Aim: Retinoblastoma is the most common primary ocular malignancy in childhood, but little has been documented on the clinical and biological differences in children diagnosed before one year of age. We observed patients in this age group and followed them for up to 19 years.
Methods: This retrospective, descriptive, observational study reviewed the medical records of Mexican patients, who were diagnosed with retinoblastoma before one year of age at a national paediatric hospital from 1995 to 2014. The variables analysed were age at diagnosis, weight, presenting signs, the time from first symptoms to diagnosis, family history, laterality, ocular rescue and survival rate.
Results: The 108 patients had a mean age of 7.65 months and 15.7% had a family history of retinoblastoma. The majority (55.5%) had bilateral retinoblastoma, the most common presenting sign was leukocoria (86.1%), and the most common stage of diagnosis was Group V (84.1%). More than half were underweight for their age. The overall survival rate was 92% and the disease-free survival rate was 84%.
Conclusion: Retinoblastoma is a malignancy that can be present at birth, especially if it is a bilateral hereditary form of the disease. Leukocoria was the main presenting sign. Early diagnosis dramatically improved the prognosis for ocular rescue.
Keywords: Infants; Ocular rescue; Retinoblastoma; Second malignancy; Survival.
©2015 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.