Atypical Teratoid Rhabdoid Tumor, often called ATRT, is a rare and aggressive type of brain tumor that primarily affects young children, usually under the age of 3. While it can develop in older children and even adults, it is most commonly seen in infants and toddlers. ATRT can form in different parts of the central nervous system, including the brain and spinal cord, but it is most frequently found in the brain.

Due to its fast-growing nature, ATRT can lead to serious symptoms in a short period. Parents and caregivers often notice signs like persistent headaches, vomiting, changes in behavior, and issues with movement or coordination. Because these symptoms can be subtle initially, it’s important to seek medical attention quickly if they appear, especially in young children.

Although ATRT is considered a rare condition, advancements in medical research have led to better understanding and treatment options over the years. However, it remains a challenging diagnosis, and families affected by ATRT often face a difficult journey. This blog will explore what ATRT is, its symptoms, how it’s diagnosed, and the treatment options available to help families understand this complex condition.

Atypical Teratoid Rhabdoid Tumor (ATRT) Treatment 

ATRT Tumor

The treatment of ATRT is complex and typically involves a multimodal approach. Here, we explore the various treatment options available for ATRT in India and provide a general overview of their costs.

Surgical Intervention

Surgery is often the first line of treatment for ATRT. The primary goal is to remove as much of the tumor as possible without causing significant damage to the surrounding brain tissue. The cost of neurosurgery in India can vary widely depending on the hospital and the complexity of the procedure. On average, the price can range from INR 2,00,000 to INR 10,00,000.

Chemotherapy

Following surgery, chemotherapy is usually administered to target any remaining cancer cells. Chemotherapy regimens can differ based on the patient’s age and the specifics of the tumor. The cost of chemotherapy treatment for ATRT in India can range from INR 50,000 to INR 2,00,000 per cycle, with multiple cycles often required over several months.

Radiation Therapy

Radiation therapy may be used, particularly in older children or when the tumor has spread. This treatment involves targeting the tumor with high-energy rays to destroy cancer cells. The cost of radiation therapy in India can vary but generally ranges from INR 1,00,000 to INR 2,50,000.

Targeted Therapy & Clinical Trials

Some patients may be eligible for targeted therapy or may participate in clinical trials that offer new treatment options. These treatments are often part of research studies and may have varying costs associated with them. Depending on the trial’s funding and design, participation in clinical trials may sometimes be at a reduced cost or free of charge.

Supportive Care

Supportive care is also a critical component of ATRT treatment, which includes managing symptoms and improving the quality of life. This can involve various healthcare professionals and services, and the costs can vary greatly.

It’s important to note that the costs mentioned are approximate and can vary based on several factors, including the treatment center, the specific needs of the patient, and the length of treatment required. Consulting with a medical professional specializing in pediatric oncology is recommended for the most accurate and up-to-date information regarding treatment options and costs. They can provide personalized information based on the patient’s specific circumstances.

Atypical Teratoid Rhabdoid Tumor (ATRT) Cancer Life Expectancy

Several factors, including age at diagnosis, tumor location, and the extent of surgical resection, influence life expectancy for ATRT patients. Historically, the prognosis for ATRT has been poor, with rapid disease progression and limited treatment options. However, recent advancements in multimodal therapy, including surgery, chemotherapy, and radiation therapy, have improved patient outcomes.

A recent clinical trial highlighted that patients with ATRT treated with surgery followed by high-dose chemotherapy with stem cell rescue and focal radiation had an overall survival rate of 43%. This indicates a significant improvement in life expectancy compared to historical data, suggesting that aggressive and targeted treatment protocols can extend patients’ lives with ATRT.

Atypical Teratoid Rhabdoid Tumor (ATRT) Survival Rate

Due to the severity of the condition, the survival rate for ATRT has been the subject of extensive research and discussion. According to the National Cancer Institute, the 5-year survival rate for ATRT stands at approximately 32.2%. This average statistic encompasses various factors that can influence a patient’s prognosis. These factors include the tumor’s grade and type, the characteristics of the cancer, the age and overall health of the patient, their response to treatment, and how well they tolerate the treatment.

It is important to note that ATRT is a rapidly progressing tumor, with most fatalities occurring within the first 12 months after the onset of symptoms. The tumor can metastasize to other areas of the body, including the lungs, abdomen, liver, and, less commonly, the brain. Survival rates tend to be higher in older children compared to younger ones. With advancements in treatment, survival rates have shown improvement. If the tumor is detected early and treated before it spreads, and if it can be completely removed surgically, the chances of survival increase.

Atypical Teratoid Rhabdoid Tumor In Adults

Atypical Teratoid Rhabdoid Tumor (ATRT) is an aggressive and fast-growing type of brain tumor that is most commonly found in children, especially those under the age of 3. However, though it is extremely rare, ATRT can also occur in adults. When it happens in adults, the tumor behaves similarly but may be harder to diagnose because doctors don’t usually expect to see it in this age group.

What Is ATRT?

ATRT is a cancerous tumor that forms in the central nervous system (CNS), which includes the brain and spinal cord. It originates from certain cells called rhabdoid cells, which grow out of control. These tumors can form in various parts of the brain, including the cerebellum (which controls balance and coordination) and the cerebral hemispheres (which are responsible for higher functions like movement, thinking, and speech).

Atypical Teratoid Rhabdoid Tumor (ATRT) Symptoms

An Atypical Teratoid Rhabdoid Tumor (ATRT) is a rare and fast-growing brain tumor that mainly affects young children, usually under the age of 3. It can also occur in older children and, very rarely, in adults. The symptoms of ATRT depend on the size and location of the tumor in the brain, but here are the most common signs:

1. Headaches

   – Headaches are among the first signs, especially in older children who can describe the pain. These headaches are often worse in the morning or when lying down.

