Clinical features and survival among children with retinoblastoma in Uganda

Keith M Waddell; Kenneth Kagame; Andrew Ndamira; Amos Twinamasiko; Susan V Picton; Ian G Simmons; Tom Johnston; Robert Newton

doi:10.1136/bjophthalmol-2014-305564

Clinical features and survival among children with retinoblastoma in Uganda

Keith M Waddell, Kenneth Kagame, Andrew Ndamira, Amos Twinamasiko, Susan V Picton, Ian G Simmons, Tom Johnston, Robert Newton

Health Sciences

Research output: Contribution to journal › Article › peer-review

Abstract

AIMS: To characterise the clinical features, treatment and outcome of children diagnosed with retinoblastoma in Uganda.

METHODS: The study comprised a 6-year nationwide enrolment with follow-up.

RESULTS: In total, 282 cases were enrolled, 26% (72) were bilateral; 6% were lost to follow-up. Almost all diagnoses in the first affected eye were International Classification of Retinoblastoma group E or worse. Histology was available for 92%; of those, 45%, had extraocular tumour at diagnosis. Enucleation of the first eye was done for 271; 94 received radiotherapy to the socket and in the last 2 years, 70 children received chemotherapy. At close of study, 139 children had died. Survival, as determined in a proportional hazards model adjusted for age, sex, laterality and treatment era (pre or post introduction of chemotherapy), varied by extent of the tumour (p<0.001); children with only intraocular involvement were 80% less likely to die (HR=0.21, 95% CI 0.12 to 0.35) compared with children with extraocular involvement.

CONCLUSIONS: Diagnostic delay results in relatively high mortality among children with retinoblastoma in Uganda. There is an urgent need for more effective treatment modalities, particularly chemotherapy, and nationwide efforts to encourage earlier access to medical care.

Original language	English
Pages (from-to)	387-390
Journal	British Journal of Ophthalmology
Volume	99
Issue number	3
DOIs	https://doi.org/10.1136/bjophthalmol-2014-305564
Publication status	Published - 2015

Bibliographical note

Published by the BMJ Publishing Group Limited. This is an author-produced version of the published paper. Uploaded in accordance with the publisher’s self-archiving policy. Further copying may not be permitted; contact the publisher for details

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10.1136/bjophthalmol-2014-305564

Br J Ophthalmol-2014-Waddell-bjophthalmol-2014-305564
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http://bjo.bmj.com/content/99/3/387

Cite this

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title = "Clinical features and survival among children with retinoblastoma in Uganda",

abstract = "AIMS: To characterise the clinical features, treatment and outcome of children diagnosed with retinoblastoma in Uganda.METHODS: The study comprised a 6-year nationwide enrolment with follow-up.RESULTS: In total, 282 cases were enrolled, 26% (72) were bilateral; 6% were lost to follow-up. Almost all diagnoses in the first affected eye were International Classification of Retinoblastoma group E or worse. Histology was available for 92%; of those, 45%, had extraocular tumour at diagnosis. Enucleation of the first eye was done for 271; 94 received radiotherapy to the socket and in the last 2 years, 70 children received chemotherapy. At close of study, 139 children had died. Survival, as determined in a proportional hazards model adjusted for age, sex, laterality and treatment era (pre or post introduction of chemotherapy), varied by extent of the tumour (p<0.001); children with only intraocular involvement were 80% less likely to die (HR=0.21, 95% CI 0.12 to 0.35) compared with children with extraocular involvement.CONCLUSIONS: Diagnostic delay results in relatively high mortality among children with retinoblastoma in Uganda. There is an urgent need for more effective treatment modalities, particularly chemotherapy, and nationwide efforts to encourage earlier access to medical care.",

author = "Waddell, {Keith M} and Kenneth Kagame and Andrew Ndamira and Amos Twinamasiko and Picton, {Susan V} and Simmons, {Ian G} and Tom Johnston and Robert Newton",

note = "Published by the BMJ Publishing Group Limited. This is an author-produced version of the published paper. Uploaded in accordance with the publisher{\textquoteright}s self-archiving policy. Further copying may not be permitted; contact the publisher for details ",

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T1 - Clinical features and survival among children with retinoblastoma in Uganda

AU - Waddell, Keith M

AU - Kagame, Kenneth

AU - Ndamira, Andrew

AU - Twinamasiko, Amos

AU - Picton, Susan V

AU - Simmons, Ian G

AU - Johnston, Tom

AU - Newton, Robert

N1 - Published by the BMJ Publishing Group Limited. This is an author-produced version of the published paper. Uploaded in accordance with the publisher’s self-archiving policy. Further copying may not be permitted; contact the publisher for details

PY - 2015

Y1 - 2015

N2 - AIMS: To characterise the clinical features, treatment and outcome of children diagnosed with retinoblastoma in Uganda.METHODS: The study comprised a 6-year nationwide enrolment with follow-up.RESULTS: In total, 282 cases were enrolled, 26% (72) were bilateral; 6% were lost to follow-up. Almost all diagnoses in the first affected eye were International Classification of Retinoblastoma group E or worse. Histology was available for 92%; of those, 45%, had extraocular tumour at diagnosis. Enucleation of the first eye was done for 271; 94 received radiotherapy to the socket and in the last 2 years, 70 children received chemotherapy. At close of study, 139 children had died. Survival, as determined in a proportional hazards model adjusted for age, sex, laterality and treatment era (pre or post introduction of chemotherapy), varied by extent of the tumour (p<0.001); children with only intraocular involvement were 80% less likely to die (HR=0.21, 95% CI 0.12 to 0.35) compared with children with extraocular involvement.CONCLUSIONS: Diagnostic delay results in relatively high mortality among children with retinoblastoma in Uganda. There is an urgent need for more effective treatment modalities, particularly chemotherapy, and nationwide efforts to encourage earlier access to medical care.

AB - AIMS: To characterise the clinical features, treatment and outcome of children diagnosed with retinoblastoma in Uganda.METHODS: The study comprised a 6-year nationwide enrolment with follow-up.RESULTS: In total, 282 cases were enrolled, 26% (72) were bilateral; 6% were lost to follow-up. Almost all diagnoses in the first affected eye were International Classification of Retinoblastoma group E or worse. Histology was available for 92%; of those, 45%, had extraocular tumour at diagnosis. Enucleation of the first eye was done for 271; 94 received radiotherapy to the socket and in the last 2 years, 70 children received chemotherapy. At close of study, 139 children had died. Survival, as determined in a proportional hazards model adjusted for age, sex, laterality and treatment era (pre or post introduction of chemotherapy), varied by extent of the tumour (p<0.001); children with only intraocular involvement were 80% less likely to die (HR=0.21, 95% CI 0.12 to 0.35) compared with children with extraocular involvement.CONCLUSIONS: Diagnostic delay results in relatively high mortality among children with retinoblastoma in Uganda. There is an urgent need for more effective treatment modalities, particularly chemotherapy, and nationwide efforts to encourage earlier access to medical care.

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