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Treatment for eye cancer varies by the type and by how advanced it is. It may include surgery, radiation therapy, freezing or heat therapy, or laser therapy.

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—      Those with calcium and/or protein build-up, or those who are not capable of adequately cleaning their ocular appliance

—      Those who are currently cleaning with just soap and water

—      Those who suffer infections due to bacteria build-up

—      Ocularists as a practice builder and to promote patient ocular hygiene

—      Health care professionals (nurses, caregivers, etc.) who are responsible for the care and cleaning of patients’ plastic artificial eyes or scleral shells

—      Not for use with glass artificial eyes

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PURPOSE:

Many epidemiologic studies indicate an increased incidence of certain vision threatening conditions in postmenopausal women. These data suggest that changes in sex steroid homeostasis may affect the physiology of the eye. To provide support to this hypothesis, the expression of estrogen receptor alpha (ER) in human eye tissues was investigated.

METHODS:

 Complementary studies including RNA analysis by reverse transcription polymerase chain reaction, western blot analysis, and immunocytochemistry were used to provide evidence of ER expression. Protein detection was carried out using a mouse monoclonal antibody raised against an epitope located in the ligand binding domain of the human receptor. Cellular localization was studied on formalin-fixed paraffin-embedded eye sections using conventional immunohistochemical techniques.

RESULTS:

 Gender and age differences in ER mRNA expression were observed in retina. The 65-kDa ER protein was detected in the retina and retinal pigment epithelium (RPE) of young female eyes but not in eye tissues dissected from men and postmenopausal women. Immunocytochemistry corroborated ER staining of a young female neurosensory retina and RPE. In addition, ER could be detected in the ciliary body, in the iris, and in the epithelium of the lens.

CONCLUSIONS:

 The presence of the ER in the human eye suggests that the sex steroid hormone axis may play a role in the pathogenesis of certain ocular diseases.

Significant gender-based differences in the incidence of many important ocular conditions raise the possibility that estrogens may have direct effects on the eye. Disorders such as age-related macular degeneration (AMD), idiopathic full-thickness macular hole, and cataract have been conclusively associated with gender and age in several epidemiologic studies. These investigations demonstrate an increased prevalence of high morbidity eye diseases in the elderly, and in postmenopausal women in particular.1 2 3 Furthermore, an elderly female population under estrogen replacement therapy has shown a reduced prevalence of nuclear cataract, indicating some beneficial effects of estrogens on lens physiology.4 Although other epidemiologic studies have suggested the prevalence of dry eye and glaucoma among the elderly, especially women, they involved different populations and different clinical end points and as such are inconclusive.5 6 7 Thus, the body of epidemiologic data points to an involvement of estrogens in the normal physiology of the human eye. A single study reported previously by Gans et al. failed to find estrogen and progesterone receptors in conjunctival human tissues.8

Estrogens are steroid hormones long known for their profound effects on both male and female reproductive systems. Estrogens regulate growth, differentiation, and function of diverse tissues both within and outside the reproductive system. The effects of estrogens are mediated by specific nuclear receptors, the estrogen receptor (ER) and ß types, that act as hormone-inducible transcription factors.9 10 11 Relatively recent findings demonstrate important roles of the steroid hormones in the cardiovascular system, in specific brain regions, in the liver, and in the maintenance of bone tissue. Despite the wide range of tissues influenced by estrogens and the broad transcriptional regulatory properties of the ligand-activated ERs, the literature contains limited evidence of these receptors in normal ocular tissues. This, and the large spectrum of physiological alterations occurring in women after the normal hormonal decline of menopause, or in males during andropause, motivated our studies. We report here that the type of estrogen receptor is normally present in the human eye and that gender and age differences may influence its expression.

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Retinoblastoma

General Information about Retinoblastoma

Retinoblastoma is a disease in which malignant (cancer) cells form in the tissues of the retina.

The retina is the nerve tissue that lines the inside of the back of the eye. The retina senses light and sends images to the brain by way of the optic nerve.

