21st Century Pediatric Cancer Sourcebook: Neuroblastoma, plus Opsoclonus/Myoclonus Syndrome - Clinical Data for Patients, Families, and Physicians
Edition 1.0 - March 2011
National Cancer Institute
Smashwords Edition
Copyright 2011 Progressive Management
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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/
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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
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Last Modified: 01/20/2011
Neuroblastoma is a disease in which malignant (cancer) cells form in nerve tissue of the adrenal gland, neck, chest, or spinal cord.
Neuroblastoma is sometimes caused by a gene mutation passed from the parent to the child.
Possible signs of neuroblastoma include bone pain and a lump in the abdomen, neck, or chest.
Tests that examine many different body tissues and fluids are used to detect (find) and diagnose neuroblastoma.
Certain factors affect prognosis (chance of recovery) and treatment options.
Neuroblastoma is a disease in which malignant (cancer) cells form in nerve tissue of the adrenal gland, neck, chest, or spinal cord.
Neuroblastoma often begins in the nerve tissue of the adrenal glands. There are two adrenal glands, one on top of each kidney in the back of the upper abdomen. The adrenal glands produce important hormones that help control heart rate, blood pressure, blood sugar, and the way the body reacts to stress. Neuroblastoma may also begin in the abdomen, in the chest, in nerve tissue near the spine in the neck, or in the spinal cord.
Anatomy of the female urinary system showing the kidneys, adrenal glands, ureters, bladder, and urethra. Urine is made in the renal tubules and collects in the renal pelvis of each kidney. The urine flows from the kidneys through the ureters to the bladder. The urine is stored in the bladder until it leaves the body through the urethra.
Neuroblastoma most often begins during early childhood, usually in children younger than 5 years. It sometimes forms before birth but is usually found later, when the tumor begins to grow and cause symptoms. In rare cases, neuroblastoma may be found before birth by fetal ultrasound.
By the time neuroblastoma is diagnosed, the cancer has usually metastasized (spread), most often to the lymph nodes, bones, bone marrow, liver, and skin.
Neuroblastoma is sometimes caused by a gene mutation passed from the parent to the child.
Neuroblastoma is sometimes inherited (passed from the parent to the child). It usually occurs at a younger age than neuroblastoma that is not inherited. There also may be more than one tumor in the adrenal medulla in inherited neuroblastoma.
Possible signs of neuroblastoma include bone pain and a lump in the abdomen, neck, or chest.
The most common symptoms of neuroblastoma are caused by the tumor pressing on nearby tissues as it grows or by cancer spreading to the bone. These and other symptoms may be caused by neuroblastoma. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:
Lump in the abdomen, neck, or chest.
Bulging eyes.
Dark circles around the eyes ("black eyes").
Bone pain.
Swollen stomach and trouble breathing in infants.
Painless, bluish lumps under the skin in infants.
Weakness or paralysis (loss of ability to move a body part).
Less common signs of neuroblastoma include the following:
Fever.
Shortness of breath.
Feeling tired.
Easy bruising or bleeding.
Petechiae (flat, pinpoint spots under the skin caused by bleeding).
High blood pressure.
Severe watery diarrhea.
Jerky muscle movements.
Uncontrolled eye movement.
Swelling of the legs, ankles, feet, or scrotum.
Tests that examine many different body tissues and fluids are used to detect (find) and diagnose neuroblastoma.
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.
Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of certain substances. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. A higher than normal amount of the substances homovanillic acid (HMA) and vanillyl mandelic acid (VMA) may be a sign of neuroblastoma.
Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. A higher than normal amount of the hormones dopamine and norepinephrine may be a sign of neuroblastoma.
Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.
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.
Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer.
X-ray: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, 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.
Neurological exam: A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.
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. The picture can be printed to be looked at later.
Immunohistochemistry study: A laboratory test in which a substance such as an antibody, dye, or radioisotope is added to a sample of cancer tissue to test for certain antigens. This type of study is used to tell the difference between different types of cancer.
