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Unusual Cancers of Childhood Treatment (PDQ®)
Patient Version   Health Professional Version   En español   Last Modified: 09/30/2008



General Information






Head and Neck Cancers






Thoracic Cancers






Abdominal Cancers






Genital/Urinary Tumors






Other Rare Childhood Cancers






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Changes to This Summary (09/30/2008)






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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 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 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. Treatment of oral cancer in children may include surgery, chemotherapy, and radiation therapy. (Refer to the PDQ summaries on adult Oropharyngeal Cancer Treatment and Lip and Oral Cavity Cancer Treatment 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 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 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.

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