Atypical Teratoid / Rhabdoid Tumor (AT/RT)

About Atypical Teratoid/Rhabdoid Tumor

Atypical teratoid/rhabdoid tumors are a rare form of tumor that usually develop in children aged three years or younger, though they can develop at any age, and typically affect the brain or spinal cord.

Most atypical teratoid/rhabdoid tumors occur in two areas of the brain: the brainstem or the cerebellum. These are areas at the back of the skull, and each plays an important role in functions of the body that are occurring at all times, even though you may not necessarily think about it.

The cerebellum is a processing center of the brain responsible for controlling things like balance, posture and coordination of movements that happen together, such as when we walk or speak. The brainstem is responsible for things like breathing, your heart rate and swallowing. It is also an important area of the body because it contains the nerves that go to the head and neck, which are vital for things like vision, hearing, speaking and eating.

Symptoms of Atypical Teratoid/Rhabdoid Tumors

The back of the skull, called the posterior fossa, houses an area of the brain that, among other things, is related to coordination of movement. The symptoms seen with atypical teratoid/rhabdoid tumors could be related to compression of that area of the brain, or they could be due to compression and/or destruction of parts of the cerebellum or brainstem, if that is where the tumor is located.

Oftentimes, because of the rapid-growing nature of an atypical teratoid/rhabdoid tumor, the symptoms come on quickly and severely. If your child has an atypical teratoid/rhabdoid tumor, he or she may have the following symptoms:

  • Headaches, particularly headaches that are worse upon waking
  • Nausea and/or vomiting, as well as headaches that go away after vomiting
  • Unusual eye movements
  • Difficulty with balance, walking or coordination of movements
  • Excessive sleepiness
  • In infants, there may be an increase in head size

If the tumor is located in another area of the brain, the symptoms may be different. Because they can occur anywhere within the central nervous system, your child may have different symptoms. However, because the majority of atypical teratoid/rhabdoid tumors do occur within the brainstem or cerebellum, the symptoms above are the most commonly observed.

Treatments for Atypical Teratoid/Rhabdoid Tumor

The exact treatment for atypical teratoid/rhabdoid tumors will vary depending upon the location of the tumor and the age of your child, but the approach will be similar. Your child’s healthcare team will come together to develop a specific plan, and it will typically involve a combination of surgery and chemotherapy, with further radiation therapy when the child is old enough, if necessary.

The reason for the two different treatment modalities relates to the nature of your child’s condition. A brain tumor surgeon will remove the visible tumor through surgery, but because an atypical teratoid/rhabdoid tumor grows rapidly, adjunct therapy needs to be used to ensure all the tumor cells are eradicated. If any cells remain, there is an increased risk that the atypical teratoid/rhabdoid tumor will grow back, even if most of it has been removed.

If your child is very young and the brain is still developing, the healthcare team will likely recommend the use of chemotherapy as an adjunct therapy following surgery, as opposed to radiation therapy. Again, this will be something your child’s healthcare team will take into consideration when developing the treatment approach that is best for your child.

The surgical removal of your child’s atypical teratoid/rhabdoid tumor will be completed by a brain tumor surgeon, known as a neurosurgeon. Like many medical fields, there are specialists within neurosurgery, and some surgeons are experts specifically in brain tumors. Complete removal of the tumor has been shown to be the most important favorable prognostic factor contributing to overall survival in these children.

Why Choose Neurosurgeons of New Jersey?

As a parent, it can be very stressful and unsettling when your child is diagnosed with a medical issue. However, there are surgeons who specialize in the treatment of brain tumors and who can guide you through the process while providing expert care to your child.

Neurosurgeons of New Jersey is the largest subspecialized practice in the Tristate area, meaning they have surgeons who are focused on one area of practice and are authorities in their subspecialty. The brain tumor surgeons at Neurosurgeons of New Jersey are at the front line of clinical research, employing top-of-the line technology in their practices, all while maintaining a patient-centric focus.

The surgeons at Neurosurgeons of New Jersey will work with you to answer your questions and address your concerns to help you manage your child’s condition. As specialists in brain tumor surgery, they have assisted many parents in understanding the treatment process, both before and after therapy. That way, perhaps you can rest a little easier knowing your child’s health is in good hands.