Atypical teratoid rhabdoid tumor

What is an atypical teratoid rhabdoid tumor?

Atypical teratoid rhabdoid tumors (ATRT) are very rare, aggressive central nervous system tumors that occur mainly in the cerebellum or brain stem (the part of the brain that regulates movement and balance) (the part of the brain that controls basic body functions).

• ATRTs usually occur by age 3 but occasionally arise in older children.
• ATRTs represent only 1 to 2 percent of childhood brain tumors.
• These tumors are part of a larger group of malignant tumors called rhabdoid tumors, which can occur outside the brain (such as a rhabdoid tumor of the kidney).
• ATRTs may be localized to one part of the brain. They can also spread to other locations in the brain, spine, or body.

The atypical teratoid rhabdoid tumor (ATRT) is a rare and fast-growing brain and spinal cord cancer tumor. The cerebellum or brain stem starts with about half of these tumors:

• The cerebellum, located at the base of the brain, controls movement, balance and posture.
• The brain stem controls breathing, heart rate and all the muscles used in seeing, hearing, walking, talking and eating.

ATRT also tends to result from changes in a gene that usually produces proteins to stop the growth of tumors. This gene does not work properly in ATRT, the protein is not produced, and tumor growth is uncontrolled. This gene deficiency is associated to more than 90 percent of ATRT cases. Although this mutation typically only occurs in cancer, this gene defect can be inherited and the need for genetic testing can be addressed by your doctor.

How common are atypical teratoid rhabdoid tumors?

• ATRT is very rare and is found in fewer than 10% of children with brain tumors.
• The disease is most often seen in children age 3 or younger, but it can also occur in older children and adults.

Symptoms of an atypical teratoid rhabdoid tumor

Depending on the patient’s age and the tumor’s location, ATRT symptoms differ widely. Since ATRTs are fast-growing, in a short period of time, symptoms typically develop rapidly.

Symptoms of ATRT may include the following:

• Morning headaches or headaches that are less painful after vomiting
• Nausea and vomiting
• Changes in activity levels
• Feeling sleepy
• Loss of balance, increasing problems with coordination or trouble walking
• Asymmetric eye movements or face movements
• Increase in head size (in infants)

Treatment of atypical teratoid rhabdoid tumor

Treatment depends on the tumor’s size and location, as well as the age of the patient. Many patients undergo many forms of treatment, which may include surgery, chemotherapy and radiation, because of the aggressive nature of these tumors.

• Surgery — is used to both diagnose and treat ATRT:
  • The surgeon removes part of the patient’s skull, uses a needle to take out a sample (biopsy) of the tumor and sends it to the laboratory. There, scientists examine the sample under the microscope.
  • If cancer is confirmed, the surgeon continues the operation by taking out as much of the tumor as it is safe to remove.
• Chemotherapy (“chemo”) — uses powerful medicines to kill cancer cells or stop them from growing (dividing) and making more cancer cells. Chemo is usually given after surgery.
  • Chemo may be injected into the bloodstream, so that it can travel throughout the body.
  • Some chemo may be given by mouth.
  • Combination therapy uses more than one type of chemo at a time.
• Radiation therapy — uses high-energy X-rays or other types of radiation to kill cancer cells or stop them from growing.

What are the survival rates for atypical teratoid rhabdoid tumor?

ATRT is an aggressive and difficult to cure type of cancer. Survival is weak, but improvements are being made in treatment. Older children have been benefited by recent advancements in treatment, and children with tumors that can be entirely removed have increased survival. In clinical trials, new treatments are being tested. In very young children and children with diseases that can not be surgically removed, the aim is to increase the cure rates and boost survival.

