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Unusual Cancers of Childhood

Unusual Cancers of the Head and Neck

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, 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:

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

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

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:

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 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:

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:

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). 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:

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:

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:

Prognosis

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

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 Cancer

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:

Oral cancer may cause any of the following signs and symptoms. Check with you doctor if any of the following problems occur:

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

Treatment

Treatment of oral cancer in children may include the following:

See the following PDQ summaries for more information:

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). 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:

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:

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.

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:

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:

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

Other tests used to diagnose laryngeal cancer include the following:

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:

Midline Tract Cancer with <GeneName>NUT</GeneName> Gene Changes

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 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. Taking part in a clinical trial should be considered.