MIBG (metaiodobenzylguanidine) scan: A procedure used to find neuroendocrine tumors, such as neuroblastomas and pheochromocytomas. A small amount of radioactive material called MIBG is injected into a vein and travels through the bloodstream. Neuroendocrine tumor cells take up the radioactive material and are detected by a scanner. Scans may be taken over 1-3 days. An iodine solution may be given before or during the test to prevent the thyroid gland from absorbing too much of the MIBG.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
Age of the child when diagnosed.
Stage of the cancer.
Where the tumor is in the body.
Tumor histology (the shape, function, and structure of the tumor cells).
Whether there is cancer in the lymph nodes.
Prognosis and treatment decisions for neuroblastoma are also affected by tumor biology, which includes:
The patterns of the tumor cells.
How different the tumor cells are from normal cells.
How fast the tumor cells are growing.
The tumor biology is said to be favorable or unfavorable, depending on these factors. A favorable tumor biology means there is a better chance of recovery.
In some infants, neuroblastoma may disappear without treatment. The infant is closely watched for symptoms of neuroblastoma. If symptoms occur, treatment may be needed.
Stages of Neuroblastoma
Key Points for This Section
After neuroblastoma has been diagnosed, tests are done to find out if cancer has spread from where it started to other parts of the body.
There are three ways that cancer spreads in the body.
The following stages are used for neuroblastoma:
Stage 1
Stage 2
Stage 3
Stage 4
Treatment of neuroblastoma is based on risk groups.
After neuroblastoma has been diagnosed, tests are done to find out if cancer has spread from where it started to other parts of the body.
The process used to find out the extent or spread of cancer is called staging. The information gathered from the staging process helps determine the stage of the disease. For neuroblastoma, stage is one of the factors used to plan treatment. The following tests and procedures may be used to determine the stage:
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.
Lymph node biopsy: The removal of all or part of a lymph node. A pathologist views the tissue under a microscope to look for cancer cells. One of the following types of biopsies may be done:
Excisional biopsy: The removal of an entire lymph node.
Incisional biopsy: The removal of part of a lymph node.
Core biopsy: The removal of tissue from a lymph node using a wide needle.
Fine-needle aspiration (FNA) biopsy: The removal of tissue or fluid from a lymph node using a thin needle.
CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, 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. This procedure is also called nuclear magnetic resonance imaging (NMRI).
X-rays of the chest, bones, and abdomen: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
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. The picture can be printed to be looked at later.
Radionuclide scan: A procedure to find areas in the body where cells, such as cancer cells, are dividing rapidly. A very small amount of radioactive material is swallowed or injected into a vein and travels through the bloodstream. The radioactive material collects in the bones or other tissues and is detected by a radiation -measuring device.
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.
The following stages are used for neuroblastoma:
Stage 1
In stage 1, the tumor is in only one area and all of the tumor that can be seen is completely removed during surgery.
Stage 2
Stage 2 is divided into stage 2A and 2B.
Stage 2A: The tumor is in only one area and all of the tumor that can be seen cannot be completely removed during surgery.
Stage 2B: The tumor is in only one area and all of the tumor that can be seen may be completely removed during surgery. Cancer cells are found in the lymph nodes near the tumor.
Stage 3
In stage 3, one of the following is true:
the tumor cannot be completely removed during surgery and has spread from one side of the body to the other side and may also have spread to nearby lymph nodes; or
the tumor is in only one area, on one side of the body, but has spread to lymph nodes on the other side of the body; or
the tumor is in the middle of the body and has spread to tissues or lymph nodes on both sides of the body, and the tumor cannot be removed by surgery.
Stage 4
Stage 4 is divided into stage 4 and stage 4S.
In stage 4, the tumor has spread to distant lymph nodes, the skin, or other parts of the body.
In stage 4S, the following are true:
the child is younger than 1 year; and
the cancer has spread to the skin, liver, and/or bone marrow; and
the tumor is in only one area and all of the tumor that can be seen may be completely removed during surgery; and/or
cancer cells may be found in the lymph nodes near the tumor.
