Currently one in every 330 children in the United States develops cancer before the age of nineteen.

According to the research from 1996-2000 New York State Registry findings, the average number of cases of childhood cancer in New York State is 857.4 cases. Of these cases, the average number of children with pediatric cancer in Columbia County was 4.0 and 2.2 in Greene County.

The cancers that attack children are different from the principal cancers of adults.

Children frequently have a more advanced stage of cancer when they are first diagnosed than adults. Only about 20% of adults with cancer show evidence that the disease has spread at the time of diagnosis, yet 80% of the children who are diagnosed with cancer show disease that has already spread to distant sites in the body.

Most adult cancers result from lifestyle factors, such as smoking, diet, occupation, and other prolonged exposure to cancer-causing agents. The causes of most childhood cancers are not yet known.

Adult cancers are primarily those of the lung, colon, breast, prostate and pancreas. Childhood cancers are mostly those of the white blood cells [leukemia] (23%), brain (18%), the lymphatic system (15%), bone (6%) and tumors of the muscles (7%), nervous system (5%) and kidneys (4%). Each of these behaves differently, but all are characterized by an uncontrolled proliferation of abnormal cells.

The majority of adult cancer sufferers are treated in their local community by their family physician, consulting surgeons, medical oncologists or other cancer specialists. Family physicians or pediatricians rarely treat children with cancer. A child with cancer must be diagnosed precisely and treated by teams of clinical and laboratory scientists who have expertise in the management of children with cancer. Such teams are found only in major children's hospitals, university medical centers, and cancer centers.

Progress in the development of effective new treatments and cures for children with cancer has been spectacular during the past three decades, but progress is beginning to plateau. Most children now can be cured if they are treated at childhood cancer treatment and research centers by teams of experts in childhood cancer. But many types of childhood cancer have not yet yielded to research progress.

RISK FACTORS

The causes of childhood cancers are largely unknown. A few conditions, such as Down Syndrome, other specific chromosomal and genetic abnormalities, and ionizing radiation exposures, explain a small percentage of cases.

Environmental causes of childhood cancer have long been suspected by many scientists, but have been difficult to pin down. This is partly because cancer in children is rare, and partly because it is so difficult to identify past exposure levels in children, particularly during potentially important periods such as pregnancy or even prior to conception.

In addition, each of the distinctive types of childhood cancers develops differently — with a potentially wide variety of causes and a unique clinical course in terms of age, race, gender, and many other factors.

