21st Century Pediatric Cancer Sourcebook: Unusual Cancers of Childhood, Head, Thyroid, Thoracic, Esophageal, Heart, Thymomas, Abdominal, Stromal, MEN, Carney Complex, Skin, Chordoma, Others
Edition 1.0 - March 2011
National Cancer Institute
Smashwords Edition
Copyright 2011 Progressive Management
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PART ONE
Chapter 1A: Unusual Cancers of Childhood
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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Chapter 1A: Unusual Cancers of Childhood
General Information * Head and Neck Cancers Cancer of the Nose and Throat * Esthesioneuroblastoma * Thyroid Tumors * Oral (Mouth) Cancers * Salivary Gland Tumors * Laryngeal Cancer and Papillomatosis * Respiratory Tract Cancer with Chromosome 15 Changes * Thoracic Cancers Breast Cancer * Bronchial Tumors * Pleuropulmonary Blastoma * Esophageal Tumors * Thymomas and Thymic Carcinoma * Tumors of the Heart * Mesothelioma * Abdominal Cancers Cancer of the Adrenal Cortex * Cancer of the Stomach * Cancer of the Pancreas * Colorectal Cancer * Carcinoid Tumors * Gastrointestinal Stromal Tumors * Genital/Urinary Tumors Bladder Cancer * Testicular Cancer * Ovarian Cancer * Carcinoma of the Cervix and Vagina * Other Rare Childhood Cancers * Multiple Endocrine Neoplasia Syndromes (MEN) and Carney Complex * Skin Cancer (Melanoma, Basal Cell Carcinoma, Squamous Cell Carcinoma) * Chordoma * Cancer of Unknown Primary Site
Cancer in children and adolescents is rare. Since 1975, the number of new cases of childhood cancer has increased slowly, but deaths from childhood cancer are less than half what they were then. Different types of rare childhood tumors are discussed in this summary. Because these tumors are rare in children, there may not be much information about the best treatment. Sometimes the treatment of these tumors in children is the same treatment used in adults.
Head and Neck Cancers
Head and neck cancers include cancers of the nose and throat, thyroid tumors, mouth cancer, salivary gland cancer, cancer of the larynx (or voice box), and respiratory tract. These cancers are discussed below.
Cancer of the Nose and Throat
Cancers that start in the lining of the nasal cavity and throat are called nasopharyngeal cancers. This type of cancer is very uncommon in children younger than 10 years, and occurs slightly more often in older children and teenagers aged 10 to 19 years.
Nasopharyngeal cancer occurs in association with Epstein-Barr virus (EBV) infection, the virus associated with infectious mononucleosis. This cancer most frequently spreads to lymph nodes in the neck, which may alert the patient, parent, or physician to the presence of this tumor. The tumor may spread to the nose, mouth, and pharynx, causing snoring, nosebleeds, obstruction of the Eustachian tubes, or hearing loss. It may invade the base of the skull, causing cranial nerve palsy or difficulty with movements of the jaw (trismus). The cancer may spread to distant sites such as the bones, lungs, and liver.
Treatment combines the use of surgery, radiation therapy, and chemotherapy. Nasopharyngeal cancer generally has spread to bones of the skull and to lymph nodes in the neck at the time of diagnosis; thus, the principal role of surgery is to obtain adequate diagnostic material from a biopsy of the involved lymph node or the primary site. Studies show that giving chemotherapy before radiation therapy is an effective treatment for this tumor.
Esthesioneuroblastoma
Esthesioneuroblastoma (olfactory neuroblastoma) is a very rare, small tumor that begins in the olfactory bulb (the organ responsible for the sense of smell) located in the front part of the brain. Most children have a tumor in the nose or throat at the time of diagnosis. The tumor may extend into the eyes, sinuses, and the front part of the brain. Esthesioneuroblastoma occurs more often in boys and usually appears during adolescence. The disease seldom spreads to other parts of the body. Treatment for this cancer is usually surgery and radiation therapy; chemotherapy may also be used before or after other treatments. Newer treatments may include sinus surgery done through an endoscope, radiosurgery, or proton beam radiation therapy.
Thyroid Tumors
Tumors of the thyroid (a gland near the windpipe that produces thyroid hormone, which helps regulate growth and metabolism) are classified as adenomas or carcinomas. Adenomas are benign (noncancerous) growths that may cause enlargement of all or part of the gland, which extends to both sides of the neck and can be quite large. Some of these tumors may secrete hormones. Transformation to a malignant carcinoma (cancer) may occur in some cells, which then may grow and spread to lymph nodes in the neck or to the lungs.
