMP) within previous three weeks: within two weeks for vinorelbine and cyclophosphamide maintenance chemotherapy
  • Medically fit to receive trial treatment
  • Documented negative pregnancy test for female patients of childbearing potential within 7 days of planned randomisation
  • Patient agrees to use contraception during therapy and for 12 months after last trial treatment (females) or 6 months after last trial treatment (males), where patient is sexually active
  • Written informed consent from the patient and/or the parent/legal guardian

  • Progression during frontline therapy without previous response (=Refractory to first line treatment)
  • Prior regorafenib or temozolomide
  • Active > grade 1 diarrhoea
  • ALT or AST >3.0 x upper limit normal (ULN)
  • Bilirubin, Total >1.5 x ULN; total bilirubin is allowed up to 3 x ULN if Gilbert's syndrome is documented
  • Patients with unstable angina or new onset angina (within 3 months of planned date of randomisation), recent myocardial infarction (within 6 months of randomisation) and those with cardiac failure New York Heart Association (NYHA) Classification 2 or higher Cardiac abnormalities such as congestive heart failure (Modified Ross Heart Failure Classification for Children = class 2) and cardiac arrhythmias requiring antiarrhythmic therapy (beta blockers or digoxin are permitted)
  • Uncontrolled hypertension > 95th centile for age and gender
  • Prior allo- or autologous Stem Cell Transplant
  • Uncontrolled inter-current illness or active infection
  • Pre-existing medical condition precluding treatment
  • Known hypersensitivity to any of the treatments or excipients
  • Second malignancy
  • Pregnant or breastfeeding women

Outcome Measures

Primary Outcome Measures

Event Free Survival (RT2)

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

From randomisation to first failure event, timeframe 36 months

Event Free Survival (CT1A)

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

From randomisation to first failure event, timeframe 36 months

Event Free Survival (CT1B)

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

From randomisation to first failure event, timeframe 36 months

Event Free Survival (CT2A)

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

From randomisation to first failure event, timeframe 36 months

Event Free Survival (CT2B)

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

Time from randomisation to first failure event, timeframe 36 months

Event Free Survival (CT3)

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)

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.

Local Failure Free Survival (RT1A and RT1B)

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

Time from randomisation to first local failure event, timeframe 36 months

Local Failure Free Survival (RT1C)

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

Time from randomisation to first local failure event, timeframe 36 months

Secondary Outcome Measures

Recommended Phase II Dose (Phase 1b)

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

From first patient first visit in dose finding study until appropriate dose level found, estimated 9 months

Maximum Tolerated Dose (Phase 1b)

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.

From first patient first visit in dose finding study until appropriate dose level

Toxicity (All chemotherapy randomisations)

Categorised and graded using Common Terminology Criteria for Adverse Events

From date of protocol defined treatment until 30 days after the administration of the last treatment

Dose Limiting Toxicity (Phase 1b)

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

From commencement of treatment until 21 days after the start of cycle 2 (each cycle is 21 days)

Response (Phase 1b, CT1A, CT1B)

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.

Response assessed after course 3 (63 days) and 6 (126 days)

Tolerability (CT3)

To determine the tolerability of the regimens.

From registration/randomisation until death/study endpoint

Overall Survival (CT1A)

Death from any cause

From randomisation to death from any cause, assessed for 36 months

Overall Survival (CT1B)

Death from any cause

From randomisation to death from any cause, assessed for 36 months

Overall Survival (CT2A)

Death from any cause

From randomisation to death from any cause, assessed for 36 months

Overall Survival (CT2B)

Death from any cause

From randomisation to death from any cause, assessed for 36 months

Overall Survival (RT1A and RT1B)

Death from any cause

From randomisation to death from any cause, assessed for 36 months

Overall Survival (RT1C)

Death from any cause

From RT1C randomisation to death from any cause, assessed for 36 months

Overall Survival (RT2)

Death from any cause

From RT2 randomisation to death from any cause, as assessed for 36 months

Overall Survival (CT3)

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

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.

Overall Survival (all patients)

Death from any cause

From randomisation/registration to death from any cause, assessed for 36 months

Acute wound complications and post-operative complications (RT1A and RT1B)

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

Within 120 days from surgery

Acute post-radiotherapy complications (All radiotherapy randomisations)

Any grade 3 and above event according to CTCAE v 4

Within 120 days from start of radiotherapy

Late complications (RT1A, RT1B. RT1C)

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

After 120 days from last local therapy

Loco-regional failure-free survival (All radiotherapy randomisations)

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.

From randomisation to first local and/or regional failure event, assessed for 36 months

Health related quality of life (RT1A and RT2) self-reported questionnaire completed by patient

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.

4 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

Health related quality of life (RT1A and RT2) self-reported questionnaire completed by the patient

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.

4 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

Health related quality of life (CT3) self-reported questionnaire completed by the patient

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.

3 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

Health related quality of life (CT3) self-reported questionnaire completed by the patient

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.

3 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

Acceptability and Palatability of Regorafenib (CT3)

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

1 timepoint: Day 8 of cycle 1 (Each Cycle is 28 days)

PET Response (if participating in PET Sub-study)

Assessed by PERCIST criteria and visual 'Deauville like' criteria

After three cycles of chemotherapy (each cycle is 21 days)

Event Free Survival (all patients)

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

From date of randomisation/registration to death from any cause, assessed for 36 months

Event Free Survival (if participating in PET Sub-study)

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

From date of randomisation/registration to death from any cause, assessed for 36 months

Local Failure Free Survival (if participating in PET Sub-study)

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

From date of randomisation/registration to first local failure event, assessed for 36 months

Where is this study conducted?

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