PRINCIPAL CANCERS OF CHILDREN

Types of Childhood Cancer

• Bone cancers: The bones may be the site to which other cancers spread, but some types originate in the skeleton. The most common bone cancer is osteogenic sarcoma. Bone cancer in children occurs most often during adolescent growth spurts, and 85% of those teenagers have tumors on their legs or arms, half of them around the knee. Ewing’s sarcoma differs from osteosarcoma in that it affects the bone shaft, and tends to be found in bones other than the long bones of the arm and the leg, such as the ribs. During the period from 1950 to 1980, there was a 50% reduction in deaths in children due to bone sarcoma
• Brain tumors: Tumors of the brain and spinal cord are the most common types of solid tumors in children. Some tumors are benign, and some children can be cured by surgery. But there has been less dramatic progress in treating brain cancer tumors than most other childhood malignancies because they are hard to diagnose and treat. Twenty percent of all primary brain tumors arise in children younger than age 15, somewhat more in boys than girls. There is a peak in incidence between the ages of 5 and 10
• Leukemias: Leukemia is cancer of the tissues of the body which make the blood cell bone marrow. When leukemia strikes, the body makes an abundance of abnormal white cells that do not perform their proper functions. They invade the marrow and crowd out the normal healthy blood cells, making the patient susceptible to infection and bruising. The most common form of this disease in young children is Acute Lymphoblastic Leukemia (ALL) which medical science has made remarkable progress in fighting
• Lymphomas: Lymphoma is cancer which arises in the lymph system, the body’s circulatory network for filtering out impurities. There are two broad varieties, Hodgkin’s disease, and Non-Hodgkin’s lymphoma. Non-Hodgkin’s lymphoma is more common in children than Hodgkin’s disease. It can arise in the tonsils, thymus, bone, small intestine, spleen, or in lymph glands anywhere in the body. The disease can spread to the central nervous system and the bone marrow. Treatments have been developed that can cure many children, and other promising treatments are coming along
• Neuroblastoma: Found only in children, neuroblastoma arises in the adrenal glands, located in the abdominal area near the kidneys, and along the sympathetic nerve chain in the chest and abdomen. It attacks very young children. One-fourth of those affected show initial symptoms during the first year of life. Neuroblastoma spreads quickly, and often is discovered only after the disease is widespread. Early stages are curable by surgery alone. Researchers have discovered new treatments for advanced stages which are increasingly effective
• Rhabdomyosarcoma: The most common soft tissue sarcoma in children, this extremely malignant neoplasm originates in skeletal muscle. Although it can occur in any muscle tissue, it is generally found in the head and neck area (including the eye socket), the genito-urinary tract, or in the extremities. Although rhabdomyosarcoma tends to grow and spread very rapidly, fortunately its symptoms are quite obvious compared to other forms of childhood cancer. Overall prognosis is improving, with the development of improved chemotherapies
• Wilms’ tumor: This rapidly-developing tumor of the kidney most often appears in children, usually between the ages of two and four, and is very different from adult kidney cancers. The disease often metastasizes to the lungs and, in the past, the mortality from this cancer was extremely high. However, newer therapies have been very effective in controlling it, combining surgery, radiation therapy and chemotherapy, and cure rates have risen sharply
• Retinoblastoma: A malignant eye tumor that occurs in young children and shows a hereditary pattern accounts for only 2% of the childhood cancer cases. However, it received a great deal of attention when it was the first cancer for which researchers were able to identify the responsible gene.
• Other: There are many other childhood cancers that are even more rare, including germ cell tumors, thyroid cancer, malignant melanoma, testicular tumors (usually during puberty,) and primary cancers in the kidney, liver, and lung. Back to Top

TREATMENT

To plan the best treatment, the doctor and treatment team will look at your child's general health, type of cancer, stage of the disease, age, and many other factors. Based on this information, the doctor will prepare a treatment plan that outlines the exact type of treatment, how often your child will receive treatment, and how long it will last.

Each child with cancer has a treatment plan that is chosen just for that child. Even children with the same type of cancer may receive different treatments. Depending on how your child responds to treatment, the doctor may decide to change the treatment plan or choose another plan.

Before treatment begins, your child's doctor will discuss the treatment plan with you, including the benefits, risks, and side effects. Then you and the treatment team will need to talk with your child about the treatment. After the doctor fully explains the treatment and answers your questions, you will be asked to give your written consent to go ahead with treatment. Depending on your child's age and hospital policy, your child may also be asked to give consent before treatment.

What Are the Different Types of Cancer Treatment?

The types of treatment used most often to treat cancer are surgery, chemotherapy, radiation therapy, immunotherapy, and bone marrow or peripheral blood stem cell transplantation. Doctors use these treatments to destroy cancer cells. Depending on the type of cancer, children may have one kind of treatment or a combination of treatments. Most children receive a combination of treatments, called combination therapy.

Treatments for cancer often cause unwanted or unpleasant side effects such as nausea, hair loss, and diarrhea. Side effects occur because cancer treatment that kills cancer cells can hurt some normal cells, too. As your child begins treatment, you may want to keep the following in mind

• The kinds of side effects and how bad they will be depend on the kind of drug, the dosage, and the way your child's body reacts.
• The doctor plans treatment so that your child has as few side effects as possible.
• The doctor and treatment team have ways to lessen your child's side effects.
• Talk with them about things that can be done before, during, and after treatment to make your child comfortable.
• Lowering the treatment dosage slightly to eliminate unpleasant side effects usually will not make the treatment less able to destroy cancer cells or hurt your child's chances of recovery.
• Most side effects go away soon after treatment ends.
• Remember that not every child gets every side effect, and some children get few, if any. Also, how serious the side effects are varies from child to child, even among children who are receiving the same treatment. The doctor or treatment team can tell you what, if any, side effects your child is likely to have and how to handle them. If you know what side effects can occur, you can recognize them early.

