Atypical Teratoid Rhabdoid Tumor (ATRT)
Medikoe Health Expert
Koramangala, bengaluru, karnataka, india, Bengaluru Feb 9, 2017
Atypical teratoid rhabdoid tumor is a rare type of brain tumor that is highly malignant and occurs mostly in young children. It is a fast-growing cancerous tumor and mainly develops in the brain and spinal cord. It can occur in various parts of the central nervous system and other parts of the body. Around half of this tumor begins in the brainstem or cerebellum:
The brain stem controls the heart rate, breathing, and the muscles which are used in walking, seeing, talking, eating, and hearing.
The cerebellum is located at the base of the brain that is responsible for controlling the balance, posture, and movement.
In the brain, it can arise in ventricles, pineal gland, cerebellar hemispheres, suprasellar region, cerebral hemispheres, brain stem, and cerebellopontine angle. It can also rarely develop in the spinal cord.
What is Rhabdoid Tumor?
Rhabdoid tumors are similar to other tumor types such as MRI and CT. However, it is impossible to differentiate between these tumors using imaging tests. The rhabdoid tumor can only be determined with the help of biopsy or a specific test called BAF47 staining.
Atypical Teratoid Rhabdoid Tumor often appears to result due to specific changes in a gene that generally makes proteins for stopping the growth of the tumor. In this tumor, this gene does not function properly due to which protein is not made, and it becomes impossible to control the growth of cancer. However, this defect occurs only within cancer; the defect of this gene may be inherited. For this reason, it becomes necessary to consult a doctor for genetic testing.
Causes of Malignant Rhabdoid Tumors
Rhabdoid tumors can occur as a part of a rhabdoid tumor predisposition syndrome which means that some children are predisposed to developing this tumor. Most children who have rhabdoid tumors have the loss of a gene or mutation.
Since this tumor occurs in children of young age or infants, some researchers have suggested that this tumor usually develops from the germ cell stage or the stem cell.
Stages of Central Nervous System Rhabdoid Tumor
Rhabdoid tumors are divided or staged into two groups, i.e. localised and metastatic, and these are based on the results that are collected from the history of treatments provided to the children. Rhabdoid tumor is considered to be localised if the cells of cancer are not spread in other parts of the brain or the fluid present in the brain, i.e. cerebrospinal fluid. This is determined with the help of a test called a Lumbar Puncture. Besides, it can also be identified with the help of an MRI Scan. On the other hand, this tumor is said to be metastatic if the cancer cells are already spread to other body parts and the cerebrospinal fluid.
To define the stage of the tumor, there are specific tools which are used, and these tools include lumbar puncture, magnetic resonance imaging scan (MRI Scan), and computerised tomography scans (CT scans).
Medical Symptoms of Rhabdoid Tumors
The signs and symptoms of rhabdoid tumors vary in every patient and depend on the age of the child and where cancer has developed. Since this tumor is fast-growing, thus the symptoms develop quickly, and the condition gets worse over weeks. Some of the signs and symptoms associated with Rhabdoid tumor in children include:
Nausea and Vomiting
Increase in the head size (in infants)
Lack of coordination, physical imbalance, or trouble walking
Diagnosis of Rhabdoid Tumor
Rhabdoid tumor / Atypical Teratoid cannot be detected with tests like CT Scan or MRI Scan alone. It is necessary to perform biopsy and BAF47 to confirm its diagnosis. A biopsy is conducted for removing a sample of tissue. For brain tumors, the biopsy is performed for removing a part of the skull using a needle. The cancer cells are viewed and observed under a microscope by a pathologist who makes it possible for doctors to remove as much of the tumor during the surgery safely. Further, the pathologist checks the cancer cells to determine the type of brain tumor. However, it is difficult to altogether remove cancer like AR/RT because there is a possibility that the tumor is already spread before it is diagnosed.
When it comes to identifying the rhabdoid tumor, there are specific tests that are conducted to examine the brain and spinal cord. Some of these tests that are used for finding the CNS atypical teratoid or rhabdoid tumor include:
Physical Exam and Medical History
Magnetic Resonance Imaging
SMARCB1 and SMARCA4
Sometimes, rhabdoid tumors can be misdiagnosed when no proper tests are performed for mutation. If rhabdoid tumors are diagnosed, doctors must identify the extent of treatment that is to be provided, and it entirely depends on the age of the child and the factors associated with the tumor.
RT Cancer Treatment
The treatment for rhabdoid tumor depends on the size and the location of cancer. Besides, it also depends on the age of the patient. Because such tumors are aggressive, so most patients receive several treatment options. There are different types of treatment options available to treat rhabdoid tumors. The three main types of options used for treating AR/RT include Surgery, Radiation Therapy, and Chemotherapy.
Surgery - Surgery is used for both to diagnose and treat Atypical Teratoid Rhabdoid Tumors. In this treatment procedure, the surgeon usually removes a part of the skull from the patient’s body and then uses a needle to collect a sample of the tumor. Further, the sample is sent to the laboratory, and it is then examined under a microscope by the scientists.
Chemotherapy - In this treatment procedure, some powerful medicines are used for killing the cancer cells. Besides, with the help of drugs, the growth of these cancer cells is stopped. Chemotherapy is usually given after the surgery. It is given orally or can be injected directly into the bloodstream so that it can travel throughout the body.
Radiation Therapy - In radiation therapy, x-rays or other radiations of high energy is used for killing the cancer cells and also preventing them from further divisions and growth.
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