Retinoblastoma in older children
Reviewed by Annes Ahmeidat

Retinoblastoma is the most common ophthalmic malignancy in childhood and over 90% are diagnosed before the age of five years. It is not often suspected in older children given its rarity and unusual clinical findings which complicates the diagnosis. The authors present a retrospective study using medical records from January 2007 – December 2013 in an Indian ophthalmology department. A total of 610 retinoblastoma patients were identified from which 48 (7.8%) were six-years-old or older at presentation (range 6-31). The characteristics of the children six years or older included: 62.5% male, 15% bilateral retinoblastoma, and none had a family history. The commonest presenting symptoms were white reflex (42%) followed by reduced visual acuity (35%). A median lag time for presentation was nine months and higher at 16 months for stage 3 and 4 retinoblastoma. A total of 14 cases were misdiagnosed initially with endophthalmitis or Coats’ disease. Examination findings at presentation were reported to be: proptosis / enlarged globe (33%), glaucoma (29%) and neovascularisation of the iris (23%). The majority of the 48 children had intraocular disease (54%) followed by 25% with locally advanced disease and 21% with metastatic disease at presentation. The authors highlight the key reasons for misdiagnosis of retinoblastoma in older children as due to a low index of suspicion and the frequency of atypical presentations such as: hyphema, pseudohypopyon, vitreous haemorrhage, glaucoma and cataract. In this study, 46% had atypical symptoms, which the authors compared to their previous study from the same centre which had a rate of 3.3%. The 46% figure also aligns with the literature on atypical features in older age retinoblastoma. This study is the largest to analyse retinoblastoma in an older cohort and has illustrated the importance of maintaining a high index of suspicion to avoid misdiagnosis, delayed treatment and mortality. Atypical presentations make certain diagnoses challenging and this study has highlighted significance of retinoblastoma suspicion in older children.

Retinoblastoma in children older than 6 years of age.
Meel R, Kashyap S, Bakhshi S, et al.
OCULAR ONCOLOGY AND PATHOLOGY
2020;6:395-404.