Thyroid cancers occur less often in children and adolescents younger than 15 years, and more often in those aged 15 to 19 years. Most thyroid cancers occur in girls. This cancer usually appears as a lump or mass in the thyroid with possible swelling of the lymph glands in the neck.
Surgery is the treatment required for all thyroid tumors. This is usually removal of all or nearly all of the thyroid and nearby lymph nodes in the neck. Treatment with a radioactive form of iodine is given after surgery to destroy cancer cells and thyroid tissue that remain. After surgery and treatment with radioactive iodine, hormone replacement therapy must be given to compensate for the lost thyroid hormone. Regular checkups are required to determine whether the cancer has spread to the lungs. Patients with thyroid cancer generally have an excellent survival with relatively few side effects. Thyroid tumors that recur (come back) are usually treated with radioactive iodine. Even patients with tumor that has spread to the lungs may expect no decrease in life span after appropriate treatment. For patients with thyroid cancer that has spread or come back, targeted therapies that block signals needed for tumors to grow are being studied in clinical trials.
Oral (Mouth) Cancers
Oral cancer in children or in adolescents is extremely rare, although the number of new cases of oral cancer in teenage girls and young women has increased since the mid-1990s. Most oral tumors are benign (not cancer). Malignant tumors include lymphomas (often Burkitt lymphoma) and sarcomas (soft tissue tumors). Oral squamous cell carcinoma (cancer of the thin, flat cells lining the mouth) is the most common type of oral cancer in adults, but is rare in children. Oral squamous cell carcinoma may develop in patients with Fanconi anemia, dyskeratosis congenita (a rare bone marrow failure syndrome), chronic graft-versus-host disease, epidermolysis bullosa, xeroderma pigmentosum, and human papillomavirus infection. Mucoepidermoid carcinomas are rare and may be cured with surgery alone. Treatment of oral cancer in children may include surgery, chemotherapy, and radiation therapy.
Salivary Gland Tumors
Salivary glands are the parts of the mouth and throat that produce saliva. Many of the tumors in these areas arise in the parotid gland. About 15% of these tumors may arise in the submandibular glands or in the minor salivary glands under the tongue and jaw. These tumors are most frequently noncancerous but on very rare occasions may be malignant (cancerous). Sialoblastomas are a type of salivary gland tumor found in the first months of life. They are usually benign (not cancer), but may rarely be cancerous. The malignant lesions include adenocarcinoma, undifferentiated carcinoma, acinic cell carcinomas, and mucoepidermoid carcinoma. These tumors may occur after radiation therapy and chemotherapy are given for treatment of primary leukemia or solid tumors. Complete surgical removal is the treatment of choice whenever possible, with additional use of radiation therapy and chemotherapy. Prognosis (outcome) for patients with these tumors is generally good.
Laryngeal Cancer and Papillomatosis
Benign and especially malignant (cancerous) tumors of the larynx (voice box) are rare. Malignant tumors may be associated with benign tumors such as polyps and papillomas. These tumors may cause hoarseness, difficulty swallowing, and enlargement of the lymph nodes of the neck. Rhabdomyosarcoma (a malignant tumor of muscle tissue) is the most common malignant tumor of the larynx in children and is usually treated with chemotherapy and radiation therapy instead of surgery. Squamous cell carcinoma of the larynx should be managed with surgery and radiation therapy. Laser surgery may be the first type of treatment used for these cancers.
Papillomatosis of the larynx is a benign overgrowth of tissues lining the larynx. It is associated with the human papillomavirus (HPV) and is treated with laser surgery. This condition is not cancerous, but may recur after treatment. These tumors can cause hoarseness because of their association with wart -like nodules on the vocal cords that rarely extend into the lung. Cancerous changes may develop over time in the larynx and lung.
Respiratory Tract Cancer with Chromosome 15 Changes
The respiratory tract includes the nose, throat, larynx, trachea, and lungs. Respiratory tract cancer may be caused by a specific chromosome change. Every cell in the body contains DNA (genetic material stored inside chromosomes) that determines how the cell looks and acts. Cancer may develop when part of the DNA from chromosome 15 moves to another chromosome, or when chromosome 15 is broken. This type of cancer may appear in parts of the airway or in other places along the midline of the body, including the thymus, the area between the lungs, and the bladder. It usually cannot be cured.
Thoracic Cancers
Thoracic cancers include breast cancer, bronchial adenomas, bronchial carcinoid tumors, pleuropulmonary blastoma, esophageal tumors, thymomas and thymic carcinomas, tumors of the heart, and mesothelioma. These cancers are rare in patients younger than 15 years of age.
