Eye neoplasm

Eye Neoplasm Microchapters
Patient Information
Overview
Classification Choroidal Tumors Conjunctival Tumors
Causes
Differential Diagnosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Roukoz A. Karam, M.D.[2]

Synonyms and keywords: Eye neoplasm; eye cancer

Overview

Ocular oncology is the branch of medicine dealing with tumors relating to the eye and its adnexa. Cancer of the eye is very rare; however, the most common neoplasm of the eye is ocular melanoma. Eye cancer can affect all parts of the eye.

Classification

Tumors in the eye and orbit can be benign like dermoid cysts, or malignant like rhabdomyosarcoma and retinoblastoma. The most common eyelid tumor is called basal cell carcinoma. This tumor can grow around the eye but rarely spreads to other parts of the body. Other types of common eyelid cancers include squamous carcinoma, sebaceous carcinoma and malignant melanoma.

The most common malignant primary intraocular tumor in adults is uveal melanoma. These tumors can occur in the choroid, iris and ciliary body. The latter are sometimes called iris or ciliary body melanoma.

The most common malignant intraocular tumor in children is called retinoblastoma. Affecting approximately 325 children per year in North America, early detection has allowed for cures exceeding 95%.

The most common orbital malignancy is orbital lymphoma. This tumor can be diagnosed by biopsy with histopathologic and immunohistochemical analysis. Most patients with orbital lymphoma can be offered chemotherapy or radiation therapy.

Orbital dermoid cysts are benign choristomas which are typically found at the junction of sutures, most commonly at the fronto-zygomatic suture. Large deep orbital dermoid cysts can have cause pressure effects on the muscles and optic nerve, leading to diplopia and loss of vision.

Choroidal tumors

• Choroidal hemangioma

• Choroidal melanoma

• Choroidal metastasis

• Choroidal nevus

• Choroidal osteoma

• Ciliary body melanoma

• The nevus of Ota

Conjunctival tumors

• Conjunctival Kaposi's sarcoma

• Epibulbar dermoid

• Malignant conjunctival tumors

• Lymphoma of the conjunctiva

• Melanoma and PAM with atypia

• Pigmented conjunctival tumors

• Pingueculum

• Pterygium

• Squamous carcinoma and intraepithelial neoplasia of the conjunctiva

Risk Factors

Risk factors that increase the chance of suffering from cancer of the eye include:

• Light skin and/or blue eyes
• Exposure to sun or tanning beds
  • Increase risk of eyelid carcinomas or melanomas
• Exposure to certain viruses
  • Human papilloma virus may increase the risk of squamous cell carcinoma of conjunctiva
• Atypical mole and melanoma syndrome (originally called dysplastic nevus syndrome)

Diagnosis

• Melanomas (choroidal, ciliary body and uveal) - In the early stages there may be no symptoms (the person does not know there is a tumor until an ophthalmologist or optometrist looks into the eye with an ophthalmoscope during a routine test). As the tumor grows, symptoms can be blurred vision, decreased vision, double vision, eventual vision loss and if they continue to grow the tumor can break past the retina causing retinal detachment. Sometimes the tumor can be visible through the pupil.

• Nevus - Are benign, freckle in the eye. These should be checked out and regular checks on the eye done to ensure it hasn't turned into a melanoma.

• Iris and conjuctival tumors (melanomas) - Presents as a dark spot. Any spot which continues to grow on the iris and the conjunctiva should be checked out.

• Retinoblastoma - Strabismus (crossed eyes), a whitish or yellowish glow through the pupil, decreasing/loss of vision, sometimes the eye may be red and painful. Retinoblastoma can occur in one or both eyes. This tumor occurs in babies and young children. It is called RB for short. Check photographs, normal healthy eyes would have the red eye reflex, but a white/yellow dot instead of the red eye reflex can indicate a tumor or some other kind of eye disease. Any photos of a child/children which have a white/yellow dot instead of the red eye reflex should be checked out.

Treatment

Surgery

• Laser therapy
• Plaque therapy
• Radiotherapy
• Enucleation of the Eye - Removal of the eye, but the muscles and eyelids are left intact. An implant is inserted, then the person wears a conformer shield and later the person will have their prosthesis made and fitted (the prosthesis is made by someone called an ocularist and is made to look like their real eye)
• Evisceration - Removal of the eye contents, leaving the sclera or the white part of the eye.
• Exenteration - Removal of the eye, all orbital contents, which can involve the eyelids as well. A special prosthesis is made to cover the defect and improve appearance.
• Iridectomy - Removal of the affected piece of the iris
• Choroidectomy - Removal of the choroid layer (the vascular tissue sandwiched between the sclera and the retina)
• Iridocyclectomy - Removal of the iris plus the ciliary body muscle.
• Eyewall resection - Cutting into the eye to remove a tumor e.g. melanoma. This operation can be quite difficult to perform.

Related Chapters

• List of eye diseases and disorders