Schonfeld SJ, et al. Long-term risk of subsequent cancer incidence among hereditary and nonhereditary retinoblastoma survivors. British journal of cancer. 2021;124:1312-1319. PMID 33473166

One-paragraph summary

The cohort included 1,128 heritable and 924 non-heritable survivors diagnosed from 1914–2006 and followed through 2016.

Key findings

• Overall SMN SIR was 11.9 (95% CI 10.4–13.5) in heritable and 0.8 (0.5–1.2) in non-heritable survivors.
• Heritable 50-year cumulative incidence was 33.1% for a first and 6.0% for a second SMN.

Limitations

• The abstract's 3.1–17 range spans melanoma, CNS, oral-cavity and breast sites; it is not a melanoma-specific SIR.

Why it matters

It is the study that makes heritability, not survivorship, the unit of risk stratification: an 11.9-fold overall excess in heritable survivors against 0.8 in non-heritable survivors means the two groups cannot share a follow-up protocol or a counselling script. It also narrows the epithelial excess to melanoma, CNS, oral cavity and breast rather than the broader list previously suggested, which removes several organs from the surveillance argument.

Caveat on transportability: the treatment era is historical (diagnoses 1914–2006) and modern local-delivery cohorts are not old enough to reproduce the lifetime estimates.

Cited by wiki pages

• second cancers and survivorship
• overview