Surgery - For many solid tumors, surgery is an essential part of the treatment. Surgery is a local therapy to remove the tumor. Tissue around the tumor and nearby lymph nodes may also be removed during the operation. Sometimes radiation or chemotherapy is used first to shrink the tumor before it is removed. Shrinking the tumor makes the surgery easier.

Chemotherapy - Chemotherapy is the use of "anticancer drugs" to treat cancer. Chemotherapy is systemic therapy, which means that the drugs flow through the bloodstream to nearly every part of the body to kill cancer cells wherever they may be. Because some anticancer drugs work better together than alone, chemotherapy may consist of more than one drug. This approach is called combination chemotherapy.

Depending on the type of cancer your child has, and which drugs are used, chemotherapy may be given in one or more of these ways:

• By mouth (oral medication) - The drugs are swallowed in liquid or pill form. If your child has trouble swallowing pills, you can break the pills into smaller pieces, or you may crush and mix them with applesauce, jam, pudding, or other food that your child likes.
• Intravenously (IV) - The drugs are injected by needle into a vein or into an IV line.
• Intramuscular injection (IM) - The drugs are injected by needle into the muscle. Your child will know it as a "shot."
• Subcutaneous injection (SC) - The drugs are injected by needle just below the skin. Your child will also know this as a "shot."
• Intrathecal injection (IT) - The drugs are injected by needle into the spinal fluid.

Although your child may be able to receive chemotherapy treatments at home, he or she will probably need to go to the hospital or doctor's office to receive IV drugs or injections. Depending on the medicine, your child may need to stay in the hospital, perhaps overnight or longer.

Radiation therapy - Radiation therapy is treatment with high-energy rays to damage or destroy cancer cells. Like surgery, radiation therapy is a local therapy. The rays are aimed at the part of the body that has cancer, and the treatment destroys cancer cells in the treated area. Doctors may use radiation therapy before surgery to shrink a tumor. After surgery, radiation therapy may be used to stop the growth of cancer cells that remain.

How Does Radiation Therapy Work?

All human body cells, including cancer cells, contain a substance called DNA. DNA tells the cells how to form and grow. In radiation therapy, the radiation harms the DNA inside the cancer cells, causing them to die before more cells are made. Tumors will shrink as the cancer cells die.

Radiation therapy does not cause pain. It is much like having a regular x-ray taken, except that your child needs to hold still longer. Because some young children are often unable to be still, the doctor may give a young child medicine to help him or her relax or to put him or her to sleep. The parts of your child's body that are not being treated will be covered by special shields made of lead to protect those body parts from the radiation.

Immunotherapy

The immune system - the body's system for defending itself - knows when substances that should not be there, such as bacteria and viruses, are in the body and then attacks them. The system also knows when cells have changed, such as when cells become cancerous, and then attacks them. Immunotherapy, also known as biological therapy, was developed to take advantage of the body's own ability to fight disease.

In immunotherapy, substances called biological response modifiers (BRMs) are given to cancer patients. BRMs are substances that are normally made by the body to fight cancer and other diseases. Scientists can make large amounts of BRMs to use in cancer treatment. These BRMs destroy cancer cells and change the way the body reacts to a tumor. They may also help the body replace non-cancerous cells destroyed by chemotherapy.

Several types of BRMs are used to treat cancer. Four are discussed here: cytokines, interleukins, colony stimulating factors, and monoclonal antibodies.

• Cytokines are proteins formed in small amounts by all human cells to help control the working of cells. Interferon, one type of cytokine, helps the immune system slow the rate of growth and division of cancer cells, causing them to become sluggish and die. In children who have cancer, these agents have been tested against leukemia, osteosarcoma, brain tumors, and neuroblastoma.
• Interleukins, another type of cytokine, are made by lymphocytes. Interleukin-2, one of the most studied interleukins, causes certain kinds of white blood cells to grow and destroy tumors.
• Colony-stimulating factors are proteins that cause bone marrow cells to develop platelets, red blood cells, and white blood cells.
• Monoclonal antibodies recognize specific antigens, substances that the body senses do not belong or regards as "outsiders," on the surface of cancer cells. These antibodies can be directed against a certain cell type. They can be used to attack and damage or destroy cancer cells. They may also be used to deliver anticancer drugs or radiation directly to specific cancer cells. This technique is still being developed and is used only in clinical trials.
  Bone Marrow and Peripheral Blood Stem Cell Transplants