Breast Cancer
Most tumors that involve the breast during childhood are benign (noncancerous) fibroadenomas that can be watched for change without the need for biopsy. Rarely, such breast tumors will show malignant change with sudden, rapid growth. These are called phyllodes tumors, and require biopsy or surgical removal without mastectomy. Other types of breast cancer have been reported in both males and females younger than 21 years. There is an increased lifetime risk of breast cancer in patients who were treated for any cancer with radiation therapy to the chest area, including female survivors of Hodgkin lymphoma. Mammograms should start at age 25 or 10 years after radiation therapy to the chest, whichever came last. Treatment options for children and adolescents with breast cancer include surgery and radiation therapy. Breast tumors may also occur as cancers that have spread from other types of cancer such as leukemia, rhabdomyosarcoma, other sarcomas, or lymphoma, particularly in patients infected with the human immunodeficiency virus (HIV).
Bronchial Tumors
Bronchial tumors (which are slow-growing cancers in the trachea or large bronchi, the large airways of the lung), are most often carcinoid tumors in children. Symptoms include cough and spitting up blood, and can sometimes be confused with asthma symptoms like wheezing, resulting in delayed diagnosis. Primary treatment is surgery to remove the tumor and lymph nodes and vessels where cancer may spread. The prognosis for most bronchial tumors in children is excellent, even when the cancer has spread to nearby areas. Rarely, carcinoid tumors develop as aggressive (fast-growing) cancers that are more likely to spread to other areas of the body by the time they are diagnosed. For bronchial carcinoid tumors, neither chemotherapy nor radiation therapy is indicated, unless evidence of metastasis (spread of cancer to other areas of the body) is documented. Cancers that begin in the epithelial cells that line the surface of the lung are rare, and tend to be advanced when they are diagnosed. The outcome for epithelial cancers of the lung depends on how the cells look under a microscope and the stage of the cancer.
Pleuropulmonary Blastoma
Pleuropulmonary blastomas are rare tumors that usually occur under the tissue covering the lungs. Changes in a specific chromosome may be found in pleuropulmonary blastoma. Every cell in the body contains DNA (genetic material stored inside chromosomes) that determines how the cell looks and acts. Part of the DNA in chromosome 8 is often abnormal in these tumors. Early-stage pleuropulmonary blastoma develops as a cyst -like tumor in the lung. It usually appears in the first 2 years of life and has a good prognosis. Late-stage tumors are solid and can spread to the brain. When the tumor can be completely removed by surgery at any time during treatment, the chance of recovery is improved. The tumor may recur or spread, in spite of surgical removal. There are no standard treatment options for pleuropulmonary blastoma. Early-stage tumors may be treated with surgery alone, or surgery with adjuvant chemotherapy to help prevent the tumor from coming back. Late-stage tumors may be treated with surgery followed by chemotherapy. Radiation may be used when the tumor cannot be surgically removed. A family history of cancer in close relatives has been noted for many young patients affected by this tumor. Pleuropulmonary blastoma may also occur in siblings of patients.
Esophageal Tumors
Cancer of the esophagus (the muscular tube through which food passes from the throat to the stomach) is rare in the pediatric age group. Symptoms are related to difficulty in swallowing, and associated weight loss. Treatment options for esophageal cancer include either radiation therapy or chemotherapy. Prognosis generally is poor for this cancer, which rarely can be completely removed by surgery.
Thymomas and Thymic Carcinoma
A cancer of the thymus (an organ in the chest, behind the breastbone) is not considered a thymoma (cancer) or a thymic carcinoma unless there are cancerous changes of the epithelial cells that cover the organ. The term thymoma usually describes cancers that do not have obvious changes in the epithelial cells. Thymic carcinoma has clear-cut changes of the epithelial cells. Other tumors that involve the thymus gland include lymphoma (cancer that arises in cells of the lymphatic system) and germ cell tumors (tumors that begin in cells that give rise to sperm or eggs); these tumors are not true thymomas or thymic carcinomas.
Thymomas and thymic carcinomas are rare in children. Various diseases and syndromes are associated with thymomas, including myasthenia gravis, polymyositis, systemic lupus erythematosus, rheumatoid arthritis, thyroiditis, Isaacs syndrome or neuromyotonia (a rare nerve disorder that causes constant muscle stiffness and cramping), and pure red cell aplasia. Endocrine (hormonal) disorders such as hyperthyroidism, Addison’s disease, and panhypopituitarism can also be associated with a diagnosis of thymoma or thymic carcinoma.
