Statistics — retinoblastoma¶
Last curated: 2026-09-01 (independent audit, same date; every figure below was re-checked against a live PubMed record).
Every row names population, year/era and method. Conflicting estimates are shown side by side and are not averaged.
Global burden and presentation¶
| Measure | Estimate | Population / era | Method | Source |
|---|---|---|---|---|
| Annual diagnoses | ~8,000 | Worldwide; estimate current to 2015 | disease-primer synthesis | Dimaras 2015, PMID 27189421 |
| Global cohort size | 4,064 | 149 countries; diagnosed 2017 | prospective cluster cohort | Global Study Group 2022, PMID 35839812 |
| Participating centres | 260 | 149 countries | prospective recruitment | PMID 35839812 |
| Median age at diagnosis | 23.2 months (IQR 11.0–36.5) | 2017 global cohort | patient-level median | PMID 35839812 |
| cT4, high-income | 0.8% (5/636) | 2017 | cTNMH staging | PMID 35839812 |
| cT4, upper-middle-income | 5.4% (55/1,027) | 2017 | cTNMH staging | PMID 35839812 |
| cT4, lower-middle-income | 19.7% (342/1,738) | 2017 | cTNMH staging | PMID 35839812 |
| cT4, low-income | 42.9% (196/457) | 2017 | cTNMH staging | PMID 35839812 |
| Enucleation availability | 100% of enrolled children | 2017 global centres | centre report | PMID 35839812 |
| IV chemotherapy availability | 98.8% (4,014/4,064) | 2017 global centres | centre report | PMID 35839812 |
| Incidence, Europe | 4.0 per million children 0–14 (95% CI 3.9–4.1) | 81 registries, 31 countries, 2000–2013 | population-based registry | Virgili 2024, PMID 39388193 |
| 5-year survival, Europe | 97.8% (95.5–98.9) | 3,180 cases | population-based registry | PMID 39388193 |
| Incidence, Argentina | 5.0 per million children 0–14 (95% CI 3.5–6.4) | 438 patients, 2000–2009 | national paediatric registry | Moreno 2014, PMID 24729462 |
| Incidence, Brazil | 2.23 per million (world-standardised, 0–14) | 675 patients, 28 registries, 2000–2018 | population-based registry | Barbosa 2022, PMID 36507141 |
| Incidence, Ethiopia | 1 per 52,156 live births | 221 patients, 4 hospitals, 2017–2020 | birth-cohort analysis; authors call it an underestimate | Sherief 2023, PMID 37221479 |
| Asia-Pacific share of global burden | 43% (3,452 of 8,099) | 2013 estimates, 6 countries | modelled apportionment | Jain 2019, PMID 30385881 |
| Median age at diagnosis, high- vs low-income | 14.1 vs 30.5 months | 4,351 patients, 153 countries, 2017 | cross-sectional | Fabian 2020, PMID 32105305 |
| Extraocular at diagnosis, low-income | 49.1% (256/521) | same | cross-sectional | PMID 32105305 |
| Metastasis at diagnosis, low-income | 18.9% (94/498) | same | cross-sectional | PMID 32105305 |
| Advanced disease, low- vs upper-middle/high-income | OR 17.92 (95% CI 12.94–24.80) | same, age-adjusted | logistic regression | PMID 32105305 |
| Genetic testing available | 74 of 145 countries (51.0%) | 438 centres, 2024 | cross-sectional service survey | Global Study Group 2026, PMID 42463304 |
| Intra-arterial chemotherapy available | 69 of 145 countries (47.6%) | same | service survey | PMID 42463304 |
| Intravitreal chemotherapy available | 101 of 145 countries (69.7%) | same | service survey | PMID 42463304 |
| Countries with no identified Rb centre | 49 | 2024 | service survey | PMID 42463304 |
Income-stratified survival¶
| Stratum | 3-year survival | Population | Method | Source |
|---|---|---|---|---|
| High income | 99.5% (95% CI 98.8–100.0) | diagnosed 2017 | time-to-event | PMID 35839812 |
| Upper-middle income | 91.2% (89.5–93.0) | diagnosed 2017 | time-to-event | PMID 35839812 |
| Lower-middle income | 80.3% (78.3–82.3) | diagnosed 2017 | time-to-event | PMID 35839812 |
| Low income | 57.3% (52.1–63.0) | diagnosed 2017 | time-to-event | PMID 35839812 |
