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PurposeTo evaluate the correlation between clinical presentations, radiological findings and high risk histopathological features of primary enucleated eyes in patients with advanced retinoblastoma at a tertiary child hospital in Thailand.Materials and methodsWe retrospectively reviewed the medical records of patients who were treated with primary enucleation of tumor eyes between 2015-2020. Demographic data, radiological assessment, and histopathological findings were collected. The association between clinical presentations and high-risk histopathological features in the primary enucleated eyes were evaluated. The radiological findings, which showed characteristic of high risk features, were compared with the histopathological findings.ResultsThirty-three enucleated eyes were enrolled in this study. The mean age at diagnosis was 23.12 months. Most patients had unilateral group E retinoblastoma, with no difference in sex and laterality of the eye. Leukocoria was the most common presentation, followed by proptosis and uveitis. Older age at presentation were statistically associated with post laminar cribrosa optic nerve invasion (P-value 0.0027) and high-risk histopathological features in enucleated eyes (P-value 0.0032). Clinical presentations with proptosis were statistically associated with post laminar cribrosa optic nerve invasion, while leukocoria and uveitis were statistically associated with anterior segment invasion. Unifocal intraocular mass with necrosis was the most common histopathological finding. High-risk features were found in 45% of primary enucleated eye. The sensitivity and specificity of magnetic resonance imaging (MRI) for detected optic nerve invasion in retinoblastoma patients were 75% and 54%, respectively.ConclusionPatients with unilateral retinoblastoma who presented with older age related to high-risk features after enucleation. Ophthalmic examination with slitlamp is the best way for detection of anterior segment invasion. Choroidal invasion was unable to predict with clinical presentation. MRI was the better imaging for detection of post laminar cribrosa optic nerve invasion.