Sebaceous carcinoma of the eyelid associated with retinoblastoma

Objective: To analyze the association between retinoblastoma (Rb) and sebac eous carcinoma (SC) of the eyelid to improve surveillance of survivors of R b, Design: Case report and systematic literature review. Methods: Ten patients who had SC develop after Rb were identified by system atic literature review, and a child who died with lymph node, lung, and liv er metastases 7 years after irradiation for Rb is described, The data were analyzed by univariate statistics, including cumulative frequency distribut ion plots and Kaplan-Meier analysis, Results: Of 11 children with SC of the eyelid who all had hereditary RE, 9 (82%; 95% confidence interval, 48-98) received a median of 46 Gy (range, 21 -89) of radiotherapy at a median age of 16 months (range, 0.5-15 years) and had SC develop within the field of radiation, Their median age at diagnosi s of SC was 14 years (range, 8-30 years), median diagnostic delay 12 months (range, 6 months-3 years), and median interval from irradiation 11 years ( range, 5-26 years); 7 of them (78%; 95% confidence interval, 40-97) were di agnosed between 5 and 15 years after radiotherapy. SC also developed at the age of 32 and 54 years in two nonirradiated Rb patients, Five patients had regional lymph node metastases after a median time of 12 months (range, 1 month-24 years). The 5-year cumulative probability of survival was 87%. Conclusions: SC df the eyelid may occur in patients with hereditary Rb rega rdless of primary treatment, especially within the field 5 to 15:years afte r radiotherapy, Ophthalmology 2001;108:1124-1128 (C) 2001 by the American A cademy of Ophthalmology.