2 3 Purpose: The aim of this study was to investigate if there was a relationship between ethnicity 4 or socioeconomic status and the presentation of advanced non-familial retinoblastoma in the 5 UK. 6 7 Methods: A cross sectional study based at the two centres providing retinoblastoma care in the 8 UK. Non-familial cases of retinoblastoma (Rb) presenting between January 2006 and December 9 2011 were included. Data collected included: age at diagnosis , gender, child's ethnicity, 10 International Intraocular Retinoblastoma Classification (IIRC) stage with Groups D and E being 11 considered advanced, laterality, treatment, and postcodes. Individual postcode (ZIP code) data 12 was used to obtain the Index of Multiple Deprivation (IMD) score. A postal questionnaire was 13 sent to participants' parents to collect further, person-level, information on languages spoken 14 and household socioeconomic position. Measures of severity of retinoblastoma also included: 15 requirement for primary enucleation; the use of adjuvant chemotherapy; and mortality. 16 17 Results: 189 cases were analyzed. 98 (52%) male and 91 (48%) female. Median age at 18 diagnosis was 16 months [IQR 8 -34 months]. 153/189 (81%) of cases presented with 19 advanced retinoblastoma; 75 (40%) group E, 78 (41%) group D. 134 (72%) of cases were 20 treated with enucleation. 21 Multivariable analysis showed that older age at presentation was associated with enucleation 22 and bilateral disease was associated with adjuvant chemotherapy. There was some indication 23 that South Asian ethnicity and being in the most deprived IMD quintile were associated with a 24 higher likelihood of presentation with advanced disease, but these estimates did not reach 25 statistical significance. 26 27 Conclusions: In this first national UK study of patients with non-familial retinoblastoma, there 28 was no evidence of an association of ethnicity or socio-economic status and the risk of 29 presenting with advanced disease. This may reflect equality in access of health care in the UK. 30 As a result, awareness campaigns should continue. 31 32 33 34 35 36 37 38 128 (93%) of whom had advanced disease. Those presenting with advanced retinoblastoma and 129 those treated with enucleation were similarly distributed by demographic and socioeconomic 130 factors to all cases (Table 1), as were those receiving adjuvant chemotherapy (68, 50% of those 131 that were enucleated).
133Multivariable analysis showed children 2 years or older and those with bilateral retinoblastoma 134 were more likely to present with advanced disease (Table 2). Older age at presentation was 135 associated with enucleation and bilateral disease with receipt of adjuvant chemotherapy. There
157(enucleation rate and adverse histopathology). Of note, there was no statistically significant 158 association between the factors listed in Table 3 and advanced disease (IIRC Groups D and 159 E).160 161 162 163 164 165 166 167 168 169 170 201 surgeon may have played a part. In addition, statistical significance was noted in mo...