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Atypical teratoid rhabdoid tumors ATRT

An ATRT is a grade IV malignant tumor with a tendency to spread and recur after treatment. The tumor is more common at a young age. Some of the children have an underlying abnormality in the DNA of the body cells (on chromosome 22 with SMARC-B1 mutation).
Symptoms connected to this disease
Signs of high brain pressure, such as headaches and vomiting, often figure in this instance. Other complaints include changed eye movement, reduced vision, difficulty walking and occasional epileptic seizures. Rapid skull growth may occur in young children because their skull is not yet fully developed.

How do we make the diagnosis?
An MRI scan of the brain detects the occurrence of a tumor, which is usually located in the cerebrum (the great brain) and sometimes in the cerebellum (the little brain). The diagnosis is only certain after the tumor is examined by means of a biopsy or through surgery (neurosurgical removal). In addition, the patient's spinal fluid is examined and an MRI scan of the spinal cord is done to determine whether there are any signs of metastases.

ATRT treatment
The aim is to surgically remove the whole tumor, however that is not possible in some instances and there might also be remnants in the form of metastases. The follow-up treatment consists of chemotherapy. After assessing the effect of the chemotherapy, a determination can be made as to whether any remnants can still be removed. The type of treatment differs based on the location of the tumor, any tumor remnants and the patient's age. Radiotherapy is usually the last step in the treatment; however, if the patient is a young child, long-term chemotherapy is often required to suppress the tumor cells until their brain can be subjected to radiotherapy.