| Low- vs high-income mortality | HR 16.67 (95% CI 4.76–50.00) | 2017 global cohort | adjusted Cox model | PMID 35839812 |
| cT4 vs cT1 mortality | HR 8.98 (4.44–18.18) | 2017 global cohort | adjusted Cox model | PMID 35839812 |
| Age effect to 3 years | HR 1.38 per year (1.23–1.56) | 2017 global cohort | adjusted Cox model | PMID 35839812 |
Delay and abandonment¶
| Measure | Estimate | Population / era | Method | Source |
|---|---|---|---|---|
| Symptom-to-treatment lag | mean 4.2 months; range 0.5–61.6 | 1,120 Indian children; prospective | interval decomposition | Das 2025, PMID 40719713 |
| Parent-lag share | 44% | same | component share | PMID 40719713 |
| Diagnosis-lag share | 26% | same | component share | PMID 40719713 |
| Treatment-lag share | 31% | same | component share | PMID 40719713 |
| Extraocular disease at diagnosis | 25.2% | same | prospective staging | PMID 40719713 |
| Stage III abandonment | 38.5% | single resource-limited cohort | retrospective | Pant 2017, PMID 29337595 |
| Stage IV abandonment | 46.6% | same | retrospective | PMID 29337595 |
| Ethiopian lag | mean 14.31 months; range 0.25–62.25 | 38 caregivers | cross-sectional phone survey | Sherief 2023, PMID 36803347 |
| ≥3-month first-care delay | 76.3% | same | caregiver report | PMID 36803347 |
| ≥1 additional facility before treatment centre | 97.4% | same | referral history | PMID 36803347 |
Classification and eye salvage¶
| System / group | Eye salvage | Population / treatment | Method | Source |
|---|---|---|---|---|
| cT1 | 95% (139/147) | 565 eyes; systemic chemotherapy + focal | retrospective prediction | Yousef 2021, PMID 33769386 |
| cT2 | 56% (230/410) | same | same | PMID 33769386 |
| cT3 | 0% | same | same | PMID 33769386 |
| ICRB A | 98% | same | same | PMID 33769386 |
| ICRB B | 93% | same | same | PMID 33769386 |
| ICRB C | 76% | same | same | PMID 33769386 |
| ICRB D | 44% | same | same | PMID 33769386 |
| Focal seeds | 62% | same | morphology subgroup | PMID 33769386 |
| Diffuse seeds | 42% | same | morphology subgroup | PMID 33769386 |
| High-risk pathology after primary enucleation | 55.1% (751/1,362) | 16 centres, 11 countries | retrospective | Kurian 2025, PMID 39922380 |
| PPV of group D for high-risk pathology | 42.0% / 35.1% / 43.2% | ICRB Philadelphia / ICRB LA / COG | same cohort | PMID 39922380 |
| PPV of group E for high-risk pathology | 58.5% / 59.0% / 59.5% | same three schemes | same cohort | PMID 39922380 |
| Group D eyes upstaged to E by ICRB vs IIRC | 17% (57/150) | 332 advanced eyes, single institution | reclassification | Singh 2024, PMID 38830602 |
| 12-month ocular survival, ICRB D / E1 / E2 / E3 | 79% / 59% / 49% / 1% | same | Kaplan-Meier | PMID 38830602 |
| Primary enucleation rate for group D, by IIRC version | 8% (Philadelphia), 34% (COG), 37% (CHLA) | 39 centres, 1,807 group D eyes | survey | Scelfo 2017, PMID 28730089 |
| Curable stage (IRSS 0/I/II) by presenting sign | strabismus 94.8%, leukocoria 90.1%, orbital mass 33.7% | 4,578 patients, 121 countries | cross-sectional | Yanagihara 2025, PMID 40334716 |
| 5-year disease-specific survival by pT stage | pT1 99.5%, pT2a 95.5%, pT3b 93.0%, pT3c/d 92.3%, pT4 40.9% | 700 primarily enucleated, 29 centres | retrospective | Zhao 2021, PMID 34944860 |
| Diffuse infiltrating retinoblastoma frequency | 4.6% (132/2,854 eyes; 95% CI 3.9–5.5) | international registry, 2001–2013 | registry series | Tomar 2025, PMID 40738737 |
| 5-year survival, cT3 diffuse infiltrating vs non-diffuse | 82% (78–86) vs 94% (93–95), P<0.001 | same | Kaplan-Meier | PMID 40738737 |
Intra-arterial chemotherapy¶
| Measure | Estimate | Population / era | Method | Source |
|---|---|---|---|---|
| 2-year progression-free globe salvage, IAC | 53% (38/72) | unilateral group D/E; six Chinese hospitals | randomized trial | Wen 2023, PMID 37536351 |
