Atypical Teratoid/Rhabdoid Tumors (AT/RT) Treatment in India

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Atypical Teratoid/Rhabdoid Tumor (AT/RT) is a rare and aggressive type of pediatric brain cancer that typically affects children under the age of three, though it can occur at any age. Characterized by rapid growth and a high risk of spreading within the central nervous system, AT/RT requires urgent and comprehensive medical intervention. In India, world-class oncology centers offer advanced, multidisciplinary treatment for AT/RT that includes surgery, chemotherapy, radiation therapy, stem cell transplantation, and access to cutting-edge clinical trials. These treatments are delivered by highly experienced specialists such as Dr. Anurag Bahl, a leading expert in pediatric neuro-oncology in nIndia. One of the key reasons international patients choose India is the affordability of care—without compromising on quality. The average cost for complete AT/RT treatment in India ranges between USD 7,500 to 10,000, significantly lower than the USD 80,000 to 150,000 typically charged in the USA or USD 25,000 to 40,000 in Thailand. This cost-effective, high-quality care, combined with personalized support for international patients, makes India a trusted destination for AT/RT treatment.

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What is Atypical Teratoid/Rhabdoid Tumor (AT/RT)?

Atypical Teratoid/Rhabdoid Tumor (AT/RT) is a rare and aggressive form of cancer that primarily affects the central nervous system (CNS), including the brain and spinal cord. It is most commonly diagnosed in children under the age of three but can occur at any age. AT/RTs are classified as Grade IV tumors, indicating a high-grade, fast-growing malignancy.

What are the Symptoms of Atypical Teratoid/Rhabdoid Tumor (AT/RT)?

The symptoms of AT/RT largely depend on the location of the tumor in the brain or spinal cord, and the age of the patient. Since this tumor commonly affects infants and young children, symptoms may be subtle and easily mistaken for common childhood illnesses. However, the disease progresses rapidly, making early recognition crucial.

General Neurological Symptoms

  • Increased Intracranial Pressure (ICP): Due to obstruction of cerebrospinal fluid (CSF) flow
    • Persistent vomiting
    • Headache (may manifest as irritability in infants)
    • Lethargy or decreased consciousness
  • Seizures: Sudden onset seizures may be the first sign
  • Macrocephaly or Bulging Fontanelle: Especially in infants under 1 year
  • Poor feeding and failure to thrive

Symptoms Based on Tumor Location

Location Associated Symptoms
Supratentorial (cerebral hemispheres) Hemiparesis, visual disturbances, behavioral changes
Infratentorial (posterior fossa/cerebellum) Ataxia, coordination problems, nystagmus, cranial nerve palsies
Spinal cord involvement Limb weakness, back pain, bladder or bowel dysfunction

In Infants and Toddlers

  • Poor head control
  • Developmental regression
  • Persistent crying or inconsolable behavior
  • Delayed milestones

What are the diagnosis of Atypical Teratoid/Rhabdoid Tumor (AT/RT)?

Timely and accurate diagnosis of AT/RT is critical due to its aggressive behavior and similarity to other CNS tumors in children. The diagnostic process combines neuroimaging, pathology, immunohistochemistry, and molecular testing to confirm AT/RT and plan the treatment strategy.

Diagnostic Tool Purpose
MRI Brain + Spine (with contrast) Gold-standard imaging to determine tumor size, location, and CNS/spinal involvement. Crucial for staging and surgery planning.
Histopathology + IHC for INI1 Microscopic examination of tumor cells and immunohistochemistry showing loss of INI1 (SMARCB1)—the definitive diagnostic marker for AT/RT.
CSF Cytology (Lumbar puncture) Detects tumor cells in cerebrospinal fluid, indicating leptomeningeal dissemination.
Genetic Testing (SMARCB1/SMARCA4) Confirms molecular signature of AT/RT. Also helpful in familial screening and counseling.
Baseline Blood Tests CBC, liver/renal profile, and electrolytes to assess the child's overall health before starting chemotherapy or surgery.

What Causes AT/RT and Who is at Risk?

