Eye

This information should not be used for self-diagnosis or in place of a qualified physician’s care.

Reviewed Aug 2016

The basics

Patients with cancers of the eye and surrounding tissues are treated through Vancouver General Hospitals' Eye Care Centre.
Eyes are the organs of vision. They are a complex organ with many parts:

Layers of the eyeball:

Sclera – the white outer part of the eye.
Middle layer – which contains the iris, the choroid and the ciliary body.
Retina – the inner part of the eye.

Around the eye are other parts, which include the muschles, eyebrows, eyelids, conjunctiva, lacrimal gland, lacrimal sac and drainage system, nerves, fat, vessels and the optic nerve. These are called the adnexal parts of the eye and when cancer is involved, it is referred to as adnexal cancer.

Cancer of this area can be divided into three categories, based where the cancer starts:

The eyeball, and it is called ocular or intraocular cancer.
The orbit - the eye socket, which is the place in the face where the eye sits. Orbital tumours include the bone surrounding the eyeball, plus the muscles and soft tissues that connect between the eyeball and the eyesocket.
The eyelids and conjunctiva, which is the clear thin membrane that coats the outside of the eyeball and the inside of the eyelid. Tumours of the eyelid include all types of skin cancers.

What causes it and who gets it?

Listed below are some of the known risk factors for these cancers. Not all of the risk factors below may cause these cancers, but they may be contributing factors.

Eye cancers can occur at any age.
Some eye cancers can be hereditary.
People with light complexions and light coloured eyes are more at risk for some kinds of eye cancers. Exposure to the sun is a factor, so sunscreen and dark glasses are even more important for people with light eyes and pale complexions.
Statistics:

Can I help to prevent it?

Protect your skin and your eyes from sun damage. Visit the BC Cancer Agency’s Prevention page on Sun Safety for information and resources.

Screening for this cancer

No effective screening program exists for this cancer yet.

Signs and Symptoms

The following is a list of symptoms that may indicate a cancer of the eye, orbit or eyelid. Sometimes there are no early symptoms for eye cancer.

Development of a squint (crossed eyes) especially in a child
A change in vision:

Seeing sparks or flashes or black spots or ‘floaters’, especially if this symptom develops suddenly
A decline in vision, slow or sudden
Distorted vision
Double vision
Physical changes in the eye:

The eye seems to begin bulging out (proptosis) or become displaced (downward, upward or outward).
The appearance of a white area in the centre of the pupil. This is the part of the eye that normally looks black or has a red reflex (especially in some photographs).
Changes in the eyelid, such as drooping, swelling or thickening, or the appearance of the lid such as a dark spot, a lump or elevation

A sore or infection on the eyelid that does not heal
A rare symptom can be inflamed eyes or glaucoma (high pressure behind the eye)
Eye cancers may be found during a routine eye examination

Diagnosis & staging

Diagnosis

These are tests that may be used to diagnose this type of cancer.

A clinical examination using routine and special ophthalmic instruments that allow a full field, three dimensional view of the back of the eye.
Examination of the front portion of the eyeball is done using a biomicroscope, which can be used with special lenses to examine the back part of the eyeball.
CT scans are particularly useful for the study of orbital tumors.
Ultrasound examinations.
Fluorescein angiography is a diagnostic test in which a dye is injected into the blood stream. It can be used to visualize the blood vessels of the eye or of the tumour. This can help to visualize and characterize an ocular tumour.
Ocular fundus colour photography allows visualization and documentation of tumours.
Ophthalmic A and B ultrasound may be used to characterize the size, tissue features and extent of an ocular melanoma or some orbital tumours.
Magnetic resonance imaging (MRI) may also rarely help to identify an ocular tumour and define its size and shape but are mostly used for orbital and adnexal tumors.
A surgical biopsy to take out tissue for examination by pathologists.
A needle biopsy using a small, hollow needle. This is called Fine Needle Aspiration Biopsy (FNAB).

For more information on tests used to diagnose cancer, see our Recommended Websites, Diagnostic Tests section.

Types and Stages

Types

There are many different types of cancer that can occur in the eye.

They may be called ocular tumours, retinoblastoma, ocular melanoma, metastatic tumours of the eyeball, intraocular tumours, choroidal tumours.
Orbital and adnexal tumors include optic nerve tumours, meningioma, glioma, sphenoid wing meningiomas, lymphoma, secondary tumours, soft tissue tumours, rhabdomyosarcoma, fibrous histiocytoma, vascular tumours, capillary hemangiomas, cavernous hemangiomas, metastatic tumours, lacrimal tumours, lymphomas, epithelial tumours, carcinoma or malignant tumours.
Cancer of the eyelids and tumours of the conjunctiva include squamous cell carcinoma, basal cell carcinoma, sebaceous carcinoma or malignant melanoma.

Stages

Staging describes the extent of a cancer. The TNM classification system is used as the standard around the world. In general a lower number in each category means a better prognosis. The stage of the cancer is used to plan the treatment.

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T describes the site and size of the main tumour (primary)

N describes involvement of lymph nodes

M relates to whether the cancer has spread (presence or absence of distant metastases)

Stage 0: Tis N0 M0
Stage IA: T1 N0 M0
Stage IB: T2a N0 M0
Stage IC: T2b N0 M0
Stage II: T3a N0 M0
Stage IIIA: T3b N0 M0
Stage IIIB: Any T N1 M0
Stage IIIC:T4 Any N M0
Stage IV: Any T Any N M1

*From the AJCC Cancer Staging Handbook - 7th Ed. (2010)

Treatment

Cancer therapies can be highly individualized, especially for cancers of the eye and surrounding tissues - your treatment may differ from what is described below.

Patients with cancers of the eye and surrounding tissues are treated through Vancouver General Hospital's Eye Care Centre.

Specialization in treating eye cancers is critical, with ophthalmologists, ocular oncologists, oculoplastic surgeons, pediatricians (pediatric oncologists for childhood tumours) and possibly other surgeons who are skilled in cancer treatment.

Surgery

local excision (cutting out the tumour)
laser treatment
enucleation (removal of the eyeball)

Chemotherapy may be used. It may be given locally or systemically.
Radiation therapy

external beam radiotherapy may be used.
plaques of iodine 125 or radioactive gold can be sewn on the outside of the eye (to the sclera) so that the required amount of radiation therapy can be delivered to the tumour. These are removed one or two weeks later.

Proton therapy uses a narrow proton beam of radiation to destroy the tumour. The procedure is done at TRIUMF on the UBC campus.
Cryotherapy (freezing)
Sometimes, small tumours are simply monitored to see how fast they are growing.

Follow-up after treatment

You will be returned to the care of your family physician or specialist for regular followup. If you do not have a family physician, please discuss this with your BC Cancer oncologist or nurse.
Follow-up testing is based on your type of cancer and your individual circumstances.
Life after Cancer focuses on the issues that cancer survivors can face.

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