Atypical Teratoid/Rhabdoid Tumor chemotherapy

In first-generation studies, including the North American CCG9921 and POG 9923 trials, which observed just 10 percent EFS, traditional dose chemotherapy was mostly non-curative for ATRT patients. The Dana-Farber group, which included doxorubicin and dactinomycin in a “modified IRS-III” protocol, recorded a significantly better 1-year progression-free survival of 53 percent with the use of a sarcoma-based regimen. While some groups have reported benefits from protocols based on methotrexate and anthracycline, others have noted no changes in survival. Similarly, there are also contradictory studies on the use of ATRT platinum and alkylating agent regimens. There is no consensus on the most promising and active agents for ATRTs, partly because of the heterogeneous, multi-agent therapies often offered to patients with ATRT, but mainly because the relative contribution of these multiple variables to this rare disorder could not be robustly investigated by large-scale clinical trials.

Atypical Teratoid/Rhabdoid Tumor Surgery (Craniotomy)

What is a craniotomy?

To expose the brain, a craniotomy is the surgical removal of part of the bone from the skull. To extract the part of bone called the bone flap, specialized tools are used. After the brain procedure has been completed, the bone flap is briefly removed and replaced.

The guidance of computers and imaging (magnetic resonance imaging [MRI] or computerized tomography [CT] scans) can be used in certain craniotomy procedures to reach the exact position inside the brain to be treated. This technique involves the use of a frame mounted on the skull or a frameless device using markers or landmarks superficially placed on the scalp. It is called stereotactic craniotomy when one of these imaging techniques is used along with the craniotomy process.

In combination with these machines and localizing frames, brain scans offer a three-dimensional view of a tumor inside the brain, for example.

It is helpful in separating the tumor tissue from the healthy tissue and in reaching the exact position of the abnormal tissue.

Stereotactic biopsy of the brain (a needle is directed into an irregular region so that a fragment of tissue can be removed under a microscope for examination), stereotactic aspiration (fluid removal from abscesses, hematomas, or cysts), and stereotactic radiosurgery are other uses (such as gamma knife radiosurgery).

An endoscopic craniotomy is another form of craniotomy that involves inserting a lighted scope through a small incision in the skull with a camera into the brain.

Another surgical procedure which may involve a craniotomy is aneurysm clipping. A cerebral aneurysm (also referred to as an intracranial aneurysm or brain aneurysm) is a bulging region in the wall of the brain’s artery that is compromised, resulting in excessive enlargement or ballooning. There is a chance of rupture (bursting) of the aneurysm because of the damaged region in the artery wall. The placing of a metal clip around the “neck” of the aneurysm isolates the aneurysm by restricting the flow of blood from the rest of the circulatory system, thereby preventing rupture.

Craniectomy is a similar procedure during which, after swelling has reduced, a part of the skull is permanently removed or replaced later during a second operation.

Other related procedures that may be used to diagnose brain disorders include cerebral arteriogram , computed tomography (CT) scan of the brain , electroencephalogram (EEG) , magnetic resonance imaging (MRI) of the brain , positron emission tomography (PET) scan , and X-rays of the skull . Please see these procedures for additional information.

Types of Craniotomy

Extended Bifrontal Craniotomy

A conventional skull base technique used to target challenging tumors at the front of the brain is prolonged bifrontal craniotomy. It is based on the idea that the removal of extra bone is better than excessively manipulating the brain.

The prolonged bifrontal craniotomy involves making an incision behind the hairline in the scalp and extracting the bone that forms the orbit and forehead contour. At the end of surgery, this bone is replaced. The temporary removal of this bone enables surgeons to operate in the space between and right behind the eyes without having to manipulate the brain excessively.

For certain tumors that are not a candidate for removal by minimally invasive methods, extended bifrontal craniotomy is usually used regardless of either the tumor structure, the potential pathology of the tumor, or the objectives of surgery.

Types of tumors treated with the extended bifrontal craniotomy include meningiomas , esthesioneuroblastomas and malignant skull base tumors .

Minimally Invasive Supra-Orbital “Eyebrow” Craniotomy

Supra-orbital craniotomy (often called “eyebrow” craniotomy) is a procedure used to remove brain tumors. In this procedure, neurosurgeons make a small incision within the eyebrow to access tumors in the front of the brain or pituitary tumors . This approach is used instead of endonasal endoscopic surgery when a tumor is very large or close to the optic nerves or vital arteries.

