21st Century Pediatric Cancer Sourcebook: Retinoblastoma (Eye Tumor of the Retina) - Clinical Data for Patients, Families, and Physicians
Edition 1.0 - March 2011
National Cancer Institute
Smashwords Edition
Copyright 2011 Progressive Management
Questions? Suggestions? Comments? Concerns? Please contact the publisher directly at
Remember, the book retailer can't answer your questions, but we can!
* * * * * * * * * * *
Smashwords Edition, License Notes
This ebook is licensed for your personal enjoyment only. This ebook may not be re-sold or given away to other people. If you would like to share this book with another person, please purchase an additional copy for each person you share it with. If you're reading this book and did not purchase it, or it was not purchased for your use only, then you should return to Smashwords.com and purchase your own copy. Thank you for respecting the hard work of this author.
* * * * * * * * * * * *
IMPORTANT NOTE: Information in this e-book is not a substitute for professional medical advice. If you have or suspect that you have any illness, you must consult with a physician or professional healthcare provider! Call 911 and get to the nearest emergency room if you have serious or worsening symptoms.
This material represents a snapshot in time, with authoritative information formatted for ebook reading that was up-to-date at the moment of publication. For the latest cancer updates, please be sure to visit the NCI website:
From our Guide to Leading Medical Websites, here are three valuable sites with authoritative cancer information:
OncoLink * http://www.oncolink.upenn.edu/
eMedicine.com * http://www.emedicine.com/
American Cancer Society (ACS) * http://www.cancer.org/
* * * * * * * * * * * *
This is a privately authored news service and educational publication of Progressive Management. Our publications synthesize official government information with original material - they are not produced by the federal government. They are designed to provide a convenient user-friendly reference work to uniformly present authoritative knowledge that can be rapidly read, reviewed or searched. Vast archives of important data that might otherwise remain inaccessible are available for instant review no matter where you are. This e-book format makes a great reference work and educational tool. There is no other reference book that is as convenient, comprehensive, thoroughly researched, and portable - everything you need to know, from renowned experts you trust. For over a quarter of a century, our news, educational, technical, scientific, and medical publications have made unique and valuable references accessible to all people. Our e-books put knowledge at your fingertips, and an expert in your pocket!
PART ONE
PART TWO
Chapter 1B Late Effects of Treatment for Childhood Cancer - Patient Version
Chapter 2B: Pediatric Supportive Care
Chapter 3B: Clinical Trials Background Information
Chapter 4B: Cancer Clinical Trials -The Basic Workbook
Chapter 5B: Cancer Clinical Trials - The In-Depth Program
Chapter 6B: Clinical Trials at NIH
Chapter 7B: How To Find A Cancer Treatment Trial: A Ten Step Guide
Chapter 8B: Taking Part in Cancer Treatment Research Studies
Chapter 9B: Cancer Clinical Trials
Chapter 10B: Access to Investigational Drugs
Chapter 12B: Taking Time: Support for People with Cancer
Chapter 13B: Facing Forward - Life After Cancer Treatment
Chapter 14B: When Someone You Love Is Being Treated For Cancer
Chapter 15B: Living Beyond Cancer: Finding a New Balance
Chapter 16B: Caring for the Caregiver
Chapter 17B: Young People With Cancer, A Handbook For Parents
Chapter 18B: When Cancer Returns
Chapter 19B: When Someone You Love Has Advanced Cancer / Support for Caregivers
Chapter 20B: Chemotherapy and You
Chapter 21B: Managing Chemotherapy Side Effects - Anemia
Chapter 22B: Managing Chemotherapy Side Effects - Appetite Changes
Chapter 23B: Managing Chemotherapy Side Effects - Bleeding Problems
Chapter 24B: Managing Chemotherapy Side Effects - Constipation
Chapter 25B: Managing Chemotherapy Side Effects - Memory Changes
Chapter 26B: Managing Chemotherapy Side Effects - Mouth and Throat Changes
Chapter 27B: Managing Chemotherapy Side Effects - Nerve Changes
Chapter 28B: Managing Chemotherapy Side Effects - Pain
Chapter 29B: Managing Chemotherapy Side Effects - Skin and Nail Changes
Chapter 30B: Managing Chemotherapy Side Effects - Swelling (Fluid retention)
Chapter 31B: Targeted Cancer Therapies
Chapter 33B : Follow-up Care After Cancer Treatment
Chapter 34B: Radiation Therapy and You
Chapter 36B: Understanding Radiation Therapy, What To Know About External Beam Radiation Therapy
Chapter 37B: Radiation Therapy for Cancer
Chapter 38B: Managing Radiation Therapy Side Effects - What To Do When Your Mouth or Throat Hurts
Chapter 39B: What To Do About Hair Loss (Alopecia)
Chapter 42B: Managing Radiation Therapy Side Effects - Changes When You Urinate
Chapter 43B: Managing Radiation Therapy Side Effects What To Do About Mild Skin Changes
Chapter 45B: General Cancer Information And Resources
Chapter 46B: Cancer And The Environment - What You Need to Know, What You Can Do
Chapter 48B: FDA Warning: Beware of Online Cancer Fraud
Chapter 49B: FDA Office of Oncology Drug Products
Chapter 50B: Understanding the HIPAA Privacy Rule
* * * * * * * * * * * *
Last Modified: 11/29/2010
Retinoblastoma is a disease in which malignant (cancer) cells form in the tissues of the retina. Retinoblastoma is sometimes caused by a gene mutation passed from the parent to the child. A child who has hereditary retinoblastoma is at risk for developing trilateral retinoblastoma and other cancers. Possible signs of retinoblastoma include "white pupil" and eye pain or redness. Tests that examine the retina are used to detect (find) and diagnose retinoblastoma. Certain factors affect prognosis (chance of recovery) and treatment options.