Cancer of the thymus may be caused by a specific chromosome change. Every cell in the body contains DNA (genetic material stored inside chromosomes) that determines how the cell looks and acts. Cancer may develop when part of the DNA from chromosome 15 moves to another chromosome, or when chromosome 15 is broken. This type of cancer may appear in the thymus or in other places along the midline of the body, including parts of the airway, the area between the lungs, and the bladder. It usually cannot be cured.
Thymomas and thymic carcinomas are usually located in the front part of the chest and are usually discovered during a routine chest x-ray. Symptoms can include cough, difficulty with swallowing, tightness of the chest, chest pain, and shortness of breath, although nonspecific symptoms may occur. These tumors generally are slow growing but are potentially invasive, with cancer spreading to distant organs or lymph nodes. Surgery is performed with the goal of a complete removal. Radiation therapy is necessary for patients with invasive thymoma or thymic carcinoma, whether or not there has been surgery. Chemotherapy is usually reserved for patients with advanced- stage disease who have not responded to radiation therapy or steroids. The prognosis for patients with invasive thymoma or thymic carcinoma usually is poor, although significantly higher survival rates have been reported for patients with tumors that have not spread to the surrounding areas.
Tumors of the Heart
The most common tumors that begin in the heart are benign. In children, these include rhabdomyomas (tumors of muscle tissue) and fibromas (tumors of fibrous tissue). Myxomas are rare, benign tumors which may be part of an inherited syndrome called Carney complex (see Multiple Endocrine Neoplasia Syndromes (MEN) and Carney Complex section below for more information). Other benign tumors include histiocytoid cardiomyopathy tumors, teratomas, hemangiomas, and neurofibromas. Malignant tumors that begin in the heart are rare, but may include malignant teratomas, rhabdomyosarcomas, and chondrosarcomas.
Patients may have no symptoms for long periods of time. Symptoms include abnormal heart rhythm, enlargement of the heart, fluid in the sac around the heart, and congestive heart failure. Sudden death with no early symptoms is possible. Successful treatment may require surgery (which may include transplantation) and chemotherapy. Benign tumors of heart muscle (rhabdomyomas) usually shrink and go away on their own.
Mesothelioma
Mesothelioma, an extremely rare cancer in children, can involve the tissue coverings of the lung, the heart, or the abdominal organs. These tumors can spread over the surface of organs, without invading far into the underlying tissue, and may spread to nearby or distant lymph nodes. Mesothelioma may develop after successful treatment of an earlier cancer, especially after treatment with radiation. In adults, these tumors have been associated with exposure to asbestos, which was used as building insulation. The amount of exposure required to develop cancer is unknown, and there is no information about the risk of children exposed to asbestos.
Abdominal Cancers
Abdominal cancers include cancer of the adrenal cortex, stomach cancer, cancer of the pancreas, colorectal cancer, carcinoid tumors of the lung or intestine, and gastrointestinal stromal cell tumors. These abdominal cancers are discussed below.
Cancer of the Adrenal Cortex
The adrenal cortex is the outside layer of the adrenal glands. The adrenal glands are a pair of organs near the front side edge of the kidney; their function is to produce hormones such as glucocorticoid and epinephrine. Cancers in this area are classified as carcinomas and adenomas. Adenomas are generally benign, whereas adrenocortical carcinomas frequently secrete hormones and may cause the patient to develop masculine traits, regardless of the patient’s gender. Adrenocortical tumors are seen in children of all ages, including in newborns and before birth. Pediatric patients with adrenocortical carcinoma often have Li-Fraumeni syndrome, an inherited condition that predisposes family members to multiple cancers, including breast cancer, rhabdomyosarcoma, and osteosarcoma (cancer of the bone).
These tumors can involve the kidneys, lungs, bones and brain. Surgical removal should be attempted but may not always be possible if the tumor has spread widely. Additional treatment may include the use of an artificial hormone that blocks the masculinizing effects of the tumor. The prognosis is generally excellent for patients who have small tumors that have been completely removed by surgery, but prognosis can be poor for patients who have large primary tumors or metastatic disease (disease that has spread to other parts of the body) at diagnosis. Tumor stage is an important factor affecting the chance of recovery for children with adrenocortical tumors. When possible, repeat surgery should be done for tumors that come back and for tumors that spread to the inferior vena cava (a large vein that empties into the heart).
Cancer of the Stomach
Cancer that begins in the stomach is rare in children. Symptoms of stomach cancer include vague upper abdominal pain, which can be associated with poor appetite, and weight loss. Many individuals become anemic but otherwise show no symptoms before the development of metastatic spread. Other symptoms may include nausea, vomiting, change in bowel habits, poor appetite and weakness, and Helicobacter pylori infection.