Because it is a minimally invasive procedure, supra-orbital “eyebrow” craniotomy may offer

• Less pain than open craniotomy
• Faster recovery than open craniotomy
• Minimal scarring

The supra-orbital craniotomy may be part of the treatment for Rathke’s cleft cysts, skull base tumors and some pituitary tumors.

Retro-Sigmoid “Keyhole” Craniotomy

Retro-sigmoid craniotomy (often called “keyhole” craniotomy) is a minimally-invasive surgical procedure performed to remove brain tumors. This procedure allows for the removal of skull base tumors through a small incision behind the ear, providing access to the cerebellum and brainstem. Neurosurgeons may use this approach to reach certain tumors, such as meningiomas and acoustic neuromas (vestibular schwannomas).

Benefits of “keyhole” craniotomy includes less pain after the procedure than after an open craniotomy, less scarring and a more rapid recovery.

The retro-sigmoid craniotomy may be performed for the following types of brain tumors:

• Acoustic neuromas (vestibular schwannomas)
• Meningiomas
• Metastatic brain or spine tumors
• Skull base tumors

Orbitozygomatic Craniotomy

A typical skull base technique used to target challenging tumors and aneurysms is the orbitozygomatic craniotomy. It is based on the idea that the removal of extra bone is better than excessively manipulating the brain.

Orbitozygomatic craniotomy, usually used for those lesions that are too complex for removal by more minimally invasive techniques, includes making an incision in the scalp below the hairline and extracting the bone that forms the orbit and cheek contour. At the end of surgery, this bone is replaced. Removing this bone temporarily enables surgeons to access deeper and more demanding areas of the brain while preventing permanent brain damage.

Craniopharyngiomas, pituitary tumors, and meningiomas include brain tumors that can be treated with orbitozygomatic craniotomy.

Translabyrinthine Craniotomy

A translabyrinthine craniotomy is a procedure involving the removal of the mastoid bone and some of the inner ear bone through an incision in the scalp behind the ear (specifically, the semicircular canals which contain receptors for balance). The surgeon will then identify and remove the tumor, or as much of the tumor as possible, without any chance of permanent brain injury.

Acoustic neuroma (vestibular schwannoma) is treated with one of three approaches: suboccipital, translabyrinthine, and middle fossa for translabyrinthine craniotomy.

The translabyrinthine solution is also considered when there is no beneficial hearing or hearing is to be sacrificed. The semicircular canals of the ear are cut during the translabyrinthine craniotomy in order to reach the tumor. As a result of the elimination of the semicircular canals, complete hearing loss occurs.

The risk of facial nerve damage can be minimized, while hearing is lost with translabyrinthine craniotomy.

Reasons for the procedure

A craniotomy may be done for a variety of reasons, including, but not limited to, the following:

• Diagnosing, removing, or treating brain tumors
• Clipping or repairing of an aneurysm
• Removing blood or blood clots from a leaking blood vessel
• Removing an arteriovenous malformation (AVM) or addressing an arteriovenous fistula (AVF)
• Draining a brain abscess, which is an infected pus-filled pocket
• Repairing skull fractures
• Repairing a tear in the membrane lining the brain (dura mater)
• Relieving pressure within the brain (intracranial pressure) by removing damaged or swollen areas of the brain that may be caused by traumatic injury or stroke
• Treating epilepsy
• Implanting stimulator devices to treat movement disorders such as Parkinson’s disease or dystonia (a type of movement disorder)

There may be other reasons for your doctor to recommend a craniotomy.

Risks of the procedure

Complications can occur, as in any surgical procedure. The risk of brain surgery is related to the particular position in the brain that will be affected by the procedure. For example, if there is surgery on the part of the brain that regulates speech, then speech will be impaired. The following include but are not limited to several more general complications:

• Infection
• Bleeding
• Blood clots
• Pneumonia (infection of the lungs)
• Unstable blood pressure
• Seizures
• Muscle weakness
• Brain swelling
• Leakage of cerebrospinal fluid (the fluid that surrounds and cushions the brain)
• Risks associated with the use of general anesthesia

The following complications are uncommon and typically refer to particular locations within the brain, so for those individuals they may or may not be legitimate risks:

• Memory problems
• Speech difficulty
• Paralysis
• Abnormal balance or coordination
• Coma

Depending upon your unique medical condition, there might be other dangers. Prior to the operation, make sure to address any questions with your doctor.