Although retinoblastoma may occur at any age, it usually occurs in children younger than 5 years of age. The tumor may be in one eye or in both eyes. Retinoblastoma rarely spreads from the eye to nearby tissue or other parts of the body. Retinoblastoma is usually found in only one eye and can usually be cured.

Retinoblastoma is sometimes caused by a gene mutation passed from the parent to the child.

Retinoblastoma is sometimes inherited (passed from the parent to the child). Retinoblastoma that is caused by an inherited gene mutation is called hereditary retinoblastoma. It usually occurs at a younger age than retinoblastoma that is not inherited. Retinoblastoma that occurs in only one eye is usually not inherited. Retinoblastoma that occurs in both eyes is always inherited. When hereditary retinoblastoma first occurs in only one eye, there is a chance it will develop later in the other eye. After diagnosis of retinoblastoma in one eye, regular follow-up exams of the healthy eye should be done every 2 to 4 months for at least 28 months. After treatment for retinoblastoma is finished, it is important that follow-up exams continue until the child is 7 years of age.

Treatment for both types of retinoblastoma should include genetic counseling (a discussion with a trained professional about inherited diseases). Brothers and sisters of a child who has retinoblastoma should also have regular check-ups and genetic counseling about the risk of developing the cancer.

A child who has hereditary retinoblastoma is at risk for developing trilateral retinoblastoma and other cancers.

A child who has hereditary retinoblastoma is at risk for developing pineal tumors in the brain. This is called trilateral retinoblastoma. Regular follow-up exams to check for this rare condition are important during treatment and for at least 4 years after the child is diagnosed with retinoblastoma. Hereditary retinoblastoma also increases the child’s risk of developing other types of cancer in later years. Regular follow-up exams are important.

Possible signs of retinoblastoma include “white pupil” and eye pain or redness.

These and other symptoms may be caused by retinoblastoma. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

—      Pupil of the eye appears white instead of red when light shines into it. This may be seen in flash photographs of the child.

—      Eyes appear to be looking in different directions.

—      Pain or redness in the eye.

Tests that examine the retina are used to detect (find) and diagnose retinoblastoma.

The following tests and procedures may be used:

—      Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken. The doctor will ask if there is a family history of retinoblastoma.

—      Eye exam with dilated pupil: An exam of the eye in which the pupil is dilated (opened wider) with medicated eyedrops to allow the doctor to look through the lens and pupil to the retina. The inside of the eye, including the retina and the optic nerve, is examined with a light. Depending on the age of the child, this exam may be done under anesthesia.

—      Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.

—      CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the eye, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.

—      MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the eye. This procedure is also called nuclear magnetic resonance imaging (NMRI).

Retinoblastoma is usually diagnosed without a biopsy (removal of cells or tissues so they can be viewed under a microscope to check for signs of cancer).

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

—      The stage of the cancer.

—      How likely it is that vision can be saved in one or both eyes.

—      The size and number of tumors.

—      Whether trilateral retinoblastoma occurs.

Stages of Retinoblastoma

After retinoblastoma has been diagnosed, tests are done to find out if cancer cells have spread within the eye or to other parts of the body.

The process used to find out if cancer has spread within the eye or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process:

—      Eye exam with dilated pupil: An exam of the eye in which the pupil is dilated (opened wider) with medicated eyedrops to allow the doctor to look through the lens and pupil to the retina. The inside of the eye, including the retina and the optic nerve, is examined using a light. Depending on the age of the child, this exam may be done under anesthesia.

—      Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.

—      CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the eye, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.

—      MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the eye. This procedure is also called nuclear magnetic resonance imaging (NMRI).

—      Lumbar puncture: A procedure used to collect cerebrospinal fluid from the spinal column. This is done by placing a needle into the spinal column. This procedure is also called an LP or spinal tap. A lumbar puncture may be done if tests show that the cancer may have spread out of the eye.

There are several staging systems for retinoblastoma. For treatment, retinoblastoma is classified as intraocular (within the eye) or extraocular (outside the eye).

