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Unusual Cancers of Childhood (PDQ®)

  • Last Modified: 08/15/2014

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Unusual Cancers of the Head and Neck

Nasopharyngeal Cancer
Esthesioneuroblastoma
Thyroid Tumors
Oral Cavity Cancer
Salivary Gland Tumors
Laryngeal Cancer and Papillomatosis
Midline Tract Cancer with NUT Gene Changes (NUT Midline Carcinoma)



Nasopharyngeal Cancer

Nasopharyngeal cancer is a disease in which malignant (cancer) cells form in the lining of the nasal cavity (inside of the nose) and throat. It is rare in children younger than 10 and more common in teenagers.

Risk Factors, Signs and Symptoms, and Diagnostic and Staging Tests

The risk of nasopharyngeal cancer is greatly increased by having an infection with the Epstein-Barr virus (EBV), which infects cells of the immune system.

Nasopharyngeal cancer may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

  • Painless lumps in the neck.
  • Nosebleeds.
  • Blocked or stuffy nose.
  • Ear infection.
  • Snoring.
  • Problems moving the jaw.
  • Hearing loss.
  • Double vision.

Other conditions that are not nasopharyngeal cancer may cause these same signs and symptoms.

When nasopharyngeal is diagnosed, it usually has already spread to lymph nodes in the neck and bones of the skull. It may also spread to the nose, mouth, throat, bones, lung, and/or liver.

Tests to diagnose and stage nasopharyngeal cancer may include the following:

See the General Information section for a description of these tests and procedures.

Other tests used to diagnose or stage nasopharyngeal cancer include the following:

  • Nasoscopy : A procedure in which a doctor inserts a nasoscope (a thin, lighted tube) into the patient’s nose to look for abnormal areas.

  • Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.

  • Epstein-Barr virus (EBV) tests: Blood tests to check for antibodies to the Epstein-Barr virus and DNA markers of the Epstein-Barr virus. These are found in the blood of patients who have been infected with EBV.

Prognosis

The prognosis (chance of recovery) for most young patients with nasopharyngeal cancer is very good. The prognosis and treatment options depend on the following:

  • The size of the tumor at diagnosis.
  • Whether the tumor has spread to nearby tissues, lymph nodes, or distant parts of the body.
  • How the cancer responds to the initial treatment.

Treatment

Treatment of nasopharyngeal cancer in children may include the following:

Young patients are more likely than adults to have problems caused by treatment, including second cancers.

See the PDQ summary on adult Nasopharyngeal Cancer Treatment for more information.

Esthesioneuroblastoma

Esthesioneuroblastoma (olfactory neuroblastoma) is a tumor that begins in the olfactory bulb in the brain. The olfactory bulb connects to the nerve that is important to the sense of smell. Even though it is rare, esthesioneuroblastoma is the most common tumor of the nasal cavity in children.

Most children have a tumor in the nose or throat at the time of diagnosis. The tumor may spread into the bone around the eyes, sinuses, and the front part of the brain. The disease rarely spreads to other parts of the body. Esthesioneuroblastoma is more common in boys and usually appears during the teen years.

Signs and Symptoms and Staging Tests

Esthesioneuroblastoma may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

  • Blocked nose.
  • Nosebleeds.
  • Loss of the sense of smell.
  • Bulging of the eye.
  • Frequent sinus infections.

Other conditions that are not esthesioneuroblastoma may cause these same signs and symptoms.

Esthesioneuroblastoma has usually spread by the time it is diagnosed. Tests to stage esthesioneuroblastoma may include the following:

See the General Information section for a description of these tests and procedures.

Prognosis

The prognosis (chance of recovery) depends on whether the cancer is only in the nose or if it has spread to nearby lymph nodes or to other parts of the body.

Treatment

Treatment of esthesioneuroblastoma in children may include the following:

Thyroid Tumors

Thyroid tumors form in the tissues of the thyroid gland, which is a butterfly-shaped gland at the base of the throat near the windpipe. The thyroid gland makes important hormones that help control growth, heart rate, body temperature, and how quickly food is changed into energy.

Most childhood thyroid tumors occur in girls and children aged 15 to 19 years. Thyroid tumors may be adenomas (noncancer) or carcinomas (cancer).