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.
Anatomy of the eye, showing the outside and inside of the eye including the sclera, cornea, iris, ciliary body, choroid, retina, vitreous humor, and optic nerve. The vitreous humor is a gel that fills the center of the eye.
Although retinoblastoma may occur at any age, it usually occurs in children younger than 5 years, most often younger than 2 years. 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 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 thought to be 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 5 years old.
Treatment for both types of retinoblastoma should include genetic counseling (a discussion with a trained professional about inherited diseases). The parents of a child with retinoblastoma should have an eye exam by an ophthalmologist (a doctor with special training in diseases of the eye) and genetic counseling about whether they should be tested for the gene that causes retinoblastoma and the risk of the child's brothers or sisters developing retinoblastoma. The child's brothers and sisters also should have regular eye exams by an ophthalmologist until age 5 years.
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 and usually occurs more than 20 months after retinoblastoma is diagnosed. Regular screening using MRI (magnetic resonance imaging) every 6 months for 5 years may be recommended for a child with hereditary retinoblastoma or with retinoblastoma in one eye and a family history of the disease. CT scans (computerized tomography) should not be used for routine screening to avoid exposing the child to ionizing radiation. Hereditary retinoblastoma also increases the child's risk of developing other types of cancer such as bone or soft tissue sarcoma or melanoma 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.
The age of the patient.
How likely it is that vision can be saved in one or both eyes.
The size and number of tumors.
Whether the patient has glaucoma.
Whether trilateral retinoblastoma occurs.
Stages of Retinoblastoma
Key Points for This Section
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 following stages are used for retinoblastoma:
Intraocular retinoblastoma
Extraocular retinoblastoma
There are three ways that cancer spreads in the body.
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.
Bone marrow aspiration and biopsy: The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer.
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
In intraocular retinoblastoma, cancer is found in the eye and may be only in the retina or may also be in other parts of the eye such as the choroid, ciliary body, or part of the optic nerve. Cancer has not spread to tissues around the outside of the eye or to other parts of the body.
Extraocular retinoblastoma
In extraocular (metastatic) retinoblastoma, 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 such as the bone marrow or lymph nodes.
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
Through tissue. Cancer invades the surrounding normal tissue.
Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.
When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.
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.
Treatment Option Overview
Key Points for This Section
There are different types of treatment for patients with retinoblastoma.
Children with retinoblastoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children.
Some cancer treatments cause side effects months or years after treatment has ended.
Six types of standard treatment are used:
Enucleation
Radiation therapy
Cryotherapy
Thermotherapy
Chemotherapy
New types of treatment are being tested in clinical trials.
Subtenon chemotherapy
Ophthalmic arterial infusion therapy
High-dose chemotherapy with stem cell rescue
Biologic therapy
Patients may want to think about taking part in a clinical trial.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Follow-up tests may be needed.
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. 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.
Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.
Children with retinoblastoma should have their treatment planned by a team of health care providers who are experts 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 health care providers 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.
Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following:
Physical problems such as problems with seeing clearly.
Changes in mood, feelings, thinking, learning, or memory.
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 effects cancer treatment can have on your child.
Children with the inherited form of retinoblastoma have an increased risk of developing second cancers. Children, especially those younger than 12 years, who have been treated for retinoblastoma with radiation therapy 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. Close follow-up is needed for 2 years or more to check the other eye and to check for signs of recurrence in the area around the eye.
Radiation therapy
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. 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 and stereotaxic radiation therapy.
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.
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). Thermotherapy may be used alone for small tumors or combined with chemotherapy for larger tumors.
Chemotherapy
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them 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 cerebrospinal fluid, 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, or thermotherapy).
New types of treatment are being tested in clinical trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site.
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, or thermotherapy).
