National Cancer Institute National Cancer Institute
U.S. National Institutes of Health National Cancer Institute
Send to Printer
Unusual Cancers of Childhood Treatment (PDQ®)     
Last Modified: 02/24/2009
Patient Version
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

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. The incidence of this tumor is approximately 1 in 100,000 persons younger than 20 years in the United States.

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 combining chemotherapy with radiation therapy is the most effective treatment for this tumor. Refer to the PDQ summary on Nasopharyngeal Cancer Treatment 1 for more information.

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. 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 carcinomas are rare and occur most often 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. (Refer to the PDQ summary on adult Thyroid Cancer Treatment 2 for more information.)

Oral (Mouth) Cancers

Oral cancer in children or in adolescents is extremely rare. 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; adolescents (teens) with oral squamous cell carcinoma should be screened for a condition called Fanconi anemia. Mucoepidermoid carcinomas are rare and may be cured with surgery alone. Treatment of oral cancer in children may include surgery, chemotherapy, and radiation therapy. (Refer to the PDQ summaries on adult Oropharyngeal Cancer Treatment 3 and Lip and Oral Cavity Cancer Treatment 4 for more information).

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. (Refer to the PDQ summary on adult Salivary Gland Cancer Treatment 5 for more information.)

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 the pediatric age group. Squamous cell carcinoma of the larynx should be managed with surgery and radiation. 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). 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; they may extend into the lung and develop into cancer in the larynx. Treatment includes laser surgery. (Refer to the PDQ summary on adult Laryngeal Cancer Treatment 6 for more information.)

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.