The following stages are used for retinoblastoma:

Intraocular retinoblastoma

Cancer is found in the eye but has not spread to tissues around the outside of the eye or to other parts of the body.

Extraocular retinoblastoma

The cancer has spread beyond the eye. It may be found in tissues around the eye or it may have spread to the central nervous system (brain and spinal cord) or to other parts of the body.

Recurrent Retinoblastoma

Recurrent retinoblastoma is cancer that has recurred (come back) after it has been treated. The cancer may recur in the eye, in tissues around the eye, or in other places in the body. Tumors that were not treated with radiation therapy or surgery commonly recur, usually within 6 months.

Treatment Option Overview

There are different types of treatment for patients with retinoblastoma.

Different types of treatment are available for patients with retinoblastoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. Before starting treatment, patients may want to think about taking part in a clinical trial. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.

Children with retinoblastoma should have their treatment planned by a team of doctors with expertise in treating cancer in children.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric doctors who are experts in treating children with eye cancer and who specialize in certain areas of medicine. These may include a pediatric ophthalmologist (children’s eye doctor) who has a lot of experience in treating retinoblastoma and the following specialists:

—      Pediatric surgeon.

—      Pediatric hematologist.

—      Radiation oncologist.

—      Neurologist.

—      Pediatric nurse specialist.

—      Rehabilitation specialist.

—      Psychologist.

—      Social workers.

—      Geneticist.

Some cancer treatments cause side effects months or years after treatment has ended.

Some cancer treatments cause side effects that continue or appear months or years after cancer treatment has ended. These are called late effects. Late effects of cancer treatment may include physical problems; changes in mood, feelings, thinking, learning or memory; and second cancers (new types of cancer.) Some late effects may be treated or controlled. It is important to talk with your child’s doctors about the possible late effects caused by some treatments. (Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.)

Children with the inherited form of retinoblastoma have an increased risk of developing second cancers. Children who have been treated for retinoblastoma with radiation therapy or certain chemotherapy agents also have a risk of developing second cancers. Regular follow-up by health professionals who are expert in finding and treating late effects is important.

Six types of standard treatment are used:

Enucleation

Enucleation is surgery to remove the eye and part of the optic nerve. The eye will be checked with a microscope to see if there are any signs that the cancer is likely to spread to other parts of the body. This is done if the tumor is large and there is little or no chance that vision can be saved. The patient will be fitted for an artificial eye after this surgery.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, plaques, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. Methods of radiation therapy used to treat retinoblastoma include the following:

—      Intensity-modulated radiation therapy (IMRT): A type of 3-dimensional (3-D) radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles. This type of radiation therapy causes less damage to healthy tissue near the tumor.

—      Stereotactic radiation therapy: Radiation therapy that uses a rigid head frame attached to the skull to aim high-dose radiation beams directly at the tumors, causing less damage to nearby healthy tissue. It is also called stereotactic external-beam radiation, stereotactic radiosurgery, and stereotaxic radiosurgery. This procedure does not involve surgery.

—      Proton beam radiation therapy: Radiation therapy that uses protons made by a special machine. A proton is a type of high-energy radiation that is different from an x-ray.

—      Plaque radiotherapy: Radioactive seeds are attached to one side of a disk, called a plaque, and placed directly on the outside wall of the eye near the tumor. The side of the plaque with the seeds on it faces the eyeball, aiming radiation at the tumor. The plaque helps protect other nearby tissue from the radiation.

Cryotherapy

Cryotherapy is a treatment that uses an instrument to freeze and destroy abnormal tissue, such as carcinoma in situ. This type of treatment is also called cryosurgery.

Photocoagulation

Photocoagulation is a procedure that uses laser light to destroy blood vessels to the tumor, causing the tumor cells to die. Photocoagulation may be used to treat small tumors. This is also called light coagulation.

Thermotherapy

Thermotherapy is the use of heat to destroy cancer cells. Thermotherapy may be given using a laser beam aimed through the dilated pupil or onto the outside of the eyeball, or using ultrasound, microwaves, or infrared radiation (light that cannot be seen but can be felt as heat).

