Breast Cancer
Bronchial Tumors
Pleuropulmonary Blastoma
Esophageal Tumors
Thymomas and Thymic Carcinoma
Tumors of the Heart
Mesothelioma

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 cancers are rare in patients younger than 15 years of age.

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 for children and adolescents with breast cancer include surgery and radiation therapy. 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).(See the PDQ summary on adult Breast Cancer Treatment ¹ for more information.)

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 blastomas are rare tumors that usually occur under the tissue covering the lungs. Changes in a specific chromosome may be found in pleuropulmonary blastoma. Every cell in the body contains DNA (genetic material stored inside chromosomes) that determines how the cell looks and acts. Part of the DNA in chromosome 8 is often abnormal in these tumors. Early-stage pleuropulmonary blastoma develops as a cyst -like tumor in the lung. It usually appears in the first 2 years of life and has a good prognosis. Late-stage tumors are solid and can spread to the brain. 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. There are no standard treatment options for pleuropulmonary blastoma. Early-stage tumors may be treated with surgery alone, or surgery with adjuvant chemotherapy to help prevent the tumor from coming back. Late-stage tumors may be treated with surgery followed by chemotherapy. 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. Pleuropulmonary blastoma may also occur in siblings of patients.

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. (See the PDQ summary on adult Esophageal Cancer Treatment ² for more information.)

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, Isaacs syndrome or neuromyotonia (a rare nerve disorder that causes constant muscle stiffness and cramping), 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. (See the PDQ summary on adult Thymoma and Thymic Carcinoma Treatment ³ for more information.)

The most common tumors that begin in the heart are benign. In children, these include rhabdomyomas (tumors of muscle tissue) and fibromas (tumors of fibrous tissue). Myxomas are rare, benign tumors which may be part of an inherited syndrome called Carney complex (see Multiple Endocrine Neoplasia Syndromes (MEN) and Carney Complex ⁴ section below for more information). Other benign tumors include histiocytoid cardiomyopathy tumors, teratomas, hemangiomas, and neurofibromas. Malignant tumors that begin in the heart are rare, but may include malignant teratomas, rhabdomyosarcomas, and chondrosarcomas.

Patients may have no symptoms for long periods of time. Symptoms include abnormal heart rhythm, enlargement of the heart, fluid in the sac around the heart, and congestive heart failure. Sudden death with no early symptoms is possible. Successful treatment may require surgery (which may include transplantation) and chemotherapy. Benign tumors of heart muscle (rhabdomyomas) usually shrink and go away on their own.

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. (See the PDQ summary on adult Malignant Mesothelioma Treatment ⁵ for more information.)

Glossary Terms

Having to do with the abdomen, which is the part of the body between the chest and the hips that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.

Not normal. An abnormal lesion or growth may be cancer, premalignant (likely to become cancer), or benign (not cancer).

Additional cancer treatment given after the primary treatment to lower the risk that the cancer will come back. Adjuvant therapy may include chemotherapy, radiation therapy, hormone therapy, targeted therapy, or biological therapy.

A quickly growing cancer.

A disease that causes inflammation and pain in the joints.

A group of minerals that take the form of tiny fibers. Asbestos has been used as insulation against heat and fire in buildings. Loose asbestos fibers breathed into the lungs can cause several serious diseases, including lung cancer and malignant mesothelioma (cancer found in the lining of the lungs, chest, or abdomen). Asbestos that is swallowed may cause cancer of the gastrointestinal tract.

A chronic disease in which the bronchial airways in the lungs become narrowed and swollen, making it difficult to breathe. Symptoms include wheezing, coughing, tightness in the chest, shortness of breath, and rapid breathing. An attack may be brought on by pet hair, dust, smoke, pollen, mold, exercise, cold air, or stress.

Not cancerous. Benign tumors may grow larger but do not spread to other parts of the body. Also called nonmalignant.

The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.

The organ that stores urine.

Glandular organ located on the chest. The breast is made up of connective tissue, fat, and breast tissue that contains the glands that can make milk. Also called mammary gland.

Cancer that forms in tissues of the breast, usually the ducts (tubes that carry milk to the nipple) and lobules (glands that make milk). It occurs in both men and women, although male breast cancer is rare.

The large air passages that lead from the trachea (windpipe) to the lungs.

Having to do with the bronchi, which are the larger air passages of the lungs, including those that lead from the trachea (windpipe) to the lungs and those within the lungs.

Cancer that forms in tissues of the bronchi (large air passages in the lungs including those that lead to the lungs from the windpipe).

A term for diseases in which abnormal cells divide without control and can invade nearby tissues. Cancer cells can also spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is a cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is a cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is a cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord. Also called malignancy.