Some children who have leukemia or other types of cancer may need a bone marrow transplant (BMT) or a peripheral blood stem cell transplant (PBSCT). In these treatments, the blood cells in the diseased or damaged bone marrow - the spongy material that fills the inside of bones and makes blood cells - are replaced with healthy cells (stem cells), which will grow new healthy cells.

Stem cells are immature cells that produce the three types of blood cells:

• white blood cells, which fight infection
• red blood cells, which carry oxygen to and remove waste products from organs and tissues
• platelets, which enable the blood to clot.
  Most stem cells are found in the bone marrow, but some, called peripheral blood stem cells (PBSCs), are collected from the bloodstream. Umbilical cord blood also contains stem cells. If your doctor proposes a stem cell transplant for your child, the stem cells may come from a donor's bone marrow, bloodstream, or umbilical cord blood or from your child's own bone marrow or bloodstream.

Healthy stem cells grow in two ways:

• They divide to form more stem cells.
• They mature into white cells, red cells, and platelets.

When transplanted into a cancer patient, stem cells can rescue the damaged blood-forming system. Two circumstances create the need for a transplant: The first is when the cancer itself has injured the bone marrow. BMT and PBSCT are commonly used in the treatment of leukemia and lymphoma.

The second circumstance is when a treatment requires such large doses of chemotherapy or radiation therapy that the child's bone marrow is damaged during treatment. Chemotherapy and radiation therapy target all cells that divide rapidly. Cancer cells divide more rapidly than most healthy cells and so are destroyed by the therapies. Bone marrow cells, however, also divide more rapidly than other healthy cells, so high-dosage treatment can have a damaging effect on them as well.

A BMT or PBSCT can be done in three ways: as an autologous transplant, an allogeneic transplant, or a syngeneic transplant.

• Autologous transplant - The child's own stem cells or PBSC cells are used. They can be obtained from the bone marrow or from blood, using the apheresis procedure. Stem cells are collected while the child is in remission. To make sure that any cancer cells that remain are destroyed, the marrow may be treated with anticancer drugs. After the marrow or PBSCs are collected, the child receives high-dose chemotherapy, usually over 2 to 6 days, and, frequently, total body irradiation, during which the whole body is radiated, in one dose or in many doses over several days, to get rid of the cancer. The collected marrow or PBSCs are then returned to the child through a vein, as in a blood transfusion.
• Allogeneic transplant - The marrow or PBSCs from a sibling, parent, or someone not related to the child are used for the transplant. The transplant material must be as closely matched to the patient's blood cells as possible. Cord blood transplant is allogeneic.
• Syngeneic transplant - The perfectly matched marrow or PBSCs from an identical twin are used for the transplant. Before the transplant, a catheter is usually inserted into a large vein in the chest for transplanting the marrow or PBSCs, as well as for giving blood, antibiotics, and other drugs, and for drawing blood. The child is given high doses of anticancer drugs and/or radiation. When the cancer cells - along with some healthy bone marrow cells - are destroyed, the new, healthy marrow or PBSCs can be given. The healthy marrow or PBSCs are given through the catheter and travel through the bloodstream to the bone marrow where they start to make red and white blood cells and platelets.
  It usually takes 14 to 30 days after the transplant for the transplanted stem cells to make enough white blood cells to fight infection and to make other blood cells. During this time, your child will be at higher risk for infections, anemia, and bleeding. Complete recovery of your child's immune system can take 1 to 2 years after an autologous, allogeneic, or syngeneic transplant. Your child may need to remain in a hospital room that is kept as free as possible from anything that may cause an infection. Supportive care, which includes receiving blood transfusions and antibiotics, will be given.

Additional websites and resources are located in the Resources section of this book, and also by calling the Cancer Support Services office at 822-8820.