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ (such as the eye), or a body cavity (such as the abdomen), the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated.

A form of chemotherapy called chemoreduction is used to treat retinoblastoma. Chemoreduction reduces the size of the tumor so it may be treated with local treatment (such as radiation therapy, cryotherapy, photocoagulation, or thermotherapy).

New types of treatment are being tested in clinical trials. These include the following:

Subtenon chemotherapy

Subtenon chemotherapy is the use of drugs injected through the membrane covering the muscles and nerves at the back of the eyeball. This is a type of regional chemotherapy. It is usually combined with systemic chemotherapy and local treatment (such as radiation therapy, cryotherapy, photocoagulation, or thermotherapy).

High-dose chemotherapy with stem cell transplant

High-dose chemotherapy with stem cell transplant is a method of giving high doses of chemotherapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body’s blood cells.

This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.

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Home Care:

For fatigue or eyestrain, try to rest your eyes. No treatment is necessary.

If you have conjunctivitis:

—      Avoid touching the infected eye and then rubbing the other eye — the infection can spread from one eye to the other.

—      Apply cool or warm compresses throughout the day.

—      Over-the-counter homeopathic eye drops may provide relief.

—      Do not use eye makeup or wear contact lenses until the infection has cleared. Throw away items like these that you used in your infected eye.

—      Wash your hands frequently.

If you have blepharitis:

—      Apply warm compresses to your eyes for 5 minutes. Do this at least 2 times per day.

—      Using a cotton swab, gently rub a solution of warm water and no-tears baby shampoo along your eyelid, where the lash meets the lid. Do this in the morning and before you go to bed.

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—      In a convergent squint, the affected eye is directed too far inwards, and in divergent squint the eye is directed outwards.

—      In rare cases, vertical strabismus exists, where one eye is directed either upwards or downwards, compared with the other eye.

—      Squint affects 3 to 5% of the population, and it is the commonest reason for infant and childhood surgery.

—      Most children with squints are otherwise normal, but there is an increased incidence in premature babies and those with cerebral palsy.

—      Many babies develop a squint, because they have not developed the basic ability to align up both eyes when fixing their vision on a single onject. Nevertheless, all children with a squint, no matter how mild, need to be referred to an eye specialist the sooner it is treated the better the outcome. In very rare cases a potentially fatal tumour of childhood, a retinoblastoma, could present as a squint.

—      A younger child with a squint has double vision, and so the brain counteracts this by suppressing the image from the deviating, or ‘out of line eye’. This affected eye loses sharpness of vision, called amblyopia, in an attempt to enhance the sharp image from the good eye. By the age of eight, this system of image suppression is so set that the resultant defect cannot be corrected.

—      In older children longsightedness (hypermetropia), can disturb perfect alignment of the eyes, with one eye attempting to excessively focus on close up objects and so producing inturning of one eye.

—      Children with squints upto about 6yrs are encouraged to wear a patch to cover the normal eye, so that the weak eye is ‘forced to see’ objects. This strenghthens connections between the weaker eye and the brain areas controlling vision. Any remaining deviation of the affected eye can be corrected, if needed, later by surgery or wearing special glasses.

—      Wearing a patch is an effective way of correcting a lazy eye, but try telling the child that! The British Orthoptic Society has started a Patch Club, and all 3,000 children between 1 and 9 who wear patches for ‘lazy eye’ can join and receive all sorts of goodies. Send an A4 sae to Nicky Edwards, Patch Club, ASt. Woolos Hospital, Stow Hill, newport, Gwent NP9 4SZ.

—      Squints in adults may result from strokes, multiple sclerosis, diabetes or over active thyroid disease, and must also be investigated.

IMPORTANT NOTICE: This content is from the Dr Chris Steele personal archive and is provided for convenience only. Information contained here may no longer agree with the most up to date medical advice. Please check with a medical professional before taking any action.

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