A slow-growing type of tumor usually found in the gastrointestinal system (most often in the appendix), and sometimes in the lungs or other sites. Carcinoid tumors may spread to the liver or other sites in the body, and they may secrete substances such as serotonin or prostaglandins, causing carcinoid syndrome.

Cancer that begins in the skin or in tissues that line or cover internal organs.

A rare, inherited disorder marked by dark spots on the skin and tumors in the heart, endocrine glands, skin, and nerves. There are two types of Carney complex, which are caused by mutations (changes) in different genes. Also called Carney syndrome.

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

Treatment with drugs that kill cancer cells.

An x-ray of the structures inside the chest. An x-ray is a type of high-energy radiation that can go through the body and onto film, making pictures of areas inside the chest, which can be used to diagnose disease.

A type of cancer that forms in bone cartilage. It usually starts in the pelvis (between the hip bones), the shoulder, the ribs, or at the ends of the long bones of the arms and legs. A rare type of chondrosarcoma called extraskeletal chondrosarcoma does not form in bone cartilage. Instead, it forms in the soft tissues of the upper part of the arms and legs. Chondrosarcoma can occur at any age but is more common in people older than 40 years. It is a type of bone cancer.

Part of a cell that contains genetic information. Except for sperm and eggs, all human cells contain 46 chromosomes.

Weakness of the heart muscle that leads to a buildup of fluid in body tissues.

To heal or restore health; a treatment to restore health.

A sac or capsule in the body. It may be filled with fluid or other material.

The process of identifying a disease, such as cancer, from its signs and symptoms.

In medicine, a disturbance of normal functioning of the mind or body. Disorders may be caused by genetic factors, disease, or trauma.

The molecules inside cells that carry genetic information and pass it from one generation to the next. Also called deoxyribonucleic acid.

A term used to describe cancer that is early in its growth, and may not have spread to other parts of the body. What is called early stage may differ between cancer types.

Refers to the cells that line the internal and external surfaces of the body.

Cancer that forms in tissues lining the esophagus (the muscular tube through which food passes from the throat to the stomach). Two types of esophageal cancer are squamous cell carcinoma (cancer that begins in flat cells lining the esophagus) and adenocarcinoma (cancer that begins in cells that make and release mucus and other fluids).

The muscular tube through which food passes from the throat to the stomach.

A record of the relationships among family members along with their medical histories. This includes current and past illnesses. A family history may show a pattern of certain diseases in a family. Also called family medical history.

Containing or resembling fibers.

A substance that flows smoothly and takes the shape of its container. Liquids and gases are fluids.

Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.

A type of tumor that begins in the cells that give rise to sperm or eggs. Germ cell tumors can occur almost anywhere in the body and can be either benign or malignant.

An organ that makes one or more substances, such as hormones, digestive juices, sweat, tears, saliva, or milk. Endocrine glands release the substances directly into the bloodstream. Exocrine glands release the substances into a duct or opening to the inside or outside of the body.

A cancer of the immune system that is marked by the presence of a type of cell called the Reed-Sternberg cell. The two major types of Hodgkin lymphoma are classical Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma. Symptoms include the painless enlargement of lymph nodes, spleen, or other immune tissue. Other symptoms include fever, weight loss, fatigue, or night sweats. Also called Hodgkin disease.

One of many chemicals made by glands in the body. Hormones circulate in the bloodstream and control the actions of certain cells or organs. Some hormones can also be made in the laboratory.

The cause of acquired immunodeficiency syndrome (AIDS). Also called HIV.

Too much thyroid hormone. Symptoms include weight loss, chest pain, cramps, diarrhea, and nervousness. Also called overactive thyroid.

Transmitted through genes that have been passed from parents to their offspring (children).

Cancer that has spread beyond the layer of tissue in which it developed and is growing into surrounding, healthy tissues. Also called infiltrating cancer.

A term used to describe cancer that is far along in its growth, and has spread to the lymph nodes or other places in the body.

Cancer that starts in blood-forming tissue such as the bone marrow and causes large numbers of blood cells to be produced and enter the bloodstream.

One of a pair of organs in the chest that supplies the body with oxygen, and removes carbon dioxide from the body.

A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph nodes filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph gland.

A thin tube that carries lymph (lymphatic fluid) and white blood cells through the lymphatic system. Also called lymphatic vessel.

The tissues and organs that produce, store, and carry white blood cells that fight infections and other diseases. This system includes the bone marrow, spleen, thymus, lymph nodes, and lymphatic vessels (a network of thin tubes that carry lymph and white blood cells). Lymphatic vessels branch, like blood vessels, into all the tissues of the body.