Treatment of neuroblastoma is based on risk groups.
For many types of cancer, stages are used to plan treatment. For neuroblastoma, treatment depends on risk groups. The stage of neuroblastoma is one factor used to determine risk group. Other factors are the age of the child, tumor histology, and tumor biology.
There are 3 risk groups: low risk, intermediate risk, and high risk.
Low-risk and intermediate-risk neuroblastoma have a good chance of being cured.
High-risk neuroblastoma may be difficult to cure.
Progressive/Recurrent Neuroblastoma
Progressive neuroblastoma is cancer that has progressed (continued to grow) during treatment. Recurrent neuroblastoma is cancer that has recurred (come back) after it has been treated. The cancer may come back in the same place or in other parts of the body.
Treatment Option Overview
Key Points for This Section
There are different types of treatment for patients with neuroblastoma.
Children with neuroblastoma should have their treatment planned by a team of doctors with expertise in treating childhood cancer.
Children who are treated for neuroblastoma may be at higher risk for second cancers.
Five types of standard treatment are used:
Surgery
Radiation therapy
Chemotherapy
Biologic therapy
Watchful waiting
New types of treatment are being tested in clinical trials.
Targeted therapy
High-dose chemotherapy and radiation therapy with stem cell transplant
Other drug 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 neuroblastoma.
Different types of treatment are available for patients with neuroblastoma. 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 neuroblastoma should have their treatment planned by a team of doctors with expertise in treating childhood cancer.
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 neuroblastoma and who specialize in certain areas of medicine. These may include the following specialists:
Medical oncologist.
Hematologist.
Pediatric surgeon.
Radiation oncologist.
Endocrinologist.
Neurologist.
Neuropathologist.
Neuroradiologist.
Pediatric nurse specialist.
Social worker.
Rehabilitation specialist.
Psychologist.
Children who are treated for neuroblastoma may be at higher risk for second cancers.
Some cancer treatments cause side effects that continue or appear 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.
Second cancers (new types of cancer).
Some late effects may be treated or controlled. It is important that parents of children who are treated for neuroblastoma talk with their doctors about the possible late effects caused by some treatments.
Five types of standard treatment are used:
Surgery
Surgery is usually used to treat neuroblastoma. Depending on where the tumor is and whether it has spread, as much of the tumor as is safely possible will be removed. If the tumor cannot be removed, a biopsy may be done instead.
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, 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.
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, 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.
The use of two or more anticancer drugs is called combination chemotherapy.
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.
Watchful waiting
Watchful waiting is closely monitoring a patient's condition without giving any treatment until symptoms appear or change.
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.
Targeted therapy
Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy is one type of targeted therapy being studied in the treatment of neuroblastoma.
Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to deliver drugs, toxins, or radioactive material directly to cancer cells.
High-dose chemotherapy and radiation therapy with stem cell transplant
High-dose chemotherapy and radiation therapy with stem cell transplant is a way of giving high doses of chemotherapy and radiation therapy 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 chemotherapy and radiation therapy are 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.
Other drug therapy
13-cis retinoic acid is a vitamin-like drug that slows the cancer's ability to make more cancer cells and changes how these cells look and act.
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.
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 Neuroblastoma
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.
Low-Risk Neuroblastoma
Treatment of low-risk neuroblastoma may include the following:
Watchful waiting alone for certain infants.
Surgery followed by watchful waiting.
Surgery followed by combination chemotherapy, when serious health problems occur.
Radiation therapy to treat tumors that are causing serious health problems and do not respond quickly to chemotherapy.
A clinical trial of surgery followed by chemotherapy. Radiation therapy is given to treat tumors that are causing serious health problems and do not respond quickly to surgery and chemotherapy.
Intermediate-Risk Neuroblastoma
Treatment of intermediate-risk neuroblastoma may include the following:
Surgery alone.
Surgery followed by chemotherapy. Sometimes a second surgery is needed.
