Atrt Cancer

Overview

ATRT (atypical teratoid rhabdoid tumor) is a rare, fast-growing type of brain tumor. It starts in the central nervous system, which includes the brain and spinal cord. 'Atypical' means the cells look unusual under a microscope. 'Teratoid' and 'rhabdoid' describe the cell shapes. It is a serious condition that needs rapid treatment.

No, ATRT is very rare. It makes up about 1–2% of all childhood brain tumors.

ATRT affects mostly infants and young children, but it can also happen in older children and rarely in adults.

Symptoms

Common symptoms

• Headache that is worse in the morning
• Nausea or vomiting, especially in the morning
• Seizures
• Vision problems or unusual eye movements
• Difficulty with balance or walking
• Facial weakness or drooping
• Changes in behavior or mood

Symptoms in children

• A bulging soft spot on the top of the head
• The head growing faster than expected
• Irritability or crying that is hard to soothe
• Poor feeding or loss of appetite
• Sleepiness or difficulty waking up
• Unusual eye movements, such as eyes turning inward or outward

Symptoms in older adults

• Memory problems
• Personality changes
• Weakness on one side of the body
• Difficulty speaking or finding words
• Trouble with balance or coordination

Causes

Main causes

• Most ATRT tumors are caused by a missing or altered piece of DNA in the tumor cells, specifically in a gene called SMARCB1. This change usually happens randomly, not inherited.

Risk factors

• The main risk factor is young age.
• Some children inherit a changed SMARCB1 gene, which is called rhabdoid tumor predisposition syndrome.
• In rare cases, a family history of this syndrome can increase the risk.

When to see a doctor

See a doctor urgently if:

• If your child has any of the emergency symptoms listed, call for emergency help.
• If your child has new vomiting, headaches, balance problems, or behavior changes, take them to a doctor the same day.

Book a routine appointment if:

• If your child is not meeting developmental milestones, or you have concerns about their head size, vision, or coordination, speak to your GP or pediatrician.

Diagnosis

To diagnose ATRT, a doctor will take a detailed history and examine the child. The main test is an imaging scan of the brain and spine to look for a tumor.

Tests that may be done

• MRI scan of the brain and spine
• CT scan if MRI is not immediately available
• Biopsy (a small sample of the tumor examined under a microscope)
• Spinal tap (lumbar puncture) to check cerebrospinal fluid for tumor cells
• Genetic testing of the tumor tissue

What to expect at your appointment

If a tumor is found, the child will be referred to a specialist pediatric oncology team. The team will explain the findings and plan the next steps. You will not be alone in this process.

Treatment

Treatment for ATRT usually starts quickly after diagnosis. The exact plan depends on the child's age, the size and position of the tumor, and whether it has spread. A team of specialists (neurosurgeons, oncologists, radiologists) will work together.

Self-care at home

• While your child is in treatment, focus on supporting their comfort and nutrition.
• Follow the care team's advice about managing side effects.
• Keep a diary of symptoms to share with the team.

Medical treatments

Medical treatment for ATRT may include one or more of the following: surgery to remove as much of the tumor as possible, chemotherapy (medicines that kill cancer cells), and in some cases radiation therapy (high-energy rays) or targeted therapy. The exact combination is tailored to each child. No single drug is recommended for everyone.

When is surgery considered?

Surgery is usually the first step, if the tumor can be safely reached. The goal is to remove as much tumor as possible without harming healthy brain tissue.

Living with this condition

Living with ATRT means frequent hospital visits, therapy sessions, and regular imaging tests. It helps to build a strong support network of family, friends, and healthcare professionals.

Lifestyle tips

• Keep a regular daily routine as much as possible.
• Ensure the child gets rest and good nutrition.
• Encourage safe physical activity when the care team allows.
• Create a calm space for times of fatigue or recovery.

Diet and exercise

A balanced diet helps support recovery, but treatment can affect appetite. Ask to speak with a pediatric dietitian if your child is eating poorly. Gentle exercise, like short walks or play, can help with mood and strength, but only follow the care team's advice.

Mental health and emotional wellbeing

A cancer diagnosis affects the whole family. Parents and siblings may feel anxious, scared, or overwhelmed. It is normal to have these feelings. Ask your care team about counseling or family support services.

Prevention

ATRT cannot be prevented. The gene change that causes it usually happens by chance before birth. For families with an inherited gene change, genetic counseling can help discuss risks and testing.

Vaccines

There is no vaccine to prevent ATRT. Children with cancer should stay up to date with general vaccines, but only as recommended by their treating team.

Screening programmes

There is no routine screening test for ATRT in the general population. If a family has a known inherited risk, specialist genetic counseling may consider monitoring.

Complications

If left untreated

• If ATRT is not treated, the tumor will continue to grow and can put pressure on the brain, leading to serious damage or death.
• It may also spread to the spinal fluid and other parts of the central nervous system.

Long-term outlook

ATRT is an aggressive cancer, but some children can be cured with intensive treatment. The outlook depends on the child's age, the amount of tumor removed, and whether the tumor has spread. Doctors and researchers continue to improve treatments and offer hope to families.

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