Glossary Terms

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 noncancerous tumor that 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 cancer. Benign tumors may grow larger but do not spread to other parts of the body.
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.
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 cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is 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.
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.
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.
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.
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.
hormone replacement therapy (HOR-mone ree-PLAYS-ment THAYR-uh-pee)
Hormones (estrogen, progesterone, or both) given to women after menopause to replace the hormones no longer produced by the ovaries. Also called HRT and menopausal hormone therapy.
human papillomavirus (HYOO-mun PA-pih-LOH-muh-VY-rus)
A member of a family of viruses that can cause abnormal tissue growth (for example, genital warts) and other changes to cells. Infection with certain types of human papillomavirus increases the risk of developing cervical cancer. Also called HPV.
incidence
The number of new cases of a disease diagnosed each year.
infection
Invasion and multiplication of germs in the body. Infections can occur in any part of the body and can spread throughout the body. The germs may be bacteria, viruses, yeast, or fungi. They can cause a fever and other problems, depending on where the infection occurs. When the body’s natural defense system is strong, it can often fight the germs and prevent infection. Some cancer treatments can weaken the natural defense system.
iodine (I-oh-dine)
An element that is necessary for the body to make thyroid hormone. It is found in shellfish and iodized salt.
larynx (LAYR-inks)
The area of the throat containing the vocal cords and used for breathing, swallowing, and talking. Also called voice box.
laser surgery (LAY-zer SER-juh-ree)
A surgical procedure that uses the cutting power of a laser beam to make bloodless cuts in tissue or to remove a surface lesion such as a tumor.
lesion (LEE-zhun)
An area of abnormal tissue. A lesion may be benign (not cancer) or malignant (cancer).
leukemia (loo-KEE-mee-uh)
Cancer that starts in blood-forming tissue such as the bone marrow and causes large numbers of blood cells to be produced and enter the bloodstream.
liver
A large organ located in the upper abdomen. The liver cleanses the blood and aids in digestion by secreting bile.
lung
One of a pair of organs in the chest that supplies the body with oxygen, and removes carbon dioxide from the body.
lymph gland
A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph glands filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph node.
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.
lymphoma (lim-FOH-muh)
Cancer that begins in cells of the immune system. There are two basic categories of lymphomas. One kind is Hodgkin lymphoma, which is marked by the presence of a type of cell called the Reed-Sternberg cell. The other category is non-Hodgkin lymphomas, which includes a large, diverse group of cancers of immune system cells. Non-Hodgkin lymphomas can be further divided into cancers that have an indolent (slow-growing) course and those that have an aggressive (fast-growing) course. These subtypes behave and respond to treatment differently. Both Hodgkin and non-Hodgkin lymphomas can occur in children and adults, and prognosis and treatment depend on the stage and the type of cancer.
malignant (muh-LIG-nunt)
Cancerous. Malignant tumors can invade and destroy nearby tissue and spread to other parts of the body.
metabolism (meh-TA-boh-lih-zum)
The total of all chemical changes that take place in a cell or an organism. These changes make energy and the materials needed for growth, reproduction, and maintaining health. They also help get rid of toxic substances.
nasal (NAY-zul)
By or having to do with the nose.
nasopharynx (NAY-zoh-fayr-inx)
The upper part of the throat behind the nose. An opening on each side of the nasopharynx leads into the ear.
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.
nodule (NOD-yool)
A growth or lump that may be cancerous or noncancerous.
obstruction
Blockage of a passageway.
oral cancer (OR-ul KAN-ser)
Cancer that forms in tissues of the oral cavity (the mouth) or the oropharynx (the part of the throat at the back of the mouth).
organ
A part of the body that performs a specific function. For example, the heart is an organ.
PDQ
PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.
pediatric (pee-dee-A-trik)
Having to do with children.
pharynx (FAIR-inks)
The hollow tube inside the neck that starts behind the nose and ends at the top of the trachea (windpipe) and esophagus (the tube that goes to the stomach). The pharynx is about 5 inches long, depending on body size. Also called throat.
physician (fih-ZIH-shun)
Medical doctor.
polyp (PAH-lip)
A growth that protrudes from a mucous membrane.
primary tumor
The original tumor.
prognosis (prog-NO-sis)
The likely outcome or course of a disease; the chance of recovery or recurrence.
proton beam radiation therapy (PROH-ton beem RAY-dee-AY-shun THAYR-uh-pee)
A type of high-energy, external radiation therapy that uses streams of protons (small, positively charged particles) that come from a special machine. Proton beam radiation is different from x-ray radiation.
radiation therapy (RAY-dee-AY-shun THAYR-uh-pee)
The use of high-energy radiation from x-rays, gamma rays, neutrons, protons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body near cancer cells (internal radiation therapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that travels in the blood to tissues throughout the body. Also called irradiation and radiotherapy.
radioactive (RAY-dee-oh-AK-tiv)
Giving off radiation.
radiosurgery (RAY-dee-oh-SER-juh-ree)
A type of external radiation therapy that uses special equipment to position the patient and precisely give a single large dose of radiation to a tumor. It is used to treat brain tumors and other brain disorders that cannot be treated by regular surgery. It is also being studied in the treatment of other types of cancer. Also called radiation surgery, stereotactic radiosurgery, and stereotaxic radiosurgery.
recur
To come back or to return.
respiratory tract (RES-pih-ruh-TOR-ee trakt)
The organs that are involved in breathing. These include the nose, throat, larynx, trachea, bronchi, and lungs. Also called respiratory system.
rhabdomyosarcoma (RAB-doh-MY-oh-sar-KOH-muh)
Cancer that forms in the soft tissues in a type of muscle called striated muscle. Rhabdomyosarcoma can occur anywhere in the body.
saliva (suh-LIE-vuh)
The watery fluid in the mouth made by the salivary glands. Saliva moistens food to help digestion and it helps protect the mouth against infections.
salivary gland (SA-lih-VAYR-ee gland)
A gland in the mouth that produces saliva.
sarcoma
A cancer of the bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue.
side effect
A problem that occurs when treatment affects healthy tissues or organs. Some common side effects of cancer treatment are fatigue, pain, nausea, vomiting, decreased blood cell counts, hair loss, and mouth sores.
soft tissue
Refers to muscle, fat, fibrous tissue, blood vessels, or other supporting tissue of the body.
solid tumor
An abnormal mass of tissue that usually does not contain cysts or liquid areas. Solid tumors may be benign (not cancer), or malignant (cancer). Different types of solid tumors are named for the type of cells that form them. Examples of solid tumors are sarcomas, carcinomas, and lymphomas. Leukemias (cancers of the blood) generally do not form solid tumors.
squamous cell carcinoma (SKWAY-mus sel KAR-sih-NOH-muh)
Cancer that begins in squamous cells, which are thin, flat cells that look like fish scales. Squamous cells are found in the tissue that forms the surface of the skin, the lining of the hollow organs of the body, and the passages of the respiratory and digestive tracts. Also called epidermoid carcinoma.
surgery (SER-juh-ree)
A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.
throat (throte)
The hollow tube inside the neck that starts behind the nose and ends at the top of the trachea (windpipe) and esophagus (the tube that goes to the stomach). The throat is about 5 inches long, depending on body size. Also called pharynx.
thymus
An organ that is part of the lymphatic system, in which T lymphocytes grow and multiply. The thymus is in the chest behind the breastbone.
thyroid (THY-royd)
A gland located beneath the voice box (larynx) that makes thyroid hormone and calcitonin. The thyroid helps regulate growth and metabolism.
thyroid hormone (THY-royd HOR-mone)
A hormone that affects heart rate, blood pressure, body temperature, and weight. Thyroid hormone is made by the thyroid gland and can also be made in the laboratory.
tissue (TISH-oo)
A group or layer of cells that work together to perform a specific function.
trachea (TRAY-kee-uh)
The airway that leads from the larynx (voice box) to the bronchi (large airways that lead to the lungs). Also called windpipe.
transformation (TRANZ-for-MAY-shun)
In medicine, the change that a normal cell undergoes as it becomes malignant.
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.
undifferentiated
A term used to describe cells or tissues that do not have specialized ("mature") structures or functions. Undifferentiated cancer cells often grow and spread quickly.
virus (VY-rus)
In medicine, a very simple microorganism that infects cells and may cause disease. Because viruses can multiply only inside infected cells, they are not considered to be alive.
vocal cord (VOH-kul kord)
One of two small bands of muscle within the larynx that vibrates to produce the voice.
wart
A raised growth on the surface of the skin or other organ.
windpipe
The airway that leads from the larynx (voice box) to the bronchi (large airways that lead to the lungs). Also called trachea.


Table of Links

1http://www.cancer.gov/cancertopics/pdq/treatment/nasopharyngeal/Patient
2http://www.cancer.gov/cancertopics/pdq/treatment/thyroid/Patient
3http://www.cancer.gov/cancertopics/pdq/treatment/oropharyngeal/Patient
4http://www.cancer.gov/cancertopics/pdq/treatment/lip-and-oral-cavity/Patient
5http://www.cancer.gov/cancertopics/pdq/treatment/salivarygland/Patient
6http://www.cancer.gov/cancertopics/pdq/treatment/laryngeal/Patient