| Same, IV chemotherapy | 27% (19/71) | same | randomized trial | PMID 37536351 |
| Relative effect | RR 1.97 (95% CI 1.27–3.07) | same | intention-to-treat | PMID 37536351 |
| Myelosuppression, IAC | 51% (37/72) | same | prespecified safety | PMID 37536351 |
| Myelosuppression, IV | 70% (50/71) | same | prespecified safety | PMID 37536351 |
| Ophthalmic-artery occlusion | 3% (2/72) | IAC arm | safety | PMID 37536351 |
| Ophthalmic-artery stenosis | 18% (13/72) | IAC arm | safety | PMID 37536351 |
| Group D OAC avoidance of enucleation/EBRT | 78.6% (88/112) | single-centre; mixed prior treatment | retrospective | Abramson 2016, PMID 26756643 |
| Treatment-naive group D | 85.1% (40/47) | same | subgroup | PMID 26756643 |
| Primary IAC group D salvage | 94% | 70-eye series | retrospective | Shields 2014, PMID 24656794 |
| Primary IAC group E salvage | 36% | same | retrospective | PMID 24656794 |
| Pooled globe salvage, IAC | 73% (95% CI 68–77) | 43 studies, 3,174 eyes | random-effects meta-analysis | Weinberger 2025, PMID 40541288 |
| Pooled globe salvage, group D vs E | 66% (60–71) vs 49% (39–59) | same | meta-analysis | PMID 40541288 |
| IAC vs IV overall success | 75.7% (65.7–83.6) vs 69.5% (51.9–82.8), P<0.001 | 26 studies, 1,541 eyes | meta-analysis | Chen 2018, PMID 29703164 |
| IAC vs IV, group D salvage | 79.5% (71.8–85.4) vs 55.1% (45.6–64.2), P<0.001 | same | meta-analysis | PMID 29703164 |
| Multi-institutional IAC feasibility success | 67% | 14 patients, 9 institutions (COG ARET12P1) | prospective feasibility | Chintagumpala 2024, PMID 37817345 |
| Catheterisation success, single high-volume centre | 98.5% of procedures | 95 eyes, 289 injections | prospective registry | Gobin 2011, PMID 21320950 |
Intravitreal chemotherapy¶
| Measure | Estimate | Population | Method | Source |
|---|---|---|---|---|
| Injections in initial safety series | 135 in 30 eyes | selected eyes | retrospective | Munier 2012, PMID 22368262 |
| Extraocular spread | 0 detected | same; median follow-up 13.5 months | clinical/pathologic observation | PMID 22368262 |
| Vitreous haemorrhage | 3/135 injections | same | adverse events | PMID 22368262 |
| Continuing seed regression | 100% (40/40 eyes) | median 3-year follow-up | retrospective series | Shields 2016, PMID 26630319 |
| Globe salvage | 88% (35/40) | same | eye-level outcome | PMID 26630319 |
| Complete regression | 78% (21/27) | recurrent/refractory seeds | retrospective | Yousef 2021, PMID 34322023 |
| Focal-seed regression | 100% (10/10) | same | subgroup | PMID 34322023 |
| Diffuse-seed regression | 65% (11/17) | same | subgroup | PMID 34322023 |
| Any ocular adverse effect | 59% (16/27) | same | adverse-event collection | PMID 34322023 |
Enucleation pathology and adjuvant treatment¶
| Measure | Estimate | Population | Method | Source |
|---|---|---|---|---|
| 5-year DFS, 3 CEV cycles | 90.4% | 187 unilateral high-risk enucleated patients | randomized noninferiority trial | Ye 2024, PMID 39432296 |
| 5-year DFS, 6 cycles | 89.2% | same | randomized trial | PMID 39432296 |
| Difference | 1.2% (95% CI −7.5 to 9.8) | same | noninferiority margin 12% | PMID 39432296 |
| RFS with adjuvant chemotherapy for isolated massive choroidal invasion | 97.2% | 60-child observational cohort | retrospective comparison | Feng 2023, PMID 37603354 |
| RFS without adjuvant chemotherapy | 55.6% | same | retrospective comparison | PMID 37603354 |
| OS with vs without adjuvant | 97.2% vs 66.7% | same | retrospective comparison | PMID 37603354 |
| Number treated to prevent one relapse/death | 3 | same | derived estimate | PMID 37603354 |
