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Unusual Cancers of Childhood
Unusual Cancers of the Head and Neck
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, Symptoms, and Diagnostic and Staging Tests
The risk of nasopharyngeal cancer is 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 doctor if any of the following problems occur:
- Painless lumps in the neck.
- Snoring.
- Nosebleeds.
- Hearing loss.
- Problems moving the jaw.
- Double vision.
Other conditions that are not nasopharyngeal cancer may cause these same 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 that examine the nasal cavity and throat are used to diagnose and stage nasopharyngeal cancer. They may include:
See the
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) test: A blood test to check for antibodies to the Epstein-Barr virus. Antibodies to EBV 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:
- Radiation therapy.
- Chemotherapy given before and at the same time as radiation therapy.
- A clinical trial of radiation therapy and a drug to prevent radiation therapy side effects. These may be used with or without chemotherapy given before and at the same time as the radiation therapy.
Young patients are more likely than adults to have problems caused by treatment, including second cancers.
See the PDQ summary on adult
Esthesioneuroblastoma (olfactory neuroblastoma) is a very rare 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.
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.
Symptoms
Esthesioneuroblastoma may cause any of the following signs and symptoms. Check with your doctor if any of the following problems occur:
- Blocked nose.
- Loss of the sense of smell.
- Frequent sinus infections.
- Nosebleeds.
Other conditions that are not esthesioneuroblastoma may cause these same symptoms.
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:
- Surgery and radiation therapy. Newer treatments include sinus surgery done through an endoscope, radiosurgery, or proton beam radiation therapy.
- Chemotherapy and/or radiation therapy followed by surgery.
- Surgery followed by chemotherapy.
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). Adenomas can grow very large and sometimes make hormones. Adenomas may become malignant (cancer) and spread to the lungs or lymph nodes in the neck. Thyroid cancer usually grows and spreads slowly.
Risk Factors, 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.
Thyroid tumors may cause any of the following symptoms. Check with your doctor if any of the following problems occur:
- A lump in the neck.
- Trouble breathing.
- Trouble swallowing.
- Hoarseness or a change in the voice.
Other conditions that are not thyroid tumors may cause these same symptoms.
Tests that examine the thyroid are used to diagnose and stage thyroid tumors. They may include:
- Physical exam and history.
- Fine-needle aspiration (FNA) biopsy.
- Open biopsy or surgery to remove all or part of the thyroid.
See the
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:
- Where cancer is found in the body when it is diagnosed.
- The size of the tumor.
Treatment
Treatment of thyroid tumors in children may include the following:
- Surgery to remove most or all of the thyroid gland and lymph nodes with cancer, followed by radioactive iodine (RAI) to kill any thyroid cancer cells that are left. Hormone replacement therapy (HRT) is given to make up for the lost thyroid hormone.
- Surgery to remove the lobe in which thyroid cancer is found, followed by HRT to make up for the lost thyroid hormone.
- Radioactive iodine (RAI) for cancer that has recurred (come back).
- Targeted therapy with tyrosine kinase inhibitors (TKIs) or vascular endothelial growth factor inhibitors (VEGFs) for cancer that has spread to other parts of the body or that has recurred.
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
Oral cancer is a disease in which malignant (cancer) cells form in the tissues of the oral cavity. Most tumors in the oral cavity are benign (not cancer). The most common type of oral cancer in adults, squamous cell carcinoma (cancer of the thin, flat cells lining the mouth), is very rare in children. However, the number of new cases of oral cancer in teenage girls and young women has increased since the mid-1990s. Malignant tumors in children include lymphomas and sarcomas.
Risk Factors, Symptoms, and Diagnostic and Staging Tests
The risk of oral cancer is increased by the following:
- Tobacco use: Using any tobacco product increases the risk of oral cancer. Use of smokeless tobacco may cause mouth cancer. Changes in the texture, color, and shape of tissue inside the mouth have been seen in more than half of all teenagers who use smokeless tobacco.
- Previous radiation therapy: Oral cancer is more likely in people who have had other childhood tumors and were treated with radiation therapy to the oral cavity.
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Having certain diseases or conditions, such as:
- Fanconi anemia.
- Dyskeratosis congenita (a rare bone marrow disorder that affects red blood cells, white blood cells, and platelets).
- A mutation in connexin genes (changes the way proteins that connect cells are made).
- Chronic graft-versus-host disease (GVHD).
- Epidermolysis bullosa (an illness that causes the skin to be easily injured and causes painful blisters).
- Xeroderma pigmentosum.
- Human papillomavirus (HPV) infection.
Oral cancer may cause any of the following signs and symptoms. Check with you doctor if any of the following problems occur:
- 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, tonsils, or lining of the mouth.
- Bleeding, pain, or numbness in the mouth.
Other conditions that are not oral cancer may cause these same symptoms.
Tests that examine the mouth are used to diagnose and stage oral cancer. They may include:
See the
Treatment
Treatment of oral cancer in children may include the following:
- Surgery for most benign tumors.
- Surgery, chemotherapy, and radiation therapy for malignant tumors.
See the following PDQ summaries for more information:
- Oropharyngeal Cancer Treatment
- Lip and Oral Cavity Cancer Treatment
- Langerhans Cell Histiocytosis Treatment
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). Malignant (cancer) salivary gland tumors are rare. Malignant tumors sometimes form after treatment with radiation therapy for leukemia or solid tumors.
Symptoms and Diagnostic and Staging Tests
Salivary gland tumors may cause any of the following signs and symptoms. Check with your doctor if any of the following problems occur:
- 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 symptoms.
Tests that examine the mouth are used to diagnose and stage salivary gland cancer. They may include:
- Physical exam and history.
- MRI of the head and neck.
- CT scan.
- PET scan.
- Ultrasound.
- Endoscopy.
- Fine-needle aspiration (FNA) biopsy.
See the
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
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.
Symptoms and Diagnostic and Staging Tests for Laryngeal Cancer
Laryngeal cancer may cause any of the following signs and symptoms. Check with your doctor if any of the following problems occur:
- 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 symptoms.
Tests that examine the throat and larynx are used to diagnose and stage laryngeal cancer. They may include:
- Physical exam and history.
- MRI of the head and neck.
- CT scan.
- Ultrasound.
- Endoscopy.
- Fine-needle aspiration (FNA) biopsy.
See the
Other tests used to diagnose laryngeal cancer include the following:
- Laryngoscopy: A procedure in which the doctor examines the larynx (voice box) with a mirror or with a laryngoscope (a thin, lighted tube).
- 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:
- Chemotherapy and radiation therapy after a biopsy, for rhabdomyosarcomas.
- Laser surgery and radiation therapy for squamous cell cancer. Laser surgery uses a laser beam (a narrow beam of intense light) to turn the cancer cells into a gas that evaporates (dissolves into the air).
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 keep come back after being removed by surgery four times in one year.
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 may be 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
Prognosis
Midline tract cancer with
Treatment
There is no standard treatment for midline tract cancer with
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