Ocular oncology: diagnosis and management of malignant intraocular tumors

Abstract

In this article, we review the key clinical and diagnostic features, histological characteristics, treatment options and prognosis for malignant intraocular tumors, including uveal melanoma, retinoblastoma, metastatic uveal tract and retinal tumors, tumors of the pigmented and nonpigmented epithelia, intraocular lymphoma and medulloepithelioma. The findings of the Collaborative Ocular Melanoma Study have demonstrated that most patients with uveal melanoma can have good local control with brachytherapy and that the risk of metastasis remains the same whether enucleation or brachytherapy is performed. New insights regarding the molecular classification of uveal melanomas may help select both patients at risk of metastasis and those who may be candidates for adjuvant systemic therapy. In the last decade, chemoreduction for retinoblastoma has revolutionized the management of this tumor in children. The nuances of chemoreduction in combination with local forms of treatment delivery will be discussed in this review. The recent developments in the management of intraocular lymphoma will also be highlighted.

Keywords::

• ciliary pigment epithelium adenocarcinoma
• intraocular lymphoma
• medulloepithelioma
• ocular oncology
• retinal pigment epithelium adenocarcinoma
• retinoblastoma
• uveal melanoma