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Atypical teratoid rhabdoid tumors ATRT

Every year, approximately 3 children in the Netherlands are diagnosed with an atypical teratoid rhabdoid tumor (ATRT). Most of those children are not yet 5 years old. An ATRT is a malignant tumor that tends to spread and to recur after treatment. An ATRT usually occurs in the brain, but can also develop in other parts of the body, such as in the neck or in the abdomen near the kidneys.

Causes

Usually, a piece of DNA on chromosome 22 is missing in the tumor cells. This causes cells to divide uncontrollably and form a tumor. The cause of this error is unknown.

Symptoms

Most children suffer from headaches, nausea and vomiting. This is due to increased pressure in the brain: because the tumor continues to grow, there is less room and the cerebrospinal fluid cannot drain away. Other symptoms include abnormal eye movement, impaired vision, poor walking ability, stumbling, inability to sit, slow speech and sometimes epilepsy. In very young children the skull is not yet closed and the excess cerebrospinal fluid accelerates head growth. They are less affected by increased pressure, but their development often stagnates.

How are atypical teratoid rhabdoid tumors diagnosed?

An MRI scan is performed and, if safe to do so, cerebrospinal fluid is drawn under sedation. The neurosurgeon then strives to surgically remove the entire tumor. If that is not possible, the neurosurgeon removes only a piece of tumor tissue. The pathologist examines the tumor tissue under the microscope. Only then is a final diagnosis made.

Treatment

Children with ATRT are treated according to a protocol drawn up by national and international experts.
The (neuro)surgeon strives to surgically remove the entire tumor. This is followed by intensive chemotherapy treatment. Part of the chemotherapy is administered directly into ventricles of the brain via an ommaya. A second operation might be required if a tumor remnant is detected by means of an MRI after chemotherapy.
The treatment is usually concluded with radiotherapy. Very young children are not given radiotherapy, but usually receive a high dose of chemotherapy followed by high-dose chemotherapy with autologous stem cell transplantation.

Survival rate

It is difficult to get an ATRT under control. About 60% of children over 3 years old whose tumor was completely removed, who had no metastases and who received chemotherapy and radiotherapy, are still alive five years later. The survival rate of the other children is much lower. We hope research will improve this percentage in the coming years.

Because many children suffer effects of the treatment, they are monitored for a long time by a team of specialists: pediatric neurologist, pediatric oncologist, pediatric endocrinologist, rehabilitation doctor, as well as remedial educationalist, neuropsychologist, physiotherapist and speech therapist.

Questions?

Children and adolescents with ATRT are treated in the neuro-oncology department.