Surgery followed by chemotherapy. Radiation therapy may given to treat tumors that are causing serious health problems and do not respond quickly to surgery and chemotherapy.
Chemotherapy followed by surgery.
Radiation therapy to treat tumors that are causing serious problems and do not respond quickly to chemotherapy.
A clinical trial of lower doses of chemotherapy.
High-Risk Neuroblastoma
Treatment of high-risk neuroblastoma may include the following:
Combination chemotherapy followed by surgery to remove as much of the tumor as possible, followed by high-dose chemotherapy and stem cell transplant.
Radiation therapy to the tumor site and, sometimes, if needed, to other parts of the body with cancer.
13-cis retinoic acid after recovery from previous treatment.
A clinical trial of new regimens of chemotherapy and radiation therapy with stem cell transplant.
A clinical trial of monoclonal antibody therapy, biologic therapy, and 13-cis retinoic acid after chemotherapy.
A clinical trial of radiation therapy with radioactive iodine and chemotherapy before stem cell transplant.
Progressive/Recurrent Neuroblastoma
Patients First Treated for Low-Risk Neuroblastoma
Treatment for recurrent neuroblastoma that is found in one place in the body may include the following:
Surgery followed by watchful waiting or chemotherapy.
High-dose chemotherapy, stem cell transplant, and 13-cis retinoic acid.
A clinical trial of surgery and if needed, chemotherapy and another surgery.
Treatment for recurrent neuroblastoma that has spread to other parts of the body may include the following:
Watchful waiting.
Surgery followed by chemotherapy.
High-dose chemotherapy, stem cell transplant, and 13-cis retinoic acid.
A clinical trial of a new treatment.
Patients First Treated for Intermediate-Risk Neuroblastoma
Treatment for recurrent neuroblastoma that is found in one place in the body may include the following:
Surgery, with or without chemotherapy.
A clinical trial of surgery and more courses of chemotherapy.
A clinical trial of a new treatment.
For recurrent neuroblastoma that has spread to other parts of the body, treatment is usually high-dose chemotherapy, stem cell transplant, and 13-cis retinoic acid.
Patients First Treated for High-Risk Neuroblastoma
There is no standard treatment for recurrent neuroblastoma in patients first treated for high-risk neuroblastoma.
Glossary Terms
abdomen (AB-doh-men)
The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.
adrenal gland (uh-DREE-nul...)
A small gland that makes steroid hormones, adrenaline, and noradrenaline. These hormones help control heart rate, blood pressure, and other important body functions. There are two adrenal glands, one on top of each kidney. Also called suprarenal gland.
adrenal medulla (uh-DREE-nul meh-DOO-luh)
The inner part of the adrenal gland (a small organ on top of each kidney). The adrenal medulla makes chemicals such as epinephrine (adrenaline) and norepinephrine (noradrenaline) which are involved in sending nerve signals.
antibody (AN-tee-BAH-dee)
A protein made by plasma cells (a type of white blood cell) in response to an antigen (a substance that causes the body to make a specific immune response). Each antibody can bind to only one specific antigen. The purpose of this binding is to help destroy the antigen. Some antibodies destroy antigens directly. Others make it easier for white blood cells to destroy the antigen.
antigen (AN-tih-jen)
Any substance that causes the body to make a specific immune response.
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.
blood
A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.
blood chemistry study (blud KEH-mih-stree STUH-dee)
A procedure in which a sample of blood is examined to measure the amounts of certain substances made in the body. An abnormal amount of a substance can be a sign of disease in the organ or tissue that produces it.
blood pressure (blud PREH-sher)
The force of circulating blood on the walls of the arteries. Blood pressure is taken using two measurements: systolic (measured when the heart beats, when blood pressure is at its highest) and diastolic (measured between heart beats, when blood pressure is at its lowest). Blood pressure is written with the systolic blood pressure first, followed by the diastolic blood pressure (for example 120/80).
bone marrow (bone MAYR-oh)
The soft, sponge-like tissue in the center of most bones. It produces white blood cells, red blood cells, and platelets.
