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According to the National Cancer Institute, the 5-year survival rate for an atypical teratoid rhabdoid tumor (ATRT) is 32.2%, although survival rates vary

According to the National Cancer Institute, the 5-year survival rate for an atypical teratoid rhabdoid tumor (ATRT) is 32.2%. Survival rates depend on many factors, however, including:

• Tumor grade and type
• Characteristics of cancer
• Age and comorbidities
• Response to treatment
• Treatment tolerance

ATRT is a rapidly progressing tumor, with most deaths occurring in the first 12 months after onset of symptoms. It can spread to other areas of the body, including:

• Lungs
• Abdomen
• Liver
• Brain (less common)

Older children generally have better survival rates than younger children. With current advances in treatment, survival rates have been improving. Survival rates are higher if the tumor is:

• Treated before it spreads
• Completely removed surgically

Clinical trials for new treatments aim to target specific cancerous cells while sparing healthy cells, thereby improving survival rates.

What is ATRT?

ATRT is a rare and rapidly growing tumor that originates in the brain and spinal cord. It is an aggressive form of cancer that affects the cerebellum and brainstem.

The leading cause of ATRT seems to be a mutation in the SMARCB1 or SMARCA4 genes. These genes are responsible for producing a protein that regulates cell growth. In ATRT, these genes malfunction and result in uncontrolled tumor growth.

ATRT can form anywhere in the central nervous system (CNS), particularly in the brain, and spread to the spinal cord. In rare cases, ATRT can form outside the CNS, especially in the kidneys.

Who is at risk of ATRT?

ATRT primarily occurs in children younger than 3 years old but can occur in older children and rarely in adults:

• ATRTs are the most common cancerous brain tumors in infants under 1 year of age.
• Most diagnoses are in infants and toddlers between 1-2 years old.
• ATRT represents about 1%-2% of all pediatric brain tumors in children up to 16 years of age.
• Adults are very rarely diagnosed with ATRT. For example, if 58 people are diagnosed with ATRT globally per year, only 2 adults older than 16 years are likely to have it.

What are the symptoms of ATRT?

Symptoms of atypical teratoid rhabdoid tumors may depend on the location of the tumor and the person’s age. Possible symptoms of ATRT may include:

• Morning headaches
• Loss of balance
• Changes in activity levels
• Vomiting
• Increase in head size (in infants)
• Sleepiness
• Asymmetric eye movements or face movements

ATRT can spread from the brain to other parts through the cerebrospinal fluid. Therefore, symptoms may progress rapidly within a short period.

How is ATRT treated?

The primary treatment of ATRT is surgery. The resected tissue is biopsied to identify the tumor type. For the surgery to be maximally effective, the maximum portion of the tumor and surrounding tissue is resected. 

Apart from surgery, other treatment choices include:

• Radiation therapy: Uses high-energy rays to kill cancer cells
• Chemotherapy: Uses special medications to kill or shrink cancer cells
• Clinical trials: Available with new chemotherapeutic molecules, targeted therapy, or immunotherapy drugs

Physicians may determine the treatment of ATRT depending on the following factors:

• Age
• Remaining tumor after surgery
• Tumor type
• Tumor location