Abstract
Purpose: To elucidate the clinical features which predict high-risk histopathological factors for subsequent metastatic disease as well as to report the incidence of these high-risk histopathological factors in a cohort of Asian patients with retinoblastoma.
 Design: A retrospective and non-randomized sequential cases series.
 Methods: A retrospective study was done on 334 eyes with retinoblastoma at Vietnam National Institute of Ophthalmology during a 10 year period (January 2004 – December 2013). All pathology specimens and medical records were reviewed and assessed for invasion and clinical signs.
 Results: Among 334 eyes, 225 (67.4%) had high-risk retinoblastoma and 109 (22.6%) had non-high-risk features on histopathology. The high-risk histopathological features included anterior chamber seeding (48.2%), iris infiltration (14.7%), ciliary body involvement (14.1%), massive choroidal invasion (29.9 %), post-laminar optic nerve invasion (21.2%), invasion of optic nerve transection (9.6 %), combined choroidal and optic nerve invasion (9.6 %), scleral invasion (3.3%), and extra-scleral infiltration (11.4%). The significant clinical features in high-risk group versus non-high-risk group included hyphema (19.6% vs 3.7%, p < 0.001), pseudohypopyon (19.1% vs 6.4%, p = 0.001), iris neovascularization (25.3% vs 5.5%, p < 0.001), vitreous seeding (72.4% vs 37.6%, p < 0.001), staphyloma (24% vs 4.6%, p < 0.001) and scleritis (20% vs 3.7%, p < 0.001).
 Conclusions: Clinical signs including hyphema, pseudohypopyon, iris neovascularization, vitreous seeding, staphyloma, and scleritis were significantly associated with high-risk features on histopathology. Globe preserving methods should be used with caution in patients with these signs.
Highlights
Retinoblastoma (Rb) is the most common malignant intraocular tumor in children with an incidence of one in 15000-20000 live births.[1]
The use of adjuvant chemotherapy after enucleation has been associated with increased survival of patients at risk of metastasis
High-risk features on histopathology were defined as the presence of anterior segment involvement (Fig. 1), massive (≥ 3 mm) choroidal invasion, post-laminar optic nerve invasion, optic nerve margin involvement, combined non-massive choroidal and prelaminar/laminar optic nerve invasion, or scleral/extra-scleral infiltration.[4,5,6]
Summary
Retinoblastoma (Rb) is the most common malignant intraocular tumor in children with an incidence of one in 15000-20000 live births.[1] In developed countries, the cure rate for children with Rb is greater than 95%.2. The success rate is limited to less than 50% in developing countries because of late diagnosis and insufficient treatment.[3] The use of adjuvant chemotherapy after enucleation has been associated with increased survival of patients at risk of metastasis. Studies have shown that histopathologic factors may predict high-risk metastatic tumors. Those can be listed as anterior chamber seeding, iris invasion, ciliary body infiltration, massive choroidal infiltration, post-laminar optic nerve invasion, optic nerve margin involvement, combined choroidal and optic nerve infiltration, and sclera/ extra-scleral invasion.[4,5,6]
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