2. Nausea and Vomiting

   – Children with ATRT may experience frequent nausea and vomiting. This is because the tumor can increase pressure inside the skull, leading to discomfort.

3. Balance and Coordination Problems

   – Since ATRT can affect areas of the brain that control movement, children may have trouble walking, seem unsteady, or fall more often than usual.

4. Seizures

   – Some children with ATRT may have seizures, which are sudden, uncontrolled movements or episodes where they lose awareness. Seizures are a serious sign and require immediate medical attention.

5. Weakness on One Side of the Body

   – A tumor on one side of the brain may cause weakness or decreased movement on the opposite side of the body. For example, if the tumor is on the left side of the brain, the right arm or leg might be weaker.

6. Increased Head Size (in Babies)

   – In infants, ATRT can cause the soft spot on their head (called the fontanelle) to bulge, and their head size might increase quickly because the tumor causes pressure to build up in the brain.

7. Irritability and Changes in Behavior

   – Young children with ATRT may become more irritable, cry frequently, or seem less interested in activities they usually enjoy. They might also have trouble sleeping or feeding.

8. Vision Problems

   – A tumor affecting certain parts of the brain might cause vision issues. This could include difficulty seeing clearly or problems with eye movement.

9. Delayed Development (in Children)

   – Children with ATRT might show delayed development, meaning they may not reach milestones like walking, talking, or crawling at the usual age.

These symptoms can overlap with other health issues, so getting a medical evaluation is important if they persist, especially in young children. Early diagnosis & treatment are crucial for managing ATRT.

Atypical Teratoid Rhabdoid Tumor (ATRT) Prognosis

ATRT in adults is a very rare and serious condition. The prognosis depends on several factors, such as the size and location of the tumor, how much of it can be removed, and how well it responds to treatment. Because ATRT grows quickly and can spread to other parts of the brain or spinal cord, early detection, and aggressive treatment are crucial for improving outcomes.

However, the rarity of ATRT in adults means that there is less research and fewer treatment guidelines compared to pediatric cases. This makes managing the disease more difficult, and doctors often have to rely on treatments designed for other types of brain tumors.

Atypical Teratoid Rhabdoid Tumor (ATRT) Diagnosis

1. Medical History & Physical Exam

   – The doctor will first ask about the child’s symptoms, including how long they have been happening and whether they are getting worse. They will also ask about the child’s overall health, family medical history, and any past illnesses.

   – After this, the doctor will perform a physical exam to check for any signs of problems with movement, balance, vision, or general health.

2. Neurological Exam

   – Since ATRT affects the brain, a neurological exam is done to test how well the nervous system is working. This exam checks reflexes, muscle strength, coordination, and the child’s ability to think and respond to different tasks.

3. Imaging Tests

   – Doctors use imaging tests to get a clearer view of what’s happening inside the brain. The two most common ones are:

     – MRI (Magnetic Resonance Imaging): This scan uses magnets and radio waves to take detailed brain and spinal cord pictures. It can help doctors determine whether a tumor exists and how big it is.

     – CT Scan (Computed Tomography): A CT scan is sometimes used to check for a tumor quickly. It uses X-rays to take detailed images of the brain.

4. Biopsy

   If a tumor is found, the next step is often a biopsy. In this procedure, doctors take a small sample of the tumor tissue to examine under a microscope. This is the most important test because it tells doctors what kind of tumor it is, confirming whether it’s ATRT.

   – In some cases, surgery might be done to remove the tumor, and a biopsy is performed on the tissue that was removed.

5. Genetic Testing

   – ATRT is often linked to changes in a specific gene called SMARCB1 (or sometimes SMARCA4). After the biopsy, doctors will test the tumor cells for mutations in this gene. If this gene is missing or abnormal, it can confirm the diagnosis of ATRT.

   – Sometimes, the child and family may also undergo genetic testing to check for hereditary conditions that might increase the risk of ATRT.

6. Spinal Tap (Lumbar Puncture)

   – Since ATRT can spread to the spinal cord, doctors may perform a spinal tap (also called a lumbar puncture). This procedure takes a small amount of fluid from the spine to check for cancer cells. This helps doctors see if the tumor has spread beyond the brain.

7. Blood Tests

   – Although blood tests don’t directly diagnose ATRT, they are often done to check the overall health of the child and ensure they are strong enough for certain treatments, like surgery or chemotherapy.

The earlier ATRT is diagnosed, the better the chances for effective treatment, so it’s important to visit a doctor if symptoms persist or worsen.

Conclusion

Atypical Teratoid Rhabdoid Tumor (ATRT) is a rare and aggressive brain tumor, most commonly affecting young children but can also occur in adults. Due to its fast-growing nature, early diagnosis and treatment are crucial. Treatment often involves a combination of surgery, radiation therapy, and chemotherapy, all of which can be physically and emotionally challenging. The journey for families dealing with ATRT is difficult, but advances in medical care are helping improve treatment options.

However, the cost of ATRT treatment can be extremely high, especially with the need for specialized care and long-term therapies. In India, accessing the best medical facilities and treatments puts a heavy financial burden on families. This is where a fundraising platform can play a vital role. Fundraising Platforms like ImpactGuru allow families to raise funds from people willing to donate and support their cause. These platforms make it easier to share the patient’s story with a wide audience, ensuring that even small contributions from many people can help cover the high medical costs associated with ATRT treatment.