Before the procedure

• Your doctor will explain the procedure to you and you can ask questions.
• You will be asked to sign a consent form that gives permission to do the surgery. Read the form carefully and ask questions if something is not clear.
• In addition to a complete medical history, your doctor will do a physical exam to ensure you are in good health before you undergo the surgery. You may also need blood tests and other diagnostic tests.
• You will receive a preoperative neurological exam that will be used to compare with postoperative exams.
• You will be asked to fast before the procedure, generally after midnight.
• If you are pregnant or think you may be, tell your healthcare provider.
• Tell your doctor if you are sensitive to or are allergic to any medicines, latex, tape and anesthetic agents (local or general).
• Tell your doctor of all medicines (prescribed and over-the-counter) and herbal supplements that you are taking.
• Tell your doctor if you have a history of bleeding disorders or if you are taking any anticoagulant (blood-thinning) medicines, aspirin, or other medicines that affect blood clotting. It may be necessary for you to stop these medicines before the procedure.
• If you smoke, you should stop smoking as soon as possible before the procedure to improve your chances for a successful recovery from surgery and to improve your overall health status.
• You may be asked to wash your hair with a special antiseptic shampoo the night before the surgery.
• You may receive a sedative before the procedure to help you relax.
• The areas around the surgical site will be shaved.
• Based on your medical condition, your doctor may request other specific preparation.

During the procedure

A craniotomy generally requires a hospital stay of 3 to 7 days. You may also go to a rehabilitation unit for several days after your hospital stay. Procedures may vary depending on your condition and your doctor’s practices.

Generally, a craniotomy follows this process:

1. You will be asked to remove any clothing, jewelry, or other objects that may interfere with the procedure.
2. You will be given a gown to wear.
3. An intravenous (IV) line will be inserted in your arm or hand.
4. A urinary catheter will be inserted to drain your urine.
5. You will be positioned on the operating table in a manner that provides the best access to the side of the brain to be operated on.
6. The anesthesiologist will continuously monitor your heart rate, blood pressure, breathing, and blood oxygen level during the surgery.
7. Your head will be shaved and the skin over the surgical site will be cleansed with an antiseptic solution.
8. There are various types of incisions that may be used, depending on the affected area of the brain. An incision may be made from behind the hairline in front of your ear and the nape of your neck, or in another location depending on the location of the problem. If an endoscope is used, the incisions may be smaller.
9. Your head will be held in place by a device which will be removed at the end of the surgery.
10. The scalp will be pulled up and clipped to control bleeding while providing access to the brain.
11. A medical drill may be used to make burr holes in the skull. A special saw may be used to carefully cut the bone.
12. The bone flap will be removed and saved.
13. The dura mater (the thick outer covering of the brain directly underneath the bone) will be separated from the bone and carefully cut open to expose the brain.
14. Excess fluid will be allowed to flow out of the brain, if needed. Microsurgical instruments, such as a surgical microscope to magnify the area being treated, may be used. This can enable the surgeon a better view of the brain structures and distinguish between abnormal tissue and healthy tissue. Tissue samples may be sent to the lab for testing.
15. A device, such as a drain or a special type of monitor, may be placed in the brain tissue to measure the pressure inside the skull, or intracranial pressure (ICP). ICP is pressure created by the brain tissue, cerebral spinal fluid (CSF), and blood supply inside the closed skull.
16. Once the surgery is completed, the surgeon will suture (sew) the layers of tissue together.
17. The bone flap will be reattached using plates, sutures, or wires.
18. If a tumor or an infection is found in the bone, the flap may not be replaced. Also, if decompression (to reduce pressure in the brain) is required, the bone flap may not be replaced.
19. The skin incision (scalp) will be closed with sutures or surgical staples.
20. A sterile bandage or dressing will be applied over the incision.