| Massive choroidal invasion by continent (AS/AUS/EUR/NA/SA) | 31% / 7% / 13% / 19% / 27% (P=0.001) | 1,426 primarily enucleated eyes | retrospective global | Kaliki 2024, PMID 39151183 |
| Postlaminar optic-nerve invasion by continent | 27% / 0% / 16% / 21% / 19% (P=0.0006) | same | retrospective global | PMID 39151183 |
| High-risk features present after primary enucleation | 36% (145/403) | Asian Indian case-control | retrospective | Kaliki 2015, PMID 25841975 |
| Orbital recurrence without vs with adjuvant chemotherapy (PLONI) | 31% vs 2% (P<0.001) | 292 patients, 9 countries | retrospective cohort | Lin 2026, PMID 41475682 |
| Metastasis subhazard ratio, no adjuvant chemotherapy (PLONI) | 5.39 (95% CI 1.75–16.60) | same | competing-risks model | PMID 41475682 |
| 2-year EFS with vs without high-risk features (COG) | 0.96 (0.89–0.98) vs 0.99 (0.96–1.0) | 321 enucleated unilateral, central review | prospective | Chévez-Barrios 2019, PMID 31539297 |
| Central-vs-local histopathology disagreement | 23% of high-risk, 17% of non-high-risk cases | same | central review | PMID 31539297 |
| Unanimous high-risk features among specialists | 3 of 15 listed features (100% agreement) | 27 respondents, 16 countries | survey | Kaliki 2022, PMID 34762098 |
Extraocular and trilateral disease¶
| Measure | Estimate | Population | Method | Source |
|---|---|---|---|---|
| 1-year EFS stage II–III | 88.1% (90% CI 66.6–96.2) | ARET0321, 57 eligible total | prospective trial | Dunkel 2022, PMID 35820112 |
| 1-year EFS stage IVa | 82.6% (61.0–92.9) | same | prospective trial | PMID 35820112 |
| 1-year EFS stage IVb/trilateral | 28.3% (12.7–46.2) | same | prospective trial | PMID 35820112 |
| Therapy-related deaths | 2 | same | safety | PMID 35820112 |
| Heritable trilateral risk | 3.78% | 607-patient prospective cohort | incidence | de Bloeme 2024, PMID 38992673 |
| Follow-up scans per pineal tumour detected | 494 | all cystic/suspicious glands followed | diagnostic yield | PMID 38992673 |
| Scans per tumour if limited to suspicious glands | 22 | same | modeled protocol restriction | PMID 38992673 |
| Baseline MRI missed later asynchronous pineal TRb | 89% | meta-analysis, 197 cases | pooled case timing | de Jong 2022, PMID 33939299 |
| Scans per asymptomatic pineal TRb detected (6-monthly to 36 months) | ≥311 | meta-analysis, 138 pineal TRb cases | modelled screening yield | de Jong 2020, PMID 32061409 |
| Scans per life saved, same schedule | ≥776 | same | modelled | PMID 32061409 |
| Time from diagnosis to orbital disease (untreated) | median 13.7 months | 44 children who abandoned treatment | retrospective natural history | Zhao 2021, PMID 34359552 |
| Time from metastasis to death (untreated) | median 2.0 months | same | retrospective | PMID 34359552 |
| Mortality by 12 / 24 / 36 / 48 months (untreated) | 36% / 77% / 95% / 100% | same | retrospective | PMID 34359552 |
Subsequent malignancies — do not merge heritability groups¶
| Measure | Estimate | Population / era | Method | Source |
|---|---|---|---|---|
| Any SMN SIR, heritable | 11.9 (95% CI 10.4–13.5) | 1,128 survivors; diagnosis 1914–2006 | cohort vs general population | Schonfeld 2021, PMID 33473166 |
| Any SMN SIR, non-heritable | 0.8 (0.5–1.2) | 924 survivors | same | PMID 33473166 |
| First SMN cumulative incidence at 50 years | 33.1% (29.0–37.2) | heritable | competing-risk | PMID 33473166 |
| Second SMN cumulative incidence at 50 years | 6.0% (3.8–8.2) | heritable | competing-risk | PMID 33473166 |
| Melanoma/CNS/oral/breast SIR range | 3.1–17 across four sites | heritable | site-specific SIRs summarized as range | PMID 33473166 |
| Melanoma SIR, Danish cohort | 26.78 (9.78–58.30) | 133 heritable survivors; 1943–2013 | registry cohort | Gregersen 2020, PMID 33090227 |
| Radiation-associated sarcoma RR at ≥60 Gy | 10.7 | nested case-control | dose-response | Wong 1997, PMID 9333268 |
