Patients may be diagnosed in childhood or adulthood. Treatment options for atypical teratoid/rhamboid tumors vary, with survival times ranging from months to years. About 30% of affected patients live for five years or more.
Atypical teratoid/rhabdoid tumors often result from a gene defect or change in a specific cell type. They are uncommon in children, making them difficult to diagnose. The World Health Organization classifies atypical teratoid/rhomboids as embryonal grade IV neoplasms. They are made up primarily of primitive neuroectodermal cells, but can include glial cells and mesenchymal cells.
Atypical teratoid/rhabdoid tumors are associated with genetic changes. This genetic change may increase the risk of developing this cancer. However, this does not mean that the patient will develop the disease. People with certain risk factors should talk with their doctor to find out more about possible risks. These factors may increase the chance of developing the disease, but do not necessarily increase the likelihood of developing the disorder.
Atypical teratoid/rhabdoid tumors can develop at any time. Early diagnosis is important to avoid the spread of the disease to other parts of the body. Fortunately, early detection and treatment are possible. The symptoms of atypical teratoid/rhomboidal tumor include the following: (1) anemia, dysplasia, and diabetes.
Recurrent AT/RTs may occur in the central nervous system. Some of these patients may experience a recurrent tumor, which causes symptoms that include a severe headache. The signs of AT/RT may differ from patient to patient. Some of these symptoms include: The tumors may be in two locations or reoccurring. If they are located in the central nervous system, they may cause asymmetrical bleeding.
Atypical teratoid/Rhabdoid tumors usually develop due to a mutation in a specific gene. This mutation is rare and occurs in only 10% of children with brain tumors. Atypical teratoid/rhabdoid tumors occur in different parts of the body. They usually occur in the cerebellum and brain stem. Ninety percent of AT/RTs are caused by a genetic mutation in the INI1 gene.
Some patients have a family history of the disease. Atypical teratoid rhabdoid tumors are usually detected with a blood test. A physical exam will check for signs of disease, such as a lump or unusual symptom. A neurological examination will check for nerve function, mental status, and balance. A person may also have trouble walking or moving.
The symptoms of an atypical teratoid/rhabdoid tumor vary by individual. Many children with this disease have no symptoms at all. Some of the common symptoms may include generalized acromegaly, arthralgiophysis, and asymmetry. The atypical teratoid rhabdoid tumor has several characteristics that are similar to other types of brain tumors.
Atypical Teratoid/Rhaddoid tumors are aggressive, and can affect the cerebellum and spinal cord. They can spread to other parts of the brain. The signs of an ATRT are different for every patient. The signs of an ATRT may include a headache, loss of coordination, and trouble walking. A family history of the disease is essential for early detection.