After the procedure

In the hospital

You will be taken to a recovery room for observation directly after the operation, before being taken to the intensive care unit (ICU) to be closely monitored. Or you may be brought from the operating room directly to the ICU.

You may be given medication to alleviate brain swelling in the ICU.

Depending on the type of operation performed and the type of anesthesia provided, the healing process can differ. You can be brought to the ICU or your hospital room until your blood pressure, pulse, and breathing are steady and you are alert.

You can move to a room in a neurosurgical nursing unit in the hospital after you remain in the ICU. You will stay for some more days in the hospital.

After surgery, you can need oxygen for a period of time. Oxygen is usually discontinued before you go home.

To help re-expand the lungs and avoid pneumonia, you will be taught deep-breathing exercises.

The nursing and medical staff will perform regular neurological examinations to assess your brain function and to ensure that your body systems function properly after your surgery. In order to test your brain capacity, you will be asked to obey a series of simple instructions, such as shifting your arms and legs. Your students will be tested with a flash light, and questions will be asked to determine your orientation (such as your name, the date, and where you are). There will also be checks on the strength of the arms and legs.

To avoid swelling of the face and ears, the head of your bed may be elevated. It is common to have some swelling.

When your strength increases, you will be able to move around as tolerated when in bed and to get out of bed and walk around with assistance at first.

You will be asked to assess your strength, balance, and mobility by a physical therapist (PT) and give you recommendations for exercises to do both in the hospital and at home.

To avoid blood clot formation, you would possibly have sequential compression devices (SCDs) put on your legs when you are in bed. SCDs have an air compressor that pumps air slowly into and out of tailored sleeves that are located on the thighs. By passively compressing the leg veins to keep blood flowing, they help prevent blood clots from developing.

A few hours after surgery, you might be given beverages to drink, depending on your condition. When you can tolerate them, your diet can be progressively adjusted to include more solid foods.

For a day or two, or before you can get out of bed and walk about, you can have a catheter in your bladder to remove your urine. As these may be signs of an infection that can be treated, be sure to report any painful urination or other urinary symptoms which occur after the catheter is removed.

You could be moved to a rehabilitation center for a period of time to recover your power, depending on your status.

Before you are released from the hospital, plans are made with your doctor for a follow-up appointment. You will also be given directions from your doctor for home treatment.

At home

It is crucial to keep the incision clean and dry until you are home. Relevant bathing instructions are given to you by your doctor. On a follow-up office visit, if stitches or surgical staples are used, they will be removed. Keep them dry if adhesive strips are used, and they may fall off within a few days.

A loose turban or hat over the incision may be worn. You should not wear a wig until the incision has healed completely (about 3 to 4 weeks after surgery).

There may be pain in the incision and head, especially with deep breathing, coughing, and exertion. As advised by your physician, take a pain reliever for soreness. The risk of bleeding can be increased by aspirin or other blood thinning medicines. Make sure only the recommended medications are taken and ask if you are uncertain.

To stop lung infection, continue the breathing exercises used in the hospital. To prevent exposure to upper respiratory illnesses (colds and flu) and irritants such as cigarette smoke, fumes, and pollutants to the atmosphere, you will be told.

When you can control them, you can progressively increase your physical activity. Returning to your previous energy and strength level can take several weeks.

To prevent pressure on your surgical incision, you might be advised to avoid lifting heavy objects for several weeks.

Do not drive until permission is given to you by your doctor.

Call your doctor to report any of the following:

• Fever or chills
• Redness, swelling, drainage, or bleeding or other drainage from the incision site or face
• Increased pain around the incision site
• Vision changes
• Confusion or excessive sleepiness
• Weakness of your arms or legs
• Trouble with speech
• Trouble breathing, chest pain, anxiety, or change in mental status
• Green, yellow, or blood-tinged sputum (phlegm)
• Seizure activity

Following a craniotomy, your doctor can give you other directions, depending on your particular circumstance.

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• September 2nd, 2020