Treatment should include surgery. For individuals who cannot have a complete surgical removal of tissue, radiation therapy may be used along with chemotherapy. Prognosis depends on the extent of the disease at the time of diagnosis and the success of treatment that is appropriate for the clinical situation. Because of the rarity of stomach cancer in the pediatric age group, little information exists regarding treatment outcomes of children.
Cancer of the Pancreas
Tumors of the pancreas (a gland in the abdomen that makes pancreatic juices and produces hormones) are rare in children and adolescents. Tumors included within the general category can arise at any site in the pancreas. Most pancreatic tumors do not secrete hormones, although some tumors secrete insulin, which can lead to symptoms of weakness, fatigue, hypoglycemia, and coma. If a tumor interferes with the normal function of the islet cells (cells in the pancreas that produce hormones), patients may have watery diarrhea or abnormalities of salt balance. At times, there is obstruction of the head of the pancreas, which is associated with jaundice and gastrointestinal bleeding.
Treatment includes various surgical procedures to remove the pancreas and duodenum or part of the pancreas. For pediatric patients, the effectiveness of radiation therapy is not known. Chemotherapy may be useful for treatment of localized or metastatic pancreatic carcinoma, although few cases have been successfully treated. Pancreatoblastoma may be treated with combination chemotherapy given before or after surgery. Response rates and survival rates generally are not good.
Colorectal Cancer
Cancer of the large bowel is rare in the pediatric age group: one person per one million younger than 20 years in the United States annually. In children, more than half of colon tumors begin on the right side, compared with adults, who have more colon tumors on the left side. Colon cancer in children is often linked to a family colorectal cancer syndrome, or inherited pattern. There is an increasing risk of colorectal cancer in members of families with a family history of intestinal polyps, which can lead to the development of multiple adenomatous polyps (benign tumors). Juvenile polyps are not associated with an increased incidence or risk of cancer.
Colorectal cancer usually presents with symptoms related to the site of the tumor. Changes in bowel habits are associated with tumors of the rectum or lower colon. Tumors of the right colon may cause more subtle symptoms but are often associated with an abdominal mass, weight loss, decreased appetite, and blood in the stool. Any tumor that causes complete obstruction of the large bowel can cause bowel perforation and spread of the tumor cells within the abdominal cavity.
Colorectal carcinoma is rarely diagnosed in a pediatric patient; however, vague gastrointestinal symptoms should alert the physician to investigate this possibility. Most patients present with evidence of metastatic disease (cancer that has spread to other body parts), either as gross tumor or as microscopic deposits in lymph nodes, on the surface of the bowel, or other organs within the abdomen. Complete surgical removal should be the primary aim of the surgeon, but in most instances, this is impossible; removal of large portions of tumor provides little benefit for the individuals with extensive metastatic disease. Most patients with microscopic metastatic disease generally develop gross metastatic disease, and few individuals with metastatic disease at diagnosis become long-term survivors.
Current therapy includes the use of radiation therapy for rectal and lower colon tumors, in conjunction with chemotherapy.
Carcinoid Tumors
Carcinoid tumors can involve the lining of the lung, the large or small bowel, or the liver, and may be benign or cancerous. Most lung lesions are benign. Treatment of metastatic carcinoid tumors of the large bowel or stomach becomes more complicated and requires treatment similar to that given for colorectal cancer.
Gastrointestinal Stromal Tumors
Gastrointestinal stromal cell tumor (GIST) usually begins in cells in the wall of the gastrointestinal tract. It may or may not be cancerous. This tumor is usually found in adults and is rare in children. GIST is more common in girls, and usually appears in patients over the age of 10 years. Most children with GIST have tumors in the stomach and develop anemia caused by gastrointestinal bleeding. A small number of children with GIST are found to have one of two rare genetic disorders, Carney triad or Carney Stratakis syndrome, and GIST in children with these disorders has a better chance of being cured.
GIST in children is not the same as the disease in adults. Patients should be seen at centers that specialize in the treatment of GIST and all tumors should be tested for genetic changes. A small number of children have tumors with genetic changes similar to those found in adult patients. These children are treated with a type of drug called a tyrosine kinase inhibitor that blocks these changes.
Treatment for most children whose tumors do not show these genetic changes includes surgery to remove the tumor when possible. If the disease comes back in the same place or cannot be removed but is not causing symptoms, watchful waiting may be recommended. If the disease progresses or causes symptoms, treatment may include a clinical trial of a tyrosine kinase inhibitor.