Cancer that begins in cells of the immune system. There are two basic categories of lymphomas. One kind is Hodgkin lymphoma, which is marked by the presence of a type of cell called the Reed-Sternberg cell. The other category is non-Hodgkin lymphomas, which includes a large, diverse group of cancers of immune system cells. Non-Hodgkin lymphomas can be further divided into cancers that have an indolent (slow-growing) course and those that have an aggressive (fast-growing) course. These subtypes behave and respond to treatment differently. Both Hodgkin and non-Hodgkin lymphomas can occur in children and adults, and prognosis and treatment depend on the stage and the type of cancer.

Cancerous. Malignant tumors can invade and destroy nearby tissue and spread to other parts of the body.

An x-ray of the breast.

Surgery to remove the breast (or as much of the breast tissue as possible).

A benign (not cancer) or malignant (cancer) tumor affecting the lining of the chest or abdomen. Exposure to asbestos particles in the air increases the risk of developing malignant mesothelioma.

The spread of cancer from one part of the body to another. A tumor formed by cells that have spread is called a “metastatic tumor” or a “metastasis.” The metastatic tumor contains cells that are like those in the original (primary) tumor. The plural form of metastasis is metastases (meh-TAS-tuh-SEEZ).

A bundle of fibers that receives and sends messages between the body and the brain. The messages are sent by chemical and electrical changes in the cells that make up the nerves.

A benign tumor that develops from the cells and tissues that cover nerves.

A part of the body that performs a specific function. For example, the heart is an organ.

PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.

Having to do with children.

A type of tumor found in breast or prostate tissue. It is often large and bulky and grows quickly. It may be benign (not cancer) or malignant (cancer) and may spread to other parts of the body. Also called CSP and cystosarcoma phyllodes.

A rare and very aggressive (fast-growing) cancer that forms in tissues of the lung and pleura (a thin layer of tissue that covers the lungs and lines the interior wall of the chest cavity). Pleuropulmonary blastoma is most common in children.

The likely outcome or course of a disease; the chance of recovery or recurrence.

The use of high-energy radiation from x-rays, gamma rays, neutrons, protons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body near cancer cells (internal radiation therapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that travels in the blood to tissues throughout the body. Also called irradiation and radiotherapy.

To come back or to return.

Cancer that forms in the soft tissues in a type of muscle called striated muscle. Rhabdomyosarcoma can occur anywhere in the body.

A cancer of the bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue.

A person’s brother or sister who has the same parents.

The male reproductive cell, formed in the testicle. A sperm unites with an egg to form an embryo.

The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.

In medicine, treatment that experts agree is appropriate, accepted, and widely used. Health care providers are obligated to provide patients with standard therapy. Also called best practice and standard of care.

A type of drug used to relieve swelling and inflammation. Some steroid drugs may also have antitumor effects.

An organ that is part of the digestive system. The stomach helps digest food by mixing it with digestive juices and churning it into a thin liquid.

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

The percentage of people in a study or treatment group who are alive for a certain period of time after they were diagnosed with or treated for a disease, such as cancer. The survival rate is often stated as a five-year survival rate, which is the percentage of people in a study or treatment group who are alive five years after diagnosis or treatment. Also called overall survival rate.

An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.

A set of symptoms or conditions that occur together and suggest the presence of a certain disease or an increased chance of developing the disease.

A chronic, inflammatory, connective tissue disease that can affect the joints and many organs, including the skin, heart, lungs, kidneys, and nervous system. It can cause many different symptoms; however, not everyone with systemic lupus erythematosus has all of the symptoms. Also called lupus and SLE.

A type of germ cell tumor that may contain several different types of tissue, such as hair, muscle, and bone. Teratomas occur most often in the ovaries in women, the testicles in men, and the tailbone in children. Not all teratomas are malignant.

Having to do with the chest.

The hollow tube inside the neck that starts behind the nose and ends at the top of the trachea (windpipe) and esophagus (the tube that goes to the stomach). The throat is about 5 inches long, depending on body size. Also called pharynx.

A rare type of thymus gland cancer. It usually spreads, has a high risk of recurrence, and has a poor survival rate. Thymic carcinoma is divided into subtypes, depending on the types of cells in which the cancer began. Also called type C thymoma.

A tumor of the thymus, an organ that is part of the lymphatic system and is located in the chest, behind the breastbone.

An organ that is part of the lymphatic system, in which T lymphocytes grow and multiply. The thymus is in the chest behind the breastbone.

A group or layer of cells that work together to perform a specific function.

The airway that leads from the larynx (voice box) to the bronchi (large airways that lead to the lungs). Also called windpipe.

A surgical procedure in which tissue or an organ is transferred from one area of a person’s body to another area, or from one person (the donor) to another person (the recipient).

An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancer), or malignant (cancer). Also called neoplasm.