title: Mortality from second tumour among long-term survivors of retinoblastoma: a retrospective analysis of the Italian retinoblastoma registry.
abstract: Survivors of retinoblastoma (Rb) are at high risk of dying from second malignant tumour. The occurrence of second malignant neoplasm (SMN) and related mortality in a cohort of 1111 cases from the Italian Retinoblastoma Registry was analysed, considering the possible role of both genetic and iatrogenic causes. Rb patients had a greater than 10-fold excess in overall mortality compared with the general population (standardized mortality ratio (SMR) 10.73, 95% CI 9.00-12.80). Their excess risk attributable to cancers other than Rb was 14.93 95% CI 10.38-21.49). Survivors of hereditary Rb had an SMR for all causes of 16.25 (95% CI 13.20-20.00), whereas their SMR for all cancers was 25.72 (95% CI 17.38-38.07). Survivors of unilateral sporadic Rb had an SMR of 4.12 from all cancers (95% CI 1.55-10.98) and a much higher excess for overall mortality (SMR 13.34, 95% CI 10.74-16.56). As expected, survivors of hereditary Rb had higher mortality from cancers of the bone (SMR 391.90, 95% CI 203.90-753.20) and soft tissue (SMR 453.00, 95% CI 203.50-1008.40), small intestine (SMR 1375.50, 95% CI 344.00-5499.70), nasal cavity (SMR 13.71, 95% CI 1.93-97.35) and cancers of the brain and central nervous system (SMR 41.14, 95% CI 13.2-127.55).
authors.affiliation: Italian Retinoblastoma Registry, Department of Pediatrics, Obstetrics and Reproductive Medicine, University of Siena, Siena, Italy. acquaviva@unisi.it
authors.forename: A
authors.initials: A
authors.lastname: Acquaviva
authors.forename: L
authors.initials: L
authors.lastname: Ciccolallo
authors.forename: R
authors.initials: R
authors.lastname: Rondelli
authors.forename: A
authors.initials: A
authors.lastname: Balistreri
authors.forename: R
authors.initials: R
authors.lastname: Ancarola
authors.forename: R
authors.initials: R
authors.lastname: Cozza
authors.forename: D
authors.initials: D
authors.lastname: Hadjistilianou
authors.forename: S De
authors.initials: SD
authors.lastname: Francesco
authors.forename: P
authors.initials: P
authors.lastname: Toti
authors.forename: G
authors.initials: G
authors.lastname: Pastore
authors.forename: R
authors.initials: R
authors.lastname: Haupt
authors.forename: M
authors.initials: M
authors.lastname: Carli
authors.forename: N
authors.initials: N
authors.lastname: Santoro
authors.forename: A
authors.initials: A
authors.lastname: Di Cataldo
authors.forename: A
authors.initials: A
authors.lastname: Fiorillo
authors.forename: P
authors.initials: P
authors.lastname: Indolfi
authors.forename: P
authors.initials: P
authors.lastname: Nucci
authors.forename: A
authors.initials: A
authors.lastname: Sandri
authors.forename: F
authors.initials: F
authors.lastname: Porta
authors.forename: A B
authors.initials: AB
authors.lastname: Porcaro
authors.forename: P
authors.initials: P
authors.lastname: Tamaro
authors.forename: G
authors.initials: G
authors.lastname: Morgese
mesh_terms: D015331:Cohort Studies; D007839:Functional Laterality; D018095:Germ-Line Mutation; D006801:Humans; D007558:Italy; D016609:Neoplasms, Second Primary / Q000401:mortality*; D012042:Registries; D019572:Retinal Neoplasms / Q000235:genetics / Q000473:pathology*; D012175:Retinoblastoma / Q000235:genetics / Q000473:pathology*; D016019:Survival Analysis; D017741:Survivors