A Review of Childhood Ocular, Orbital, and Surface Tumors With Updated Clinical Management

Abstract

Childhood tumor is differs from adult tumor but has a wide range varieties in types and presentation due to its origin from different embryogenic layers. As Pediatric patients mostly depend on their parents or caregivers for seeking medical attention, many ocular tumor present with unnecessary delay. Most childhood ocular tumors are benign, but there are also life threatening tumors. In this study, a variety of intraocular, orbital and surface tumors are reviewed with their key sign of diagnosis and current treatment modalities. Though most of these tumor present at tertiary eye hospitals, the overall knowledge about these tumors can help ophthalmologists in diagnosis and proper referral.

Genetic Risk Factors and Clinical Outcomes in Childhood Eye Cancers: A Review

“…Medulloepithelioma is a childhood malignant intraocular tumor. It is a congenital tumor originating from the primitive medullary epithelium [ 1 , 32 ]. It is mainly located in the non-pigmented ciliary body epithelium and can also occur in the retina, iris, and optic nerve [ 32 ].…”

“…The incidence of primary eye tumors in childhood is rare compared to adult tumors. For instance, in the United Kingdom, between 1963 and 2002, the annual incidence of eye tumors in children under 15 years was 3.5 per million [ 1 ]. According to the Surveillance, Epidemiology, and End Results (SEER) programme database, researchers analyzed 658 retinoblastoma cases between 1975 and 2004 and found an annual incidence rate of 11.8 per million in children under 5 years of age in the US [ 1 , 2 ].…”

“…For instance, in the United Kingdom, between 1963 and 2002, the annual incidence of eye tumors in children under 15 years was 3.5 per million [ 1 ]. According to the Surveillance, Epidemiology, and End Results (SEER) programme database, researchers analyzed 658 retinoblastoma cases between 1975 and 2004 and found an annual incidence rate of 11.8 per million in children under 5 years of age in the US [ 1 , 2 ]. Despite their rarity, pediatric ocular tumors are significantly more debilitating, causing vision impairment and blindness in early life, as well as death in the cases of ocular malignancy [ 3 ].…”

“…Despite their rarity, pediatric ocular tumors are significantly more debilitating, causing vision impairment and blindness in early life, as well as death in the cases of ocular malignancy [ 3 ]. Pediatric eye tumors are commonly congenital with early presentation, but they may also present later in childhood [ 1 , 4 ]. Although benign ocular tumors are more common in children, a small percentage can develop malignant and metastatic forms [ 1 , 5 ].…”

“…Pediatric eye tumors are commonly congenital with early presentation, but they may also present later in childhood [ 1 , 4 ]. Although benign ocular tumors are more common in children, a small percentage can develop malignant and metastatic forms [ 1 , 5 ].…”