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

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

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).

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.

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