Table 4:

Top 30 retinoblastoma research priorities

Final rankCategoryQuestionAggregate ranking
1DiagnosisHow to increase early diagnosis of retinoblastoma (i.e., decrease age or stage at diagnosis)?71
2Second cancerWhat second cancer screening is optimal for heritable retinoblastoma survivors (including whole body magnetic resonance imaging)?11
3PsychosocialHow to provide culturally competent social, emotional and psychological support to patients with retinoblastoma, survivors, parents and families (at diagnosis and beyond)?22
4Follow-upWhat is the optimal follow-up (including ophthalmologic and oncologic) for patients with heritable retinoblastoma and survivors (by diagnosis and treatment), and how can we ensure this is provided to all?93
5TreatmentProspective retinoblastoma treatment studies with long-term follow-up54
6PsychosocialWhat is the effect of enucleation and vision loss on retinoblastoma survivors?115
7Second cancerWhat are the risk factors for second cancers in heritable retinoblastoma survivors, and, in turn, what do heritable retinoblastoma survivors need to know about living well and minimizing risk of second cancers?76
8MiscellaneousHow to improve collaboration across the different top centres caring for retinoblastoma: forming an international consortium, a unified registry and combined trials, instead of the current air of competition?1710
9Follow-upHow to provide a detailed pathway of care or plan, outlining treatment and follow-up, to patients with retinoblastoma and families?138
10Global healthHow can optimal retinoblastoma care be delivered in low-resource settings (including rural and remote communities)?49
11TreatmentClinical trials of novel agents, targeted agents added to “backbone” chemotherapy or intra-arterial chemotherapy to improve eye-salvage rates39
12Genetics and molecularWhat genetic mechanism results in second cancers in heritable retinoblastoma survivors?107
13TreatmentBetter identification of who needs chemotherapy after high-risk pathologic findings1210
14DiagnosisWhat new technology could be used to diagnose retinoblastoma earlier, including noninvasive in utero testing?611
15AwarenessHow to increase family doctor/pediatrician awareness of retinoblastoma (i.e., signs and symptoms, and the importance of early diagnosis), and screening and diagnosis of retinoblastoma?612
16PsychosocialHow can we help families cope better during diagnosis and critical stages (including enucleation)?813
17Second cancerWhat are the risks of second cancers for mosaic RB1 mutation carriers (i.e., those in whom RB1 mutation is present in some but not all cells in their body)?1814
18TreatmentHow to reduce side [adverse] effects from retinoblastoma treatments?1215
19Genetics and molecularCan we identify the key molecular event that distinguishes retinoma (benign retinoblastoma precursor) from retinoblastoma?1616
20Genetics and molecularCan a known RB1 gene mutation be corrected?1518
21Follow-upHow to improve the sensitivity of minimal residual disease (i.e., metastasized cancer cells that cannot be detected by routine tests) diagnostics in retinoblastoma?2017
22Genetics and molecularHow to communicate with and educate patients, survivors and parents about retinoblastoma genetics and their specific retinoblastoma genetic testing results (including new tools, techniques and innovations)?1619
23Family planningWhat is the best way to support and educate heritable retinoblastoma survivors before they have their own children and to ensure their children have optimal perinatal care?1420
24Genetics and molecularHow can second cancers be prevented in heritable retinoblastoma survivors?1321
25Second cancerWhat is the second cancer incidence among heritable retinoblastoma survivors?1822
26Genetics and molecularHow can we reduce the risk of second cancers in heritable retinoblastoma survivors?1922
27PsychosocialWhat social, emotional and psychologic support services are available across Canada for patients with retinoblastoma, survivors and parents (i.e., comparisons nationally)?2123
28PsychosocialWhat is the impact — on mental health, finances, employment, siblings and family life — when one must travel long distances for retinoblastoma care?2224
29VisionHow can scar tissue/calcium in the eye from retinoblastoma treatment be removed to give better vision?2325
30Genetics and molecularWhat causes heritable (germline) and nonheritable (somatic) retinoblastoma mutations?2426