Atypical Teratoid/Rhabdoid Tumor (AT/RT) is a rare and aggressive cancer primarily affecting the central nervous system (CNS) of infants and young children. Although the exact cause is not fully understood, advances in molecular genetics have revealed important clues about its origins and risk factors.

What Causes AT/RT?

The development of AT/RT is strongly linked to genetic mutations, particularly:

  • Loss or mutation of the SMARCB1 gene (also known as INI1/hSNF5/BAF47), located on chromosome 22.
    • This gene is a tumor suppressor that plays a key role in regulating cell growth and differentiation.
    • In nearly all cases of AT/RT, loss of SMARCB1 expression is confirmed via immunohistochemistry (IHC).
  • In rare cases, SMARCA4 gene mutations may be implicated, especially in extra-CNS rhabdoid tumors.

These genetic changes result in uncontrolled cell proliferation, leading to tumor formation.

  Note: These mutations are typically not inherited, but in 10–15% of cases, they may be part of a genetic syndrome known as rhabdoid tumor predisposition syndrome (RTPS).

Who Is at Risk?

While AT/RT is a rare condition, some factors are associated with increased risk:

Risk Factor Details
Age Most cases occur in children under 3 years of age; rare in adults.
Genetic Predisposition (RTPS) Children born with germline mutations in SMARCB1/SMARCA4 have a higher risk.
Family History Very rare, but a positive family history of rhabdoid tumors may suggest hereditary risk.
Male Gender Slightly more common in boys than girls.

Should Families Undergo Genetic Testing?

  • If a child is diagnosed with AT/RT, genetic counseling and testing for SMARCB1 or SMARCA4 mutations may be recommended.

  • This helps assess the risk of recurrence in future siblings or relatives and guides surveillance protocols.

What are the  AT/RT Treatment Options available in India?

A multimodal approach is essential, typically involving:

A. Surgical Resection

  • Goal: Maximal safe tumor resection
  • Gross total resection (GTR) improves survival significantly
  • Performed under neuromonitoring in pediatric neurosurgery centers

B. Chemotherapy

  • Initiated post-operatively, even in infants
  • Standard regimen: High-dose multi-agent chemotherapy, often based on COG ACNS0333 protocol
Common Agents Schedule
Vincristine, Cyclophosphamide Weeks 1–6, alternating with Cisplatin, Etoposide
Methotrexate (high-dose) Optional in selected regimens
Intrathecal Chemotherapy Methotrexate or Cytarabine for CSF prophylaxis

Note: Chemotherapy is dose-adjusted based on age and weight.

C. Radiation Therapy

  • Craniospinal irradiation (CSI) + Boost to primary tumor site
  • Typically deferred in children <3 years; used in >3 years or high-risk infants
  • Advanced techniques used: IMRT/Proton therapy (if available)

High-Dose Chemotherapy with Autologous Stem Cell Rescue (HDC-ASCR)

  • Considered for children after induction chemotherapy
  • Involves:
    • Myeloablative chemotherapy (e.g., Thiotepa, Carboplatin, Etoposide)
    • Stem cell infusion to restore marrow
  • Available at specialized centers like Fortis Memorial, Apollo Chennai, Tata Memorial

Role of Dr. Ankur Bahl in Treating Atypical Teratoid/Rhabdoid Tumors (AT/RT)

Dr. Ankur Bahl, Senior Director of Medical Oncology at Fortis Memorial Research Institute (FMRI), Gurugram, is one of India’s most trusted names in pediatric and adult neuro-oncology. His leadership in managing rare and aggressive CNS tumors like Atypical Teratoid/Rhabdoid Tumors (AT/RT) makes him a preferred choice for families seeking specialized care in India and abroad.

Expertise in AT/RT Multimodal Management

Dr. Bahl collaborates closely with pediatric neurosurgeons, radiation oncologists, and stem cell transplant specialists to ensure a comprehensive, personalized treatment approach for each AT/RT case. His involvement spans:

  • Chemotherapy Protocol Design: Tailoring multi-agent regimens to the child’s age, tumor genetics, and stage
  • High-Dose Chemