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Table of Contents 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 (02/26/2008)
About PDQ
General Information
The tumors discussed in this summary are many and different, and the discussion
is arranged in descending order from tumors of the head and neck to tumors of
the urinary system and skin. All of these cancers are rare enough that most pediatric hospitals might see fewer than two cases in a year. Most of these
tumors are more frequent in adults with cancer; thus, much of the information
about these tumors may also be sought through sources relevant to adults with
these tumors.
 Back to Top Head and Neck Cancers
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 combined with radiosurgery that targets the tumor.
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.
Back to Top Thoracic Cancers
Thoracic cancers include breast cancer, bronchial adenomas, bronchial carcinoid
tumors, pleuropulmonary blastoma, esophageal tumors, thymomas and thymic carcinomas, tumors of the
heart, and mesothelioma. These thoracic cancers are discussed below.
Breast Cancer
Most tumors that involve the breast during childhood are benign (noncancerous) fibroadenomas that can be watched for change without the need for biopsy. Rarely, such breast tumors will show malignant change with sudden, rapid growth. These are called phyllodes tumors, and require biopsy or surgical removal without mastectomy. Other types of breast cancer have been reported in both males and females younger than 21 years.
There is an increased lifetime risk of breast cancer in patients who were treated for any cancer with radiation therapy to the chest area, including female survivors of Hodgkin lymphoma. Mammograms should start at age 25 or 10 years after radiation therapy to the chest, whichever came last. Treatment options include radiation, chemotherapy, and surgery for children and adolescents with breast cancer. Breast tumors may also occur
as cancers that have spread from other types of cancer such as leukemia, rhabdomyosarcoma, other sarcomas, or lymphoma, particularly in patients infected with the human immunodeficiency virus (HIV).(Refer to the PDQ summary on adult Breast
Cancer Treatment for more information.)
Bronchial Tumors
Bronchial tumors (which are slow-growing cancers in the trachea or large bronchi, the large airways
of the lung), are most often carcinoid tumors in children. Symptoms include cough and spitting up blood, and can sometimes be confused with asthma symptoms like wheezing, resulting in delayed diagnosis. Primary treatment is surgery to remove the tumor and lymph nodes and vessels where cancer may spread. The prognosis for most bronchial tumors in children is excellent, even when the cancer has spread to nearby areas. Rarely, carcinoid tumors develop as aggressive (fast-growing) cancers that are more likely to spread to other areas of the body by the time they are diagnosed. For
bronchial carcinoid tumors, neither chemotherapy nor radiation therapy is
indicated, unless evidence of metastasis (spread of cancer to other areas of
the body) is documented.
Cancers that begin in the epithelial cells that line the surface of the lung are rare, and tend to be advanced when they are diagnosed. The outcome for epithelial cancers of the lung depends on how the cells look under a microscope and the stage of the cancer.
Pleuropulmonary Blastoma
Pleuropulmonary blastomas are rare tumors that usually occur under the tissue covering the lungs. When the tumor can be completely removed by surgery at any time during treatment, the chance of recovery is improved. The tumor may recur or spread, in spite of surgical removal. Responses to chemotherapy have been reported and adjuvant chemotherapy may help prevent the tumor from coming back. Radiation may be used
when the tumor cannot be surgically removed. A family history of cancer in
close relatives has been noted for many young patients affected by this tumor.
Esophageal Tumors
Cancer of the esophagus (the muscular tube through which food passes from the throat to the stomach) is rare in the pediatric age group. Symptoms are
related to difficulty in swallowing, and associated weight loss. Treatment
options for esophageal cancer include either radiation therapy or chemotherapy. Prognosis generally is poor for this cancer, which rarely can be completely
removed by surgery. (Refer to the PDQ summary on adult Esophageal Cancer
Treatment for more information.)
Thymomas and Thymic Carcinoma
A cancer of the thymus (an organ in the chest, behind the breastbone) is not
considered a thymoma (cancer) or a thymic carcinoma unless there are cancerous changes of the epithelial cells that cover the organ. The term thymoma usually describes cancers that do not have obvious changes in the epithelial cells. Thymic carcinoma has clear-cut changes of the epithelial cells. Other tumors that involve the thymus gland include lymphoma (cancer that arises in cells of the lymphatic system)
and germ cell tumors (tumors that begin in cells that give rise to sperm or
eggs); these tumors are not true thymomas or thymic carcinomas.
Thymomas and thymic carcinomas are rare in adults as well as children. Various diseases and syndromes are associated with thymomas, including myasthenia gravis,
polymyositis, systemic lupus erythematosus, rheumatoid arthritis, thyroiditis,
and pure red cell aplasia. Endocrine (hormonal) disorders such as hyperthyroidism, Addison’s disease, and panhypopituitarism can also be
associated with a diagnosis of thymoma or thymic carcinoma.
Cancer of the thymus 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 the thymus or in other places along the midline of the body, including parts of the airway, the area between the lungs, and the bladder. It usually cannot be cured.
Thymomas and thymic carcinomas are usually located in the front part of the chest and are usually
discovered during a routine chest x-ray. Symptoms can include cough,
difficulty with swallowing, tightness of the chest, chest pain, and shortness
of breath, although nonspecific symptoms may occur. These tumors generally are
slow growing but are potentially invasive, with cancer spreading to distant organs or lymph nodes. Surgery is performed with the goal of a complete
removal. Radiation therapy is necessary for patients with invasive thymoma or thymic carcinoma,
whether or not there has been surgery. Chemotherapy is usually reserved for
patients with advanced- stage disease who have not responded to radiation
therapy or steroids. The prognosis for patients with invasive thymoma or thymic carcinoma usually
is poor, although significantly higher survival rates have been reported for
patients with tumors that have not spread to the surrounding areas. (Refer to
the PDQ summary on adult Thymoma and Thymic Carcinoma Treatment for more information.)
Tumors of the Heart
Primary tumors of the heart may include benign (noncancerous) and malignant (cancerous) teratoma (a tumor made up of a mixture of tissues), rhabdomyosarcoma (a tumor of muscle tissue), hemangioma (a usually benign tumor made up of blood vessels), and chondrosarcoma (a type of cancer that forms in cartilage). Symptoms include abnormalities of heart rhythm, enlargement of the
heart, fluid in the pericardial sac, and congestive heart failure. Successful
treatment requires surgery (which may include transplantation) and chemotherapy appropriate for the type of cancer that is present.
Benign tumors of heart muscle (rhabdomyomas) usually shrink and go away on their own.
Mesothelioma
Mesothelioma, an extremely rare cancer in children, can involve the tissue coverings of the lung, the heart, or the abdominal organs. These tumors can spread over the surface of organs, without
invading far into the underlying tissue, and may spread to nearby or distant lymph nodes. Mesothelioma may develop after successful treatment of an earlier cancer, especially after treatment with radiation. In adults, these tumors
have been associated with exposure to asbestos, which was used as building
insulation. The amount of exposure required to develop cancer is unknown, and
there is no information about the risk of children exposed to asbestos. (Refer
to the PDQ summary on adult Malignant Mesothelioma Treatment for more
information.)
Back to Top Abdominal Cancers
Abdominal cancers include cancer of the adrenal cortex, stomach
cancer, cancer of the pancreas, colorectal cancer, carcinoid tumors of the lung or intestine, and gastrointestinal stromal cell tumors. These abdominal
cancers are discussed below.
(Refer to the PDQ summary on Wilms Tumor and Other Childhood Kidney Tumors for information about childhood renal cell carcinoma.)
Cancer of the Adrenal Cortex
The adrenal cortex is the outside layer of the adrenal glands. The adrenal glands are a pair of organs near the front side edge of the kidney; their
function is to produce hormones such as glucocorticoid and epinephrine. Cancers in this area are classified as carcinomas and adenomas. Adenomas are
generally benign, whereas adrenocortical carcinomas frequently secrete hormones and may cause the patient to develop masculine traits, regardless of the
patient’s gender. Pediatric patients with adrenocortical carcinoma often have Li-Fraumeni syndrome, an inherited condition that predisposes family members to
multiple cancers, including breast cancer, rhabdomyosarcoma, and osteosarcoma (cancer of the bone).
These tumors can involve the kidneys, lungs, bones and brain. Surgical removal
should be attempted but may not always be possible if the tumor has spread
widely. Additional treatment may include the use of an artificial hormone that
blocks the masculinizing effects of the tumor. The prognosis is generally
excellent for patients who have small tumors that have been completely
removed by surgery, but prognosis can be poor for patients who have large primary tumors or metastatic disease (disease that has spread to other parts of
the body) at diagnosis. Tumor stage is an important factor affecting the chance of recovery for children with adrenocortical tumors. When possible, repeat surgery should be done for tumors that come back and for tumors that spread to the inferior vena cava (a large vein that empties into the heart). (Refer to the PDQ summary on adult Adrenocortical
Carcinoma Treatment for more information.)
Cancer of the Stomach
The frequency of, and death rate from, stomach cancer has declined worldwide
over the past 50 years with the introduction of food preservation practices
such as refrigeration. Symptoms of stomach cancer include vague upper abdominal pain, which can be associated with poor appetite, and weight loss.
Many individuals become anemic but otherwise show no symptoms before the
development of metastatic spread. Other symptoms may include nausea, |
