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Mortality from second tumour among long-term survivors of retinoblastoma: a retrospective analysis of the Italian retinoblastoma registry

Oncogene volume 25, pages 5350–5357 (2006)Cite this article

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)

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Acknowledgements

We thank the following colleagues for their kind collaboration in the data collection: C Costagliola, Department of Ophthalmology, University of Napoli; P Fiorani, Department of Pediatrics, University of Ancona; ML Pinello and G Perilongo, Department of Pediatrics, University of Padova; PL Marradi, Department of Pediatrics, University of Verona, A Garaventa and B De Bernardi, Istituto G Gaslini, Genova; L Notarangelo, Department of Pediatric, University of Brescia; G Bernini, Department of Pediatrics, University of Firenze; C Ferrari and P Picci, Istituto Ortopedico Rizzoli; V Ninfo and R Alaggio, Department of Pathology, University of Padova; A Schiavetti, Department of Pediatrics, University ‘La Sapienza’, Roma; F Fossati-Bellani and R Luksch, Department of Pediatrics, Istituto Nazionale per lo studio e la cura dei tumori, Milano; B Ricci, Department of Ophthalmology, University Cattolica del Sacro Cuore, Roma; M Aricò, Unit of Pediatric, Hematology-Oncology, Ospedale ‘G. Di Cristina’, Palermo; A Zanasso, Department of Pediatric, University of Trieste; M Comis, Divisione di Ematologia, Azienda Ospedaliera di Reggio Calabria, Reggio Calabria; G Murgia, Department of Pediatrics, University of Cagliari; S Calabrò, Divisione di Oculistica, Ospedale Santobono, Napoli; N Delle Noci, Department of Ophthalmology, University of Bari; P Perri, Department of Ophthalmology, University of Ferrara; Magnani C, Registro Tumori Infantili del Piemonte; S Ferretti, Registro dei Tumori della Provincia of Ferrara, Dipartimento di Medicina sperimentale e diagnostica, University of Ferrara; P Zambon, Registro Tumori del Veneto, Azienda Ospedaliera of Padova, Padova; F Bellu’, Registro Tumori Alto-Adige; JE Kingston, St Bartholomew's Hospital, London; Munier F and Balmer A, Clinique Ophthalmic, Université de Lausanne; T Lehnert, Klinische Forschergruppe Ophthalmologische Onkologie un Genetik, Universitatsklinikum Essen , Germany; J Schipper, Arnhems Radiotherapeutisch Instituut, Arnhem, The Netherlands. This study was supported partly by a grant from the Italian Association for Research on Retinoblastoma (AIRR).

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Authors and Affiliations

  1. Department of Pediatrics, Italian Retinoblastoma Registry, Obstetrics and Reproductive Medicine, University of Siena, Siena, Italy

    A Acquaviva, R Ancarola & G Morgese

  2. Department of Preventive and Predictive Medicine, Epidemiology Unit, Istituto Nazionale per lo Studio e la Cura dei Tumori, Milano, Italy

    L Ciccolallo

  3. Department of Pediatrics, University of Bologna, Bologna, Italy

    R Rondelli

  4. Department of Surgery and Bioengineering, University of Siena, Siena, Italy

    A Balistreri

  5. Department of Pediatric Oncology, Bambino Gesu Pediatric Hospital, Roma, Italy

    R Cozza

  6. Department of Ophthalmological Science, University of Siena, Siena, Italy

    D Hadjistilianou & S De Francesco

  7. Department of Human Pathology and Oncology, University of Siena, Siena, Italy

    P Toti

  8. Childhood Cancer Registry of Piedmont, Cancer Epidemiology Unit, University of Torino, Torino, Italy

    G Pastore

  9. Department of Hematology-Oncology Istituto Gaslini, Genoa, Italy

    R Haupt

  10. Division of Hematology/Oncology, Department of Pediatrics, University-Hospital of Padova, Padova, Italy

    M Carli

  11. Department of Biomedicine in Childhood, University of Bari, Bari, Italy

    N Santoro

  12. Centre of Paediatric Haematology and Oncology, University of Catania, Catania, Italy

    A Di Cataldo

  13. Department of Pediatrics, University of Federico II of Napoli, Napoli, Italy

    A Fiorillo

  14. Pediatric Oncology Service, Second University of Napoli, Napoli, Italy

    P Indolfi

  15. Pediatric Ophthalmology & Strabismus Unit, San Paolo Hospital, University of Milano, Milano, Italy

    P Nucci

  16. Pediatric Oncology Department, University of Torino, Torino, Italy

    A Sandri

  17. Department of Pediatrics, University of Brescia, Brescia, Italy

    F Porta

  18. Department of Urology, Civil Major Hospital, Verona, Italy

    A B Porcaro

  19. Department of Pediatrics, Ospedale Infantile Burlo Garofolo, University of Trieste, Trieste, Italy

    P Tamaro

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  1. A Acquaviva
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  14. A Di Cataldo
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  15. A Fiorillo
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  16. P Indolfi
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  18. A Sandri
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  19. F Porta
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  20. A B Porcaro
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  21. P Tamaro
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  22. G Morgese
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Corresponding author

Correspondence to A Acquaviva.

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Acquaviva, A., Ciccolallo, L., Rondelli, R. et al. Mortality from second tumour among long-term survivors of retinoblastoma: a retrospective analysis of the Italian retinoblastoma registry. Oncogene 25, 5350–5357 (2006). https://doi.org/10.1038/sj.onc.1209786

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