| Head/neck bone sarcoma SIR | 2,213 (1,671–2,873) | 952 irradiated heritable survivors | cohort | Kleinerman 2019, PMID 31622129 |
| Body/extremity bone sarcoma SIR | 169 (115–239) | same | cohort | PMID 31622129 |
| 50-year SMN mortality, heritable | 25.5% (20.8–30.2) | US long-term survivors | competing-risk | Yu 2009, PMID 19351917 |
| 50-year SMN mortality, non-heritable | 1.0% (0.2–1.8) | same | competing-risk | PMID 19351917 |
Vision and psychosocial outcomes¶
| Measure | Estimate | Population | Method | Source |
|---|---|---|---|---|
| Detailed visual-testing sample | 11 survivors | age 5–17 | cross-sectional | Reynolds 2023, PMID 37442536 |
| Impaired contrast/saccade parameters | 10/10 completing tests | same | objective testing | PMID 37442536 |
| AYA QOL sample | 101 survivors | adolescent/young adult | cross-sectional | Belson 2023, PMID 36520266 |
| Variance in overall QOL explained by depression + social participation | 38% | same | regression | PMID 36520266 |
| Additional variance from vision psychosocial domain | 16% | same | regression | PMID 36520266 |
| School-age psychosocial sample | 69 survivors | mean age 10.89 years | survivor/parent survey | Morse 2023, PMID 36385462 |
| Ethiopian qualitative sample | 13 parents | all care phases | phenomenological interviews | Sherief 2023, PMID 36545916 |
| Strabismus after successful salvage | 69% of 42 patients | mean 93.7 months follow-up | retrospective | Fabian 2018, PMID 30009948 |
| Deviation before vs after strabismus surgery (eso) | 42.0 ± 10.4 → 8.5 ± 5.3 prism dioptres (P<0.001) | 18 salvaged patients | retrospective | Masoomian 2024, PMID 38481156 |
| Foveal atrophy after macular retinoblastoma | 77% (36/47 eyes) | globe salvaged in all | retrospective | Chandra 2022, PMID 37006899 |
| Retinoblastoma-specific PROMs available | 1 of 143 identified instruments | 110 eligible studies | scoping review | Janic 2020, PMID 32770435 |
Known conflicts and caveats¶
- The 51% 50-year second-cancer estimate in Wong 1997 and 33.1% in Schonfeld 2021 differ by cohort, era, endpoint and competing-risk methods; they must not be averaged (PMIDs: 9333268, 33473166).
- The Danish melanoma SIR 26.78 is a small national cohort estimate with a wide CI; it is not the melanoma-specific figure from Schonfeld (PMIDs: 33090227, 33473166).
- Globe-salvage estimates are treatment-, stage- and centre-selected and are not useful-vision probabilities.
- The global income analysis uses national income categories; it cannot isolate individual household income or one causal delay mechanism.
- Small zero-event procedural series cannot prove zero metastatic risk.
- Percentages may not sum exactly because of rounding.
- Incidence figures use incompatible denominators — per million children 0–14, per million 0–19, and per live birth — and differ in registry completeness (13% of Brazilian cases were death-certificate-only). They are listed side by side and must not be pooled (PMIDs: 39388193, 36507141, 37221479, 24729462).
- Trilateral screening yield estimates of ≥311 scans per detected tumour (modelled, 2020), 494 scans (prospective, all cystic glands, 2024) and 22 scans (prospective, suspicious glands only, 2024) describe different protocols, not conflicting facts (PMIDs: 32061409, 38992673).
- Melanoma SIR 26.78 (Danish) and the 3.1–17 multi-site range (Schonfeld) are different quantities from different cohorts; the Danish overall heritable SIR (11.39) and Schonfeld's (11.9) are close, but the non-heritable estimates differ (1.52 vs 0.8) (PMIDs: 33090227, 33473166).
- Meta-analytic IAC-versus-IV comparisons carry unequal follow-up (mean 21.7 vs 49.4 months), which biases the metastasis comparison toward IAC (PMID 29703164).