bone marrow aspiration (bone MAYR-oh AS-pih-RAY-shun)
A procedure in which a small sample of bone marrow is removed, usually from the hip bone, breastbone, or thigh bone. A small area of skin and the surface of the bone underneath are numbed with an anesthetic. Then, a special wide needle is pushed into the bone. A sample of liquid bone marrow is removed with a syringe attached to the needle. The bone marrow is sent to a laboratory to be looked at under a microscope. This procedure may be done at the same time as a bone marrow biopsy.
bone marrow biopsy (bone MAYR-oh BY-op-see)
A procedure in which a small sample of bone with bone marrow inside it is removed, usually from the hip bone. A small area of skin and the surface of the bone underneath are numbed with an anesthetic. Then, a special, wide needle is pushed into the bone and rotated to remove a sample of bone with the bone marrow inside it. The sample is sent to a laboratory to be looked at under a microscope. This procedure may be done at the same time as a bone marrow aspiration.
cell (sel)
The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.
chromosome (KROH-muh-some)
Part of a cell that contains genetic information. Except for sperm and eggs, all human cells contain 46 chromosomes.
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.
cytogenetics (SY-toh-jeh-NEH-tix)
The study of chromosomes and chromosomal abnormalities.
diagnosis (DY-ug-NOH-sis)
The process of identifying a disease, such as cancer, from its signs and symptoms.
diarrhea
Frequent and watery bowel movements.
fetus (FEET-us)
The developing offspring from 7 to 8 weeks after conception until birth.
fever (FEE-ver)
An increase in body temperature above normal (98.6 degrees F), usually caused by disease.
high blood pressure (hy blud PREH-sher)
A blood pressure of 140/90 or higher. High blood pressure usually has no symptoms. It can harm the arteries and cause an increase in the risk of stroke, heart attack, kidney failure, and blindness. Also called hypertension.
histology (his-TAH-loh-jee)
The study of tissues and cells under a microscope.
hormone (HOR-mone)
One of many chemicals made by glands in the body. Hormones circulate in the bloodstream and control the actions of certain cells or organs. Some hormones can also be made in the laboratory.
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."
iodine (I-oh-dine)
An element that is necessary for the body to make thyroid hormone. It is found in shellfish and iodized salt.
kidney (KID-nee)
One of a pair of organs in the abdomen. Kidneys remove waste from the blood (as urine), produce erythropoietin (a substance that stimulates red blood cell production), and play a role in blood pressure regulation.
liver (LIH-ver)
A large organ located in the upper abdomen. The liver cleanses the blood and aids in digestion by secreting bile.
lymph node (limf node)
A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph nodes filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph gland.
metastasize (meh-TAS-tuh-size)
To spread from one part of the body to another. When cancer cells metastasize and form secondary tumors, the cells in the metastatic tumor are like those in the original (primary) tumor.
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.
neuroblastoma (NOOR-oh-blas-TOH-muh)
Cancer that arises in immature nerve cells and affects mostly infants and children.
neuroendocrine (NOOR-oh-EN-doh-krin)
Having to do with the interactions between the nervous system and the endocrine system. Neuroendocrine describes certain cells that release hormones into the blood in response to stimulation of the nervous system.
neurological exam (NOOR-oh-LAH-jih-kul eg-ZAM)
A series of questions and tests to check brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, ability to walk, and how well the muscles, sensory systems, and deep tendon reflexes work.
norepinephrine (NOR-eh-pih-NEH-frin)
A chemical made by some nerve cells and in the adrenal gland. It can act as both a neurotransmitter (a chemical messenger used by nerve cells) and a hormone (a chemical that travels in the blood and controls the actions of other cells or organs). Norepinephrine is released from the adrenal gland in response to stress and low blood pressure. Also called noradrenaline.
organ
A part of the body that performs a specific function. For example, the heart is an organ.
paralysis (puh-RA-lih-sis)
Loss of ability to move all or part of the body.
pathologist (puh-THAH-loh-jist)