Second and subsequent tumours among 1927 retinoblastoma patients diagnosed in Britain 1951-2004
Background: Retinoblastoma is an eye tumour of childhood that occurs in heritable and non-heritable forms. In the heritable form, there is a predisposition to the development of non-ocular subsequent primary tumours (SPTs). Methods: This study included 1927 retinoblastoma patients diagnosed in Brita...
| Main Authors: | , , , , , , , , , , , |
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| Format: | Journal article |
| Language: | English |
| Published: |
2013
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| _version_ | 1797080240471670784 |
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| author | MacCarthy, A Bayne, A Brownbill, P Bunch, K Diggens, N Draper, G Hawkins, M Jenkinson, H Kingston, J Stiller, C Vincent, T Murphy, M |
| author_facet | MacCarthy, A Bayne, A Brownbill, P Bunch, K Diggens, N Draper, G Hawkins, M Jenkinson, H Kingston, J Stiller, C Vincent, T Murphy, M |
| author_sort | MacCarthy, A |
| collection | OXFORD |
| description | Background: Retinoblastoma is an eye tumour of childhood that occurs in heritable and non-heritable forms. In the heritable form, there is a predisposition to the development of non-ocular subsequent primary tumours (SPTs). Methods: This study included 1927 retinoblastoma patients diagnosed in Britain from 1951 to 2004. Ascertainment was through the (UK) National Registry of Childhood Tumours; cases were followed-up for the occurrence of SPTs. Standardised incidence ratios (SIRs) were calculated. Results: We identified 169 SPTs in 152 patients. The SIR analysis included 145 SPTs with cancer registrations from the years 1971 to 2009. These tumours occurred in 132 patients: 112 of the 781 heritable and 20 of the 1075 (presumed) non-heritable cases under surveillance at the start of this period developed at least one registered SPT. The SIRs for all tumours combined were 13.7 (95% confidence interval 11.3-16.5) in heritable cases and 1.5 (0.9-2.3) in non-heritable cases. The main types of SPT in the heritable cases were leiomyosarcoma, (31 cases; SIR 1018.7 (692.2-1446.0)), osteosarcoma (26 cases; SIR 444.6 (290.4-651.4)), and skin melanoma (12 cases; SIR 18.6 (9.6-32.4)). Conclusion: The risk of SPTs in heritable retinoblastoma is extremely high. This has important implications for the clinical follow-up and counselling of survivors and their families. © 2013 Cancer Research UK. All rights reserved. |
| first_indexed | 2024-03-07T00:57:19Z |
| format | Journal article |
| id | oxford-uuid:888581c7-4b38-460b-9e1f-2327c1285bde |
| institution | University of Oxford |
| language | English |
| last_indexed | 2024-03-07T00:57:19Z |
| publishDate | 2013 |
| record_format | dspace |
| spelling | oxford-uuid:888581c7-4b38-460b-9e1f-2327c1285bde2022-03-26T22:17:52ZSecond and subsequent tumours among 1927 retinoblastoma patients diagnosed in Britain 1951-2004Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:888581c7-4b38-460b-9e1f-2327c1285bdeEnglishSymplectic Elements at Oxford2013MacCarthy, ABayne, ABrownbill, PBunch, KDiggens, NDraper, GHawkins, MJenkinson, HKingston, JStiller, CVincent, TMurphy, MBackground: Retinoblastoma is an eye tumour of childhood that occurs in heritable and non-heritable forms. In the heritable form, there is a predisposition to the development of non-ocular subsequent primary tumours (SPTs). Methods: This study included 1927 retinoblastoma patients diagnosed in Britain from 1951 to 2004. Ascertainment was through the (UK) National Registry of Childhood Tumours; cases were followed-up for the occurrence of SPTs. Standardised incidence ratios (SIRs) were calculated. Results: We identified 169 SPTs in 152 patients. The SIR analysis included 145 SPTs with cancer registrations from the years 1971 to 2009. These tumours occurred in 132 patients: 112 of the 781 heritable and 20 of the 1075 (presumed) non-heritable cases under surveillance at the start of this period developed at least one registered SPT. The SIRs for all tumours combined were 13.7 (95% confidence interval 11.3-16.5) in heritable cases and 1.5 (0.9-2.3) in non-heritable cases. The main types of SPT in the heritable cases were leiomyosarcoma, (31 cases; SIR 1018.7 (692.2-1446.0)), osteosarcoma (26 cases; SIR 444.6 (290.4-651.4)), and skin melanoma (12 cases; SIR 18.6 (9.6-32.4)). Conclusion: The risk of SPTs in heritable retinoblastoma is extremely high. This has important implications for the clinical follow-up and counselling of survivors and their families. © 2013 Cancer Research UK. All rights reserved. |
| spellingShingle | MacCarthy, A Bayne, A Brownbill, P Bunch, K Diggens, N Draper, G Hawkins, M Jenkinson, H Kingston, J Stiller, C Vincent, T Murphy, M Second and subsequent tumours among 1927 retinoblastoma patients diagnosed in Britain 1951-2004 |
| title | Second and subsequent tumours among 1927 retinoblastoma patients diagnosed in Britain 1951-2004 |
| title_full | Second and subsequent tumours among 1927 retinoblastoma patients diagnosed in Britain 1951-2004 |
| title_fullStr | Second and subsequent tumours among 1927 retinoblastoma patients diagnosed in Britain 1951-2004 |
| title_full_unstemmed | Second and subsequent tumours among 1927 retinoblastoma patients diagnosed in Britain 1951-2004 |
| title_short | Second and subsequent tumours among 1927 retinoblastoma patients diagnosed in Britain 1951-2004 |
| title_sort | second and subsequent tumours among 1927 retinoblastoma patients diagnosed in britain 1951 2004 |
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