Ophthalmic arterial infusion therapy
Ophthalmic arterial infusion therapy is a type of regional chemotherapy used to deliver anticancer drugs directly to the eye. A catheter is put into an artery that leads to the eye and the anticancer drug is given through the catheter. During this treatment, a small balloon may be inserted into the artery to block it and keep most of the anticancer drug trapped near the tumor.
High-dose chemotherapy with stem cell rescue
High-dose chemotherapy with stem cell rescue is a way 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.
Biologic therapy
Biologic therapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Clinical trials for retinoblastoma are studying a biologic therapy called gene therapy.This is a treatment that changes a gene to improve the body's ability to fight the disease.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options for Retinoblastoma
A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.
Intraocular Retinoblastoma
If the cancer is in one eye and the tumor is large, treatment is usually enucleation. Chemotherapy may be given to shrink the tumor before surgery or after surgery to lower the risk that the cancer will spread to other parts of the body.
If the cancer is in one eye and it is expected that vision can be saved, treatment may include one or more of the following:
External-beam radiation therapy or plaque radiotherapy.
Cryotherapy.
Thermotherapy.
Chemotherapy (chemoreduction).
A clinical trial of chemotherapy using more than one anticancer drug.
A clinical trial of intensity-modulated radiation therapy, stereotactic radiotherapy, or proton beam radiation therapy.
If the cancer is in both eyes, treatment may include one or more of the following:
Chemotherapy (chemoreduction) followed by local treatment such as cryotherapy, thermotherapy, or plaque radiotherapy. This may be done if there is a chance to save vision in both eyes.
Enucleation of one or both eyes, when vision cannot be saved.
A clinical trial of subtenon chemotherapy combined with systemic chemotherapy and local treatment.
A clinical trial of new combinations of chemotherapy and other treatments to the eye.
A clinical trial of higher doses of systemic chemotherapy combined with regional chemotherapy, local treatment, and lower doses of radiation therapy to the eye.
A clinical trial of gene therapy.
A clinical trial of ophthalmic arterial infusion therapy.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with intraocular retinoblastoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Extraocular Retinoblastoma
There is no standard treatment for extraocular retinoblastoma. In the past, radiation therapy and intrathecal chemotherapy have been used. If treatment is enucleation, chemotherapy to shrink the tumor may be given before surgery. Treatment may be a clinical trial of chemotherapy followed by high-dose chemotherapy and stem cell rescue with radiation therapy.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with extraocular retinoblastoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Recurrent Retinoblastoma
If the cancer is small and in the eye only, treatment is usually local therapy (enucleation, plaque radiotherapy, cryotherapy, or thermotherapy). If the cancer is large and in the eye only, treatment is usually chemotherapy folllowed by radiation therapy.
If the cancer comes back outside of the eye, treatment will depend on many things and may be within a clinical trial of chemotherapy combined with high-dose chemotherapy and stem cell rescue with radiation therapy.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent retinoblastoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Glossary Terms
anesthesia (A-nes-THEE-zhuh)
A loss of feeling or awareness caused by drugs or other substances. Anesthesia keeps patients from feeling pain during surgery or other procedures. Local anesthesia is a loss of feeling in one small area of the body. Regional anesthesia is a loss of feeling in a part of the body, such as an arm or leg. General anesthesia is a loss of feeling and a complete loss of awareness that feels like a very deep sleep.
biopsy (BY-op-see)
The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.
cell (sel)
The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.
contrast material
A dye or other substance that helps show abnormal areas inside the body. It is given by injection into a vein, by enema, or by mouth. Contrast material may be used with x-rays, CT scans, MRI, or other imaging tests.
CT scan
A series of detailed pictures of areas inside the body taken from different angles. The pictures are created by a computer linked to an x-ray machine. Also called CAT scan, computed tomography scan, computerized axial tomography scan, and computerized tomography.
diagnosis (DY-ug-NOH-sis)
The process of identifying a disease, such as cancer, from its signs and symptoms.
dilate (DYE-late)
To widen or enlarge an opening or hollow structure beyond its usual size, such as the pupil of the eye or a blood vessel.
family history (FAM-ih-lee HIH-stuh-ree)
A record of the relationships among family members along with their medical histories. This includes current and past illnesses. A family history may show a pattern of certain diseases in a family. Also called family medical history.
follow-up
Monitoring a person's health over time after treatment. This includes keeping track of the health of people who participate in a clinical study or clinical trial for a period of time, both during the study and after the study ends.
gene (JEEN)
The functional and physical unit of heredity passed from parent to offspring. Genes are pieces of DNA, and most genes contain the information for making a specific protein.
genetic counseling (jeh-NEH-tik KOWN-suh-ling)
A communication process between a specially trained health professional and a person concerned about the genetic risk of disease. The person's family and personal medical history may be discussed, and counseling may lead to genetic testing.
