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Breast Cancer, Childhood
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 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 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 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 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 for more information.)
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