What is AT/RT


The following information is from Dana-Farber Cancer Institute (www.dana-farber.org)

 Central nervous system (CNS) atypical teratoid/rhabdoid tumor(AT/RT) is a very rare, fast-growing tumor of the brain and spinal cord. It usually occurs in children younger than three years of age, although it can occur in older children and adults. About half of these tumors form in the cerebellum or brain stem. The cerebellum is the part of the brain that controls movement, balance, and posture. The brain stem controls breathing, heart rate, and the nerves and muscles used in seeing, hearing, walking, talking, and eating. AT/RT may also be found in other parts of the central nervous system (brain and spinal cord). Atypical teratoid/rhabdoid tumor may be linked to a change in a tumor suppressor gene called INI1. This type of gene makes a protein that helps control cell growth. Changes in the DNA of tumor suppressor genes like INI1 may lead to cancer. Changes in the INI1 gene may be inherited (passed on from parents to offspring). When the INI1 gene change is inherited, tumors may form in two parts of the body at the same time (for example, in the brain and the kidney). If AT/RT is diagnosed, genetic counseling (a discussion with a trained professional about inherited diseases and a possible need for gene testing) may be recommended. Because atypical teratoid/rhabdoid tumor is fast growing, symptoms may develop quickly and progress over a period of days or weeks. Symptoms vary and depend on the age of the patient and where the tumor has formed. These symptoms may be caused by AT/RT or by other conditions. A doctor should be consulted if any of the following problems occur: Morning headache or headache that goes away after vomiting. Nausea and vomiting. Unusual sleepiness or change in activity level. Loss of balance, lack of coordination, or trouble walking. Increase in head size (in infants). There are many different treatment options available which may include any combination of the following: chemotherapy, stem-cell transplant and radiation. 

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