title: Clinical features and survival among children with retinoblastoma in Uganda.
abstract: OBJECTIVE
  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.
authors.affiliation: Ruharo Eye Hospital, Mbarara, Uganda Departments of Ophthalmology and Paediatrics, Mbarara University of Science and Technology, Mbarara, Uganda.
authors.forename: Keith M
authors.initials: KM
authors.lastname: Waddell
authors.affiliation: Ruharo Eye Hospital, Mbarara, Uganda Departments of Ophthalmology and Paediatrics, Mbarara University of Science and Technology, Mbarara, Uganda.
authors.forename: Kenneth
authors.initials: K
authors.lastname: Kagame
authors.affiliation: Departments of Ophthalmology and Paediatrics, Mbarara University of Science and Technology, Mbarara, Uganda.
authors.forename: Andrew
authors.initials: A
authors.lastname: Ndamira
authors.affiliation: Departments of Ophthalmology and Paediatrics, Mbarara University of Science and Technology, Mbarara, Uganda.
authors.forename: Amos
authors.initials: A
authors.lastname: Twinamasiko
authors.affiliation: Departments of Ophthalmology and Paediatric Oncology, Leeds Teaching Hospitals NHS Trust, Leeds, UK.
authors.forename: Susan V
authors.initials: SV
authors.lastname: Picton
authors.affiliation: Departments of Ophthalmology and Paediatric Oncology, Leeds Teaching Hospitals NHS Trust, Leeds, UK.
authors.forename: Ian G
authors.initials: IG
authors.lastname: Simmons
authors.affiliation: Epidemiology and Cancer Statistics Group, Department of Health Sciences, University of York, York, UK.
authors.forename: W Tom
authors.initials: WT
authors.lastname: Johnston
authors.affiliation: Epidemiology and Cancer Statistics Group, Department of Health Sciences, University of York, York, UK Medical Research Council/Uganda Virus Research Institute (MRC/UVRI) Research Unit on AIDS, Entebbe, Uganda International Agency for Research on Cancer, Lyon, France.
authors.forename: Robert
authors.initials: R
authors.lastname: Newton
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003037:Cobalt Radioisotopes / Q000627:therapeutic use; D057210:Delayed Diagnosis; D015353:Eye Enucleation; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D019572:Retinal Neoplasms / Q000175:diagnosis* / Q000401:mortality* / Q000628:therapy; D012175:Retinoblastoma / Q000175:diagnosis* / Q000401:mortality* / Q000628:therapy; D015996:Survival Rate; D014454:Uganda / Q000453:epidemiology
chemical_list: D000970:Antineoplastic Agents; D003037:Cobalt Radioisotopes
keywords: Drugs; Epidemiology; Neoplasia; Retina