PMID- 16936757 OWN - NLM STAT- MEDLINE DCOM- 20060928 LR - 20100412 IS - 0950-9232 (Print) IS - 0950-9232 (Linking) VI - 25 IP - 38 DP - 2006 Aug 28 TI - Mortality from second tumour among long-term survivors of retinoblastoma: a retrospective analysis of the Italian retinoblastoma registry. PG - 5350-7 AB - 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). FAU - Acquaviva, A AU - Acquaviva A AD - Italian Retinoblastoma Registry, Department of Pediatrics, Obstetrics and Reproductive Medicine, University of Siena, Siena, Italy. acquaviva@unisi.it FAU - Ciccolallo, L AU - Ciccolallo L FAU - Rondelli, R AU - Rondelli R FAU - Balistreri, A AU - Balistreri A FAU - Ancarola, R AU - Ancarola R FAU - Cozza, R AU - Cozza R FAU - Hadjistilianou, D AU - Hadjistilianou D FAU - Francesco, S De AU - Francesco SD FAU - Toti, P AU - Toti P FAU - Pastore, G AU - Pastore G FAU - Haupt, R AU - Haupt R FAU - Carli, M AU - Carli M FAU - Santoro, N AU - Santoro N FAU - Di Cataldo, A AU - Di Cataldo A FAU - Fiorillo, A AU - Fiorillo A FAU - Indolfi, P AU - Indolfi P FAU - Nucci, P AU - Nucci P FAU - Sandri, A AU - Sandri A FAU - Porta, F AU - Porta F FAU - Porcaro, A B AU - Porcaro AB FAU - Tamaro, P AU - Tamaro P FAU - Morgese, G AU - Morgese G LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, N.I.H., Extramural PL - England TA - Oncogene JT - Oncogene JID - 8711562 SB - IM MH - Cohort Studies MH - Functional Laterality MH - Germ-Line Mutation MH - Humans MH - Italy MH - Neoplasms, Second Primary/*mortality MH - Registries MH - Retinal Neoplasms/genetics/*pathology MH - Retinoblastoma/genetics/*pathology MH - Survival Analysis MH - Survivors EDAT- 2006/08/29 09:00 MHDA- 2006/09/29 09:00 CRDT- 2006/08/29 09:00 PHST- 2006/08/29 09:00 [pubmed] PHST- 2006/09/29 09:00 [medline] PHST- 2006/08/29 09:00 [entrez] AID - 1209786 [pii] AID - 10.1038/sj.onc.1209786 [doi] PST - ppublish SO - Oncogene. 2006 Aug 28;25(38):5350-7. doi: 10.1038/sj.onc.1209786.