Atypical Teratoid Rhabdoid Tumor (ATRT) is a rare, high-grade tumor that occurs most often in children aged 3 and younger. It is a fast-growing cancerous tumor of the brain and spinal cord that is caused by changes in the DNA of tumor suppressor genes like SMARCB1 or SMARCA4, which may lead to cancer. ATRTs are all classified as grade 4 tumors, which means they are malignant (cancerous) and fast-growing. The relative 5-year survival rate for ATRTs is 32.2%, but many factors can affect prognosis, including the tumor grade and type, traits of the cancer, the person’s age and health when diagnosed, and how they respond to treatment.

The first treatment for an ATRT is surgery, if possible. The goal of surgery is to obtain tissue to determine the tumor type and to remove as much tumor as possible without causing more symptoms for the person. People with ATRTs usually receive further treatments after surgery, which may include radiation, chemotherapy, or clinical trials. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. External radiation therapy may be given to the brain and spinal cord. Because of the aggressive nature of these tumors, most patients receive several types of treatment, which may include surgery, chemotherapy, and radiation. Clinical trials provide children with recurrent ATRT access to the latest experimental therapies. ATRT is challenging to cure, and the outcomes remain poor. However, treatment advances are being made, and current advances in therapy have helped older children, and children with tumors that can be completely removed have an improved survival.