Table 4:
Top 30 retinoblastoma research priorities
| Final rank | Category | Question | Aggregate ranking | |
|---|---|---|---|---|
| First | Second | |||
| 1 | Diagnosis | How to increase early diagnosis of retinoblastoma (i.e., decrease age or stage at diagnosis)? | 7 | 1 |
| 2 | Second cancer | What second cancer screening is optimal for heritable retinoblastoma survivors (including whole body magnetic resonance imaging)? | 1 | 1 |
| 3 | Psychosocial | How to provide culturally competent social, emotional and psychological support to patients with retinoblastoma, survivors, parents and families (at diagnosis and beyond)? | 2 | 2 |
| 4 | Follow-up | What 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? | 9 | 3 |
| 5 | Treatment | Prospective retinoblastoma treatment studies with long-term follow-up | 5 | 4 |
| 6 | Psychosocial | What is the effect of enucleation and vision loss on retinoblastoma survivors? | 11 | 5 |
| 7 | Second cancer | What 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? | 7 | 6 |
| 8 | Miscellaneous | How 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? | 17 | 10 |
| 9 | Follow-up | How to provide a detailed pathway of care or plan, outlining treatment and follow-up, to patients with retinoblastoma and families? | 13 | 8 |
| 10 | Global health | How can optimal retinoblastoma care be delivered in low-resource settings (including rural and remote communities)? | 4 | 9 |
| 11 | Treatment | Clinical trials of novel agents, targeted agents added to “backbone” chemotherapy or intra-arterial chemotherapy to improve eye-salvage rates | 3 | 9 |
| 12 | Genetics and molecular | What genetic mechanism results in second cancers in heritable retinoblastoma survivors? | 10 | 7 |
| 13 | Treatment | Better identification of who needs chemotherapy after high-risk pathologic findings | 12 | 10 |
| 14 | Diagnosis | What new technology could be used to diagnose retinoblastoma earlier, including noninvasive in utero testing? | 6 | 11 |
| 15 | Awareness | How 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? | 6 | 12 |
| 16 | Psychosocial | How can we help families cope better during diagnosis and critical stages (including enucleation)? | 8 | 13 |
| 17 | Second cancer | What 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)? | 18 | 14 |
| 18 | Treatment | How to reduce side [adverse] effects from retinoblastoma treatments? | 12 | 15 |
| 19 | Genetics and molecular | Can we identify the key molecular event that distinguishes retinoma (benign retinoblastoma precursor) from retinoblastoma? | 16 | 16 |
| 20 | Genetics and molecular | Can a known RB1 gene mutation be corrected? | 15 | 18 |
| 21 | Follow-up | How to improve the sensitivity of minimal residual disease (i.e., metastasized cancer cells that cannot be detected by routine tests) diagnostics in retinoblastoma? | 20 | 17 |
| 22 | Genetics and molecular | How 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)? | 16 | 19 |
| 23 | Family planning | What 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? | 14 | 20 |
| 24 | Genetics and molecular | How can second cancers be prevented in heritable retinoblastoma survivors? | 13 | 21 |
| 25 | Second cancer | What is the second cancer incidence among heritable retinoblastoma survivors? | 18 | 22 |
| 26 | Genetics and molecular | How can we reduce the risk of second cancers in heritable retinoblastoma survivors? | 19 | 22 |
| 27 | Psychosocial | What social, emotional and psychologic support services are available across Canada for patients with retinoblastoma, survivors and parents (i.e., comparisons nationally)? | 21 | 23 |
| 28 | Psychosocial | What is the impact — on mental health, finances, employment, siblings and family life — when one must travel long distances for retinoblastoma care? | 22 | 24 |
| 29 | Vision | How can scar tissue/calcium in the eye from retinoblastoma treatment be removed to give better vision? | 23 | 25 |
| 30 | Genetics and molecular | What causes heritable (germline) and nonheritable (somatic) retinoblastoma mutations? | 24 | 26 |