MRI Screening of Second Primary Cancer Occurring Within Radiation Fields After Treatment by External Beam Radiation Therapy for Hereditary Retinoblastoma (DepiSCARRH)

Study Overview
=================
Brief Summary
-----------------
The purpose of this study is to assess the benefit of MR screening for asymptomatic head & neck (or CNS) second primary cancers occurring in hereditary retinoblastoma patients previously treated by external beam radiation therapy (EBRT).

Detailed Description
-----------------
Patients with hereditary retinoblastoma treated during infancy by external beam radiation therapy (EBRT) the risk of second primary cancer occurring within radiation fields is high. The aim of this study is to depict by MRI secondary tumors as early as possible, before the occurrence of clinical symptoms. Affected patients will be further managed in a national expert center with dedicated diagnostic and treatment procedures. However, the benefit of such management has to be assessed in terms of prognosis.

Official Title
-----------------
MRI Screening of Second Primary Cancer Occurring Within Radiation Fields After Treatment by External Beam Radiation Therapy for Hereditary Retinoblastoma

Conditions
-----------------
Hereditary Retinoblastoma

Intervention / Treatment
-----------------
* Procedure: MRI


Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: Personal history of hereditary retinoblastoma (i.e., familial history of retinoblastoma, or bilateral retinoblastoma, or unilateral multifocal retinoblastoma, or identified germline RB1 mutation or 13q deletion) External beam radiation therapy (EBRT) used for retinoblastoma treatment Age at inclusion greater or equal to 7 years old. Time period between the end of EBRT and inclusion date of 5 years or more Written informed consent signed by patient (or legal representative) Exclusion Criteria: Personal history of non-familial unilateral unifocal retinoblastoma without RB1 germline mutation. Personal history of second primary neoplasm occurring within radiation fields Contraindication for MRI (pacemaker, intraocular metallic foreign body, defibrillators or other implanted electronic devices, intracranial ferro-magnetic clips) or associated conditions preventing from MR examination (intraocular prostheses and implants are not a contraindication for MRI; orthodontic metallic devices are not a contraindication but might decrease the image quality and should be removed, if possible) Patients unable to comply with follow-up study requirements, for any geographical, social or psychological reason

Ages Eligible for Study
-----------------
Minimum Age: 7 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Design Details

Primary Purpose: Diagnostic
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Other: MRI<br> | Procedure: MRI<br>* MRI will be performed each year during 10 years.<br>|

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Assess the benefit of MR screening for asymptomatic head & neck (or CNS) second primary cancers occurring in hereditary retinoblastoma patients previously treated by external beam radiation therapy (EBRT). | Rate of patients with R0-resection quality (versus R1 or R2) among patients with second primary cancer depicted by MRI, with comparison to historical series | Up to 10 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Assess the visual prognosis of patients with second primary cancer depicted by MRI | Measure of residual visual functions before and after local treatment of the second primary cancer in patients depicted by MRI compared to that patients who the second primary cancer was depicted by clinical symptoms. | Up to 10 year | 
| Assess the feasibility of the MR screening program. | Number of MRI performed for the radiological follow-up for patients agreeing to participate to the study. | Up to 10 years | 
| Number of non tumor detected anomalies with invasive procedures to assess the psychological consequences of the MR screening program |  | Up to 10 years | 
| Assess the psychological consequences of the MR screening program by the completion of quality of life questionnaires. | - Quality of life questionnaires completed by the patient every year (before MRI) during 10 years. | Up to 10 years | 
| Number of depicted second primary cancer.to assess the diagnostic accuracy of MRI. |  | Up to 10 years | 
| Number of asymptomatic depicted second primary cancer by MR to assess the diagnostic accuracy of MRI |  | Up to 10 years | 
| Number of symptomatic second primary cancer between 2 MRI.to assess the diagnostic accuracy of MRI |  | Up to 10 years | 
| Measure of sensibility of MRI to detect second primary cancer |  | Up to 10 years | 
| Measure of specificity of MRI to detect second primary cancer |  | Up to 10 years | 
| Collection of secondary tumors for future research purpose. |  | up to 10 years | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
hereditary retinoblastoma,, External beam radiation therapy (EBRT)