glaucoma (glaw-KOH-muh)
A condition in which there is a build-up of fluid in the eye, which presses on the retina and the optic nerve. The retina is the layer of nerve tissue inside the eye that senses light and sends images along the optic nerve to the brain. Glaucoma can damage the optic nerve and cause loss of vision or blindness.
hereditary (huh-REH-dih-tayr-ee)
Transmitted from parent to child by information contained in the genes.
inherited (in-HAYR-it-ed)
Transmitted through genes that have been passed from parents to their offspring (children).
injection
Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."
ionizing radiation (I-uh-NYZ-ing RAY-dee-AY-shun)
A type of radiation made (or given off ) by x-ray procedures, radioactive substances, rays that enter the Earth's atmosphere from outer space, and other sources. At high doses, ionizing radiation increases chemical activity inside cells and can lead to health risks, including cancer.
MRI
A procedure in which radio waves and a powerful magnet linked to a computer are used to create detailed pictures of areas inside the body. These pictures can show the difference between normal and diseased tissue. MRI makes better images of organs and soft tissue than other scanning techniques, such as computed tomography (CT) or x-ray. MRI is especially useful for imaging the brain, the spine, the soft tissue of joints, and the inside of bones. Also called magnetic resonance imaging, NMRI, and nuclear magnetic resonance imaging.
mutation (myoo-TAY-shun)
Any change in the DNA of a cell. Mutations may be caused by mistakes during cell division, or they may be caused by exposure to DNA-damaging agents in the environment. Mutations can be harmful, beneficial, or have no effect. If they occur in cells that make eggs or sperm, they can be inherited; if mutations occur in other types of cells, they are not inherited. Certain mutations may lead to cancer or other diseases.
nerve
A bundle of fibers that receives and sends messages between the body and the brain. The messages are sent by chemical and electrical changes in the cells that make up the nerves.
ophthalmologist (OF-thul-MOL-oh-jist)
A doctor who specializes in diagnosing and treating eye problems, including injury and disease.
optic nerve
The nerve that carries messages from the retina to the brain.
organ
A part of the body that performs a specific function. For example, the heart is an organ.
physical examination (FIH-zih-kul eg-ZA-mih-NAY-shun)
An exam of the body to check for general signs of disease.
pineal region tumor (PIN-ee-al...)
A type of brain tumor that occurs in or around the pineal gland, a tiny organ near the center of the brain.
prognosis (prog-NO-sis)
The likely outcome or course of a disease; the chance of recovery or recurrence.
pupil (PYOO-pil)
The round opening in the center of the iris (the colored tissue that makes the "eye color" at the front of the eye). The pupil changes size to let light into the eye. It gets smaller in bright light and larger as the amount of light decreases.
retina (RET-ih-nuh)
The light-sensitive layers of nerve tissue at the back of the eye that receive images and sends them as electric signals through the optic nerve to the brain.
retinoblastoma (REH-tih-noh-blas-TOH-muh)
Cancer that forms in the tissues of the retina (the light-sensitive layers of nerve tissue at the back of the eye). Retinoblastoma usually occurs in children younger than 5 years. It may be hereditary or nonhereditary (sporadic).
screening (SKREEN-ing)
Checking for disease when there are no symptoms. Since screening may find diseases at an early stage, there may be a better chance of curing the disease. Examples of cancer screening tests are the mammogram (breast), colonoscopy (colon), Pap smear (cervix), and PSA blood level and digital rectal exam (prostate). Screening can also include checking for a person’s risk of developing an inherited disease by doing a genetic test.
sonogram (SON-o-gram)
A computer picture of areas inside the body created by bouncing high-energy sound waves (ultrasound) off internal tissues or organs. Also called ultrasonogram.
stage
The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.
symptom
An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.
tissue (TISH-oo)
A group or layer of cells that work together to perform a specific function.
tumor (TOO-mer)
An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancer), or malignant (cancer). Also called neoplasm.
ultrasound (UL-truh-SOWND)
A procedure in which high-energy sound waves are bounced off internal tissues or organs and make echoes. The echo patterns are shown on the screen of an ultrasound machine, forming a picture of body tissues called a sonogram. Also called ultrasonography.
vein (vayn)
A blood vessel that carries blood to the heart from tissues and organs in the body.
x-ray
A type of high-energy radiation. In low doses, x-rays are used to diagnose diseases by making pictures of the inside of the body. In high doses, x-rays are used to treat cancer.
anesthesia (A-nes-THEE-zhuh)
A loss of feeling or awareness caused by drugs or other substances. Anesthesia keeps patients from feeling pain during surgery or other procedures. Local anesthesia is a loss of feeling in one small area of the body. Regional anesthesia is a loss of feeling in a part of the body, such as an arm or leg. General anesthesia is a loss of feeling and a complete loss of awareness that feels like a very deep sleep.
blood