Genital/Urinary Tumors
Genital/urinary tumors include bladder cancer, ovarian cancer, cervical cancer, and vaginal cancer. These cancers are discussed below.
Bladder Cancer
Bladder cancer is extremely rare in children. The most common carcinoma to involve the bladder is transitional cell carcinoma, which generally presents with blood in the urine. The diagnosis and treatment of bladder cancer are the same for children, adolescents, and adults. Adolescents who develop this tumor are often prone to the development of other cancers. Bladder cancer in adolescents may develop as a late effect of certain chemotherapy drugs given for other childhood tumors or leukemia.
Testicular Cancer
Testicular tumors are very rare in young boys. There are 2 types of testicular tumors, germ cell and non-germ cell tumors. Germ cell tumors begin in cells that give rise to sperm or eggs, and can be either benign or malignant. Testicular germ cell tumors include benign teratomas (the most common testicular tumors in boys), and malignant nonseminomas.
Non-germ cell tumors, such as stromal tumors, are very rare in boys, and are usually benign. Testicular tumors in young boys may be treated with surgery, but there are few studies in children.
Ovarian Cancer
Most ovarian tumors in children are benign (noncancerous). The most common cancers that affect the ovaries are of germ cell origin (beginning in cells that give rise to sperm or eggs); these are more common in children than in adults. Ovarian tumors most frequently occur in girls aged 15 to 19 years. Common symptoms of ovarian cancer include painful periods and pain in the abdomen. Treatment is stage -related and may include surgery, radiation therapy, and chemotherapy. Sertoli-Leydig cell tumors of the ovary are rare in young girls and may cause development of male sex traits or early signs of puberty. Small cell carcinomas of the ovary are very rare and fast-growing tumors that may cause higher than normal levels of calcium in the blood. Small cell carcinomas may be treated with surgery and chemotherapy, but there are few studies in children.
Carcinoma of the Cervix and Vagina
Cancer of the cervix and vagina is very rare in children and adolescents. Most young patients diagnosed with cancer of the cervix or vagina were exposed to a drug called diethylstilbestrol (DES) before birth when it was given to their mothers. DES was given to pregnant women between 1945 and 1970 to keep them from losing their babies (miscarriage). The most common symptom of cancer of the cervix or vagina is vaginal bleeding. Treatment includes surgery followed by radiation and possibly chemotherapy.
Other Rare Childhood Cancers
Other rare childhood cancers include multiple endocrine neoplasia syndrome, skin cancer, chordoma, and cancer of unknown primary site. These other rare childhood cancers are discussed below.
Multiple Endocrine Neoplasia Syndromes (MEN) and Carney Complex
Multiple endocrine neoplasia syndromes or MEN are familial disorders that cause abnormal changes in more than one endocrine organ at the same time (endocrine tissue secretes hormones). These changes may include hyperplasia (overgrowth of tissue) or benign (noncancerous) tumors.
These disorders, called MEN 1, MEN 2A, MEN 2B, and familial medullary carcinoma of the thyroid (FMTC), are caused by abnormal genes. Patients and family members at risk for these syndromes should have both genetic testing and physical examinations.
MEN 1 (Werner syndrome) may involve tumors of the pituitary and parathyroid glands, adrenal, gastric, and pancreatic structures.
MEN 2A (Sipple syndrome) is associated with medullary thyroid carcinoma (cancer that forms in the cells of the thyroid that make the hormone calcitonin) and parathyroid hyperplasia, as well as pheochromocytoma (a usually benign tumor that forms in the center of the adrenal gland, causing it to make too much adrenaline). MEN 2B is associated with medullary thyroid carcinoma, parathyroid hyperplasia, and adenomas as well as pheochromocytoma, mucosal neuromas, and ganglioneuromas.
Patients with the MEN 2A syndrome usually have surgery to remove the thyroid by the age of 5 years or at the time genetic changes are found in order to prevent cancer. Family members of patients with the MEN 2A syndrome should be tested in early childhood, before age 5, for the abnormal gene that leads to this type of cancer. If they are found to have this genetic change, they should have surgery to remove the thyroid and replace it with a transplant of one of their own parathyroid glands by a certain age.
Patients with the MEN 2B syndrome may have a slender body build, long and thin extremities, a high arch palate, and “funnel chest” (sunken chest) or an abnormally high arch in the foot. The lips may appear thickened because of tumors in the mucous membranes. In this syndrome, medullary thyroid cancer may be particularly aggressive; therefore, the thyroid should be removed in infancy to prevent cancer in these affected children.
Familial medullary thyroid carcinoma (FMTC) is an inherited form of medullary thyroid cancer that occurs without pheochromocytoma or parathyroid adenoma/hyperplasia.
Hirschsprung disease (a condition present at birth that causes blockage of the large intestine due to lack of muscle contractions) is also associated with medullary thyroid carcinoma. Patients with Hirschsprung disease should be screened for genetic changes that may be linked to this cancer. If such changes are found, surgery to remove the thyroid in order to prevent cancer may be considered.
The Carney complex is an inherited syndrome that causes myxomas (benign tumors) of the heart and skin, lentigines (brown sun spots on the skin), blue nevi (benign moles), Cushing syndrome, and endocrine and non-endocrine tumors. For patients with the Carney complex, prognosis depends on how often heart, skin, or other tumors recur.
The outcome for patients with the MEN 1 syndrome is generally good, provided adequate treatment can be obtained for parathyroid, pancreatic, and pituitary tumors. The outcome for patients with the MEN 2A syndrome is also generally good, but the possibility exists for recurrence of medullary thyroid carcinoma and pheochromocytoma. Medullary thyroid cancer in children with MEN 2B may be difficult to cure.
Treatment of medullary thyroid carcinoma may include a clinical trial of a tyrosine kinase inhibitor, a drug that interferes with cell communication and growth and may prevent tumor growth, taken by mouth.
Skin Cancer (Melanoma, Basal Cell Carcinoma, Squamous Cell Carcinoma)
Melanoma is the most common skin cancer in children, followed by basal cell and squamous cell carcinomas. The incidence of melanoma in children and adolescents represents approximately 1% of the new cases of melanoma that are diagnosed annually in the United States. Melanoma occurs less often in children younger than 10 years, and occurs more often in older children and adolescents aged 10 to 19 years. The rate of melanoma in children has increased in recent decades. Higher levels of ultraviolet light from the sun increases the risk of this cancer.
Melanoma begins in skin cells called melanocytes (cells that color the skin). Diagnosing melanoma in children is difficult because melanoma looks similar to another type of skin tumor that starts in the melanocytes, which may need different treatment. New kinds of tests are being used to tell the difference between melanoma and these other tumors.
Conditions that increase the risk of melanoma in children and adolescents include giant melanocytic nevi (large congenital black spots which may cover the trunk and thigh), xeroderma pigmentosum (a rare genetic condition marked by an extreme sensitivity to sunlight), certain disorders of the immune system, and Werner syndrome. Traits in all age groups that increase the risk of melanoma include being exposed to ultraviolet radiation, having red hair, blue eyes, or freckling, poor tanning, and family history of melanoma. Melanoma in children is similar in many ways to the disease in adults, although the thickness of lesions in children does not seem to predict how likely it is that the melanoma will be cured.
Basal cell carcinoma generally appears as a raised lump or ulcerated lesion, usually in areas with previous sun exposure. Squamous cell carcinomas are usually reddened lesions with varying degrees of scaling or crusting; they have an appearance similar to eczema, infections, trauma, or psoriasis.
Basal and squamous cell carcinomas are generally curable with surgery alone, but the treatment of melanoma requires greater consideration because of its potential for metastasis. Surgery for melanoma depends on the size, site, level of invasion, and metastatic extent or stage of the tumor. If the melanoma has not spread beyond the lymph nodes, adjuvant biologic therapy may be given to increase the chances of a cure. If the melanoma has spread beyond the lymph nodes, treatment may include a combination of chemotherapy and biologic therapy, or a clinical trial of a monoclonal antibody. Information about ongoing clinical trials for melanoma in children aged 10 years or older is available from the NCI Web site.
Chordoma
Chordoma is a very rare type of bone tumor that may develop along the spine at any point from the base of the skull to the tailbone. Chordomas start in clusters of cells leftover from spinal column development in the embryo. These cells normally disappear, but very rarely they remain and grow into tumors. In children and adolescents, chordomas develop more often in the base of the skull, making them difficult to completely remove with surgery. Symptoms vary and may include pain and nerve trouble. When chordoma recurs, it usually comes back in the same area, but may appear in the lungs or other areas of bone.
Standard treatment includes surgery and radiation therapy. The best results are seen with proton beam therapy, a special kind of high-energy radiation that is different from an x-ray.
Cancer of Unknown Primary Site
Cancer can form in any tissue of the body and can spread from the primary site (the place where the cancer first began to grow) to other parts of the body. Cancer that has spread from the place in which it started to other parts of the body is called metastatic cancer. Metastatic cancer cells usually look like cells in the type of tissue where the cancer began. For example, breast cancer cells that spread to the lung look like breast cancer cells, not lung cancer cells. Sometimes metastatic cancer cells are found in the body, but tests do not find a primary tumor. If cancer cells are found in the body but the place where the cancer started cannot be identified, the disease is called cancer of unknown primary site. Treatment is based on what the cancer cells look like under a microscope, the patient's age and symptoms, and the extent of the cancer in the body. Treatment is usually chemotherapy or radiation therapy.
To Learn More About Childhood Cancer
For more childhood cancer information and other general cancer resources from the National Cancer Institute, see the following:
Glossary Terms
cancer (KAN-ser)
A term for diseases in which abnormal cells divide without control and can invade nearby tissues. Cancer cells can also spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is a cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is a cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is a cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord. Also called malignancy.
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.
adenocarcinoma (A-den-oh-KAR-sih-NOH-muh)
Cancer that begins in cells that line certain internal organs and that have gland-like (secretory) properties.
adenoma (A-deh-NOH-muh)
A tumor that is not cancer. It starts in gland-like cells of the epithelial tissue (thin layer of tissue that covers organs, glands, and other structures within the body).
benign (beh-NINE)
Not cancerous. Benign tumors may grow larger but do not spread to other parts of the body. Also called nonmalignant.
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.
bladder (BLA-der)
The organ that stores urine.
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.
Burkitt lymphoma (BER-kit lim-FOH-muh)
An aggressive (fast-growing) type of B-cell non-Hodgkin lymphoma that occurs most often in children and young adults. The disease may affect the jaw, central nervous system, bowel, kidneys, ovaries, or other organs. There are three main types of Burkitt lymphoma (sporadic, endemic, and immunodeficiency related). Sporadic Burkitt lymphoma occurs throughout the world, and endemic Burkitt lymphoma occurs in Africa. Immunodeficiency-related Burkitt lymphoma is most often seen in AIDS patients.
cancer (KAN-ser)
A term for diseases in which abnormal cells divide without control and can invade nearby tissues. Cancer cells can also spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is a cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is a cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is a cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord. Also called malignancy.
carcinoma (KAR-sih-NOH-muh)
Cancer that begins in the skin or in tissues that line or cover internal organs.
cell (sel)
The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.
chemotherapy (KEE-moh-THAYR-uh-pee)
Treatment with drugs that kill cancer cells.
chromosome (KROH-muh-some)
Part of a cell that contains genetic information. Except for sperm and eggs, all human cells contain 46 chromosomes.
chronic (KRAH-nik)
A disease or condition that persists or progresses over a long period of time.
clinical trial (KLIH-nih-kul TRY-ul)
A type of research study that tests how well new medical approaches work in people. These studies test new methods of screening, prevention, diagnosis, or treatment of a disease. Also called clinical study.
cure
To heal or restore health; a treatment to restore health.
diagnosis (DY-ug-NOH-sis)
The process of identifying a disease, such as cancer, from its signs and symptoms.
DNA
The molecules inside cells that carry genetic information and pass it from one generation to the next. Also called deoxyribonucleic acid.
endoscope (EN-doh-SKOPE)
A thin, tube-like instrument used to look at tissues inside the body. An endoscope has a light and a lens for viewing and may have a tool to remove tissue.
epidermolysis bullosa (EP-ih-der-MOLE-uh-sis bul-OH-suh)
A group of chronic skin disorders in which fluid-filled blisters form on the skin and mucosa (the moist, inner lining of some organs and body cavities). Epidermolysis bullosa is inherited and usually starts at birth. Patients with epidermolysis bullosa may be at increased risk of squamous cell cancer of the skin.
Epstein-Barr virus (ep-stine-BAR VY-rus)
A common virus that remains dormant in most people. It causes infectious mononucleosis and has been associated with certain cancers, including Burkitt lymphoma, immunoblastic lymphoma, and nasopharyngeal carcinoma. Also called EBV.
Fanconi anemia (fan-KOH-nee uh-NEE-mee-uh)
A rare inherited disorder in which the bone marrow does not make blood cells. It is usually diagnosed in children between 2 and 15 years old. Symptoms include frequent infections, easy bleeding, and extreme tiredness. People with Fanconi anemia may have a small skeleton and brown spots on the skin. They also have an increased risk of developing certain types of cancer.
genetic (jeh-NEH-tik)
Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.
gland
An organ that makes one or more substances, such as hormones, digestive juices, sweat, tears, saliva, or milk. Endocrine glands release the substances directly into the bloodstream. Exocrine glands release the substances into a duct or opening to the inside or outside of the body.