Eye, Orbit, and Adnexal Structures
Summary of Key Points
Incidence
• Primary ocular (eye) and ophthalmic (eye and adnexa) tumors are relatively uncommon.
• The most common primary ocular tumors are choroidal melanoma and retinoblastoma. The most common adnexal tumors are lymphoma, rhabdomyosarcoma, optic nerve glioma, and epithelial and melanocytic malignancies of the eyelid and conjunctiva, respectively.
• Many systemic neoplasms can involve the eye and adnexa, especially breast and lung cancers, as well as lymphoma and leukemia.
Etiology
• The etiology of most ophthalmic tumors is unknown.
• Retinoblastoma is the prototypical model of a genetically transmissible tumor via loss of a tumor suppressor gene.
• Squamous neoplasms of the eyelids and conjunctiva are associated with sun exposure, immunosuppressed states, and viral infections such as human papillomavirus and human immunodeficiency virus.
• Ocular and orbital metastases are frequently unexpected; approximately 25% of ocular metastases are discovered at an occult stage.
Diagnosis
• Intraocular tumors can be directly visualized, greatly facilitating diagnosis, but performing a biopsy is not easy and is limited to special circumstances.
• For intraocular tumors, a combination of funduscopic examination, intravenous angiogram, ultrasonography, and computed tomography/magnetic resonance imaging (CT/MRI) can yield diagnostic accuracies greater than 90% to 95%.
• Orbital and adnexal tumors are diagnosed by CT/MRI imaging and biopsy.
Treatment
• Intraocular tumors are treated with local modalities such as external beam irradiation, brachytherapy, and photocoagulation, with or without chemotherapy. If useful vision cannot be preserved in the eye affected by a tumor, the eye is enucleated.
• Orbital malignancies are frequently treated by excision and radiation/chemotherapy, but exenteration may be necessary in far-advanced sarcomas.
• Eyelid, conjunctival, and corneal malignancies are managed by local excision, with or without topical chemotherapy, cryotherapy, or irradiation.