Risk Factors, Signs and Symptoms, and Diagnostic and Staging Tests

The risk of thyroid cancer is increased by being exposed to radiation and by certain genetic syndromes, such as multiple endocrine neoplasia (MEN) type 2A syndrome or multiple endocrine neoplasia (MEN) type 2B syndrome. See the Multiple Endocrine Neoplasia Syndromes and Carney Complex section of this summary for more information.

Thyroid tumors may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

  • A lump in the neck or near the collarbone.
  • Trouble breathing.
  • Trouble swallowing.
  • Hoarseness or a change in the voice.

Other conditions that are not thyroid tumors may cause these same signs and symptoms.

Tests to diagnose and stage thyroid tumors may include the following:

See the General Information section for a description of these tests and procedures.

Other tests used to diagnose and stage thyroid tumors include the following:

  • Ultrasound : A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. This procedure can show the size of a thyroid tumor and whether it is solid or a fluid -filled cyst. Ultrasound may be used to guide a fine-needle aspiration (FNA) biopsy.

  • Thyroid function test: The blood is checked for abnormal levels of thyroid-stimulating hormone (TSH). TSH is made by the pituitary gland in the brain. It stimulates the release of thyroid hormone and controls how fast follicular thyroid cells grow. The blood may also be checked for high levels of the hormone calcitonin.

  • Thyroglobulin test: The blood is checked for the amount of thyroglobulin, a protein made by the thyroid gland. Thyroglobulin levels are low or absent with normal thyroid function but may be higher with thyroid cancer or other conditions.

Prognosis

The prognosis (chance of recovery) depends on the following:

  • Gender.
  • The size of the tumor.
  • Whether the tumor has spread to other parts of the body at diagnosis.

Treatment

Treatment of thyroid tumors in children may include the following:

Four to six weeks after surgery a radioactive iodine scan (RAI scan) is done to find areas in the body where thyroid cancer cells that were not removed during surgery may be dividing quickly. RAI is used because only thyroid cells take up iodine. A very small amount of RAI is swallowed, travels through the blood, and collects in thyroid tissue and thyroid cancer cells anywhere in the body. If no cancer cells are found, a larger dose of RAI is given to destroy any remaining thyroid tissue. If cancer remains in the lymph nodes or has spread to other parts of the body, an even larger dose of RAI is given to destroy any remaining thyroid tissue and thyroid cancer cells.

It is common for thyroid cancer to recur, especially in children younger than 10 years and those with cancer in the lymph nodes. Lifelong follow-up of thyroid hormone levels in the blood is needed to make sure the right amount of hormone replacement therapy (HRT) is being given. It is possible that thyroid cancer will spread to the lung later. Tests are done to check for thyroid cancer in the lung.

See the PDQ summary on adult Thyroid Cancer Treatment for more information.

Oral Cavity Cancer

Oral cavity cancer is a disease in which malignant (cancer) cells form in the tissues of the mouth. Most tumors in the oral cavity are benign (not cancer). The most common type of oral cavity cancer in adults, squamous cell carcinoma (cancer of the thin, flat cells lining the mouth), is very rare in children. Malignant tumors in children include lymphomas and sarcomas.

Signs and Symptoms, and Diagnostic and Staging Tests

Oral cavity cancer may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

  • A sore in the mouth that does not heal.
  • A lump or thickening in the oral cavity.
  • A white or red patch on the gums, tongue, or lining of the mouth.
  • Bleeding, pain, or numbness in the mouth.

Other conditions that are not oral cavity cancer may cause these same signs and symptoms.

Tests to diagnose and stage oral cavity cancer may include the following:

See the General Information section for a description of these tests and procedures.

Treatment

Treatment of oral cavity cancer in children may include the following:

Salivary Gland Tumors

Salivary gland tumors form in the salivary glands, which are small organs in the mouth and throat that make saliva. Most salivary gland tumors form in the parotid glands (just in front of and below each ear) or in the salivary glands under the tongue or near the jaw.

In children, most salivary gland tumors are benign (noncancer). Some salivary gland tumors are malignant (cancer), especially in young children. Malignant tumors sometimes form after treatment with radiation therapy for leukemia or solid tumors.

Signs and Symptoms

Salivary gland tumors may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

  • A lump (usually painless) near the ear, cheek, jaw, or lip, or inside the mouth.
  • Fluid draining from the ear.
  • Trouble swallowing or opening the mouth widely.
  • Numbness or weakness in the face.
  • Pain in the face that does not go away.

Other conditions that are not salivary gland tumors may cause these same signs and symptoms.

Diagnostic and Staging Tests

Tests to diagnose and stage salivary gland cancer may include the following:

See the General Information section for a description of these tests and procedures.

Prognosis

The prognosis for salivary gland cancer is usually good.

Treatment

Treatment of salivary gland cancer in children is usually surgery to remove the cancer, with or without radiation therapy and chemotherapy.

See the PDQ summary on adult Salivary Gland Cancer Treatment for more information.

Laryngeal Cancer and Papillomatosis

Laryngeal Cancer

Laryngeal cancer is a disease in which malignant (cancer) cells form in the tissues of the larynx. The larynx is also called the voice box. It's the part of the throat that holds the vocal cords and is used in breathing, swallowing, and talking. Rhabdomyosarcoma (a malignant tumor of muscle) is the most common type of laryngeal cancer in children. Squamous cell carcinoma is a less common type of laryngeal cancer in children.

Signs and Symptoms for Laryngeal Cancer

Laryngeal cancer may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

  • Hoarseness or a change in the voice.
  • Trouble or pain when swallowing.
  • A lump in the neck or throat.
  • A sore throat or cough that does not go away.
  • Ear pain.

Other conditions that are not laryngeal cancer may cause these same signs and symptoms.

Diagnostic and Staging Tests for Laryngeal Cancer

Tests to diagnose and stage laryngeal cancer may include the following:

See the General Information section for a description of these tests and procedures.

Other tests used to diagnose laryngeal cancer include the following:

  • Laryngoscopy : A procedure to look at the larynx (voice box) for abnormal areas. A mirror or a laryngoscope (a thin, tube-like instrument with a light and a lens for viewing) is inserted through the mouth to see the larynx. A special tool on the laryngoscope may be used to remove samples of tissue. The tissues samples are viewed under a microscope by a pathologist to check for signs of cancer.

  • Barium swallow : A series of x-rays of the esophagus and stomach. The patient drinks a liquid that contains barium (a silver-white metallic compound). The liquid coats the esophagus and stomach, and x-rays are taken. This procedure is also called an upper GI series.

Treatment of Laryngeal Cancer

Treatment of laryngeal cancer in children may include the following:

See the following PDQ summaries for more information:

Papillomatosis

Papillomatosis of the larynx is a condition that causes papillomas (benign tumors that look like warts) to form in the tissue that lines the larynx. Papillomatosis may be caused by the human papillomavirus (HPV). Papillomas in the larynx may block the airway and cause trouble breathing. These growths often recur (come back) after treatment and may become cancer of the larynx.

Treatment of Papillomatosis

Treatment of papillomatosis in children may include the following:

  • Laser surgery for papillomatosis and other benign tumors.
  • Biologic therapy for papillomas that come back after being removed by surgery four times in one year.
Midline Tract Cancer with NUT Gene Changes (NUT Midline Carcinoma)

Midline tract cancer is a disease in which malignant (cancer) cells form in the respiratory tract and sometimes other places along the middle of the body. The respiratory tract is made up of the nose, throat, larynx, trachea, bronchi, and lungs. Cancer may also form in other places along the middle of the body, such as the thymus, the area between the lungs, the pancreas, liver, and bladder.

Midline tract cancer is caused by a change in a chromosome. Every cell in the body contains DNA (genetic material stored inside chromosomes) that controls how the cell looks and acts. Midline tract cancer may form when part of the DNA from chromosome 15 (called the NUT gene) moves to another chromosome, or when chromosome 15 is broken.

Prognosis

Midline tract cancer with NUT gene changes usually cannot be cured.

Treatment

There is no standard treatment for midline tract cancer with NUT gene changes. Treatment may include surgery to remove the tumor and radiation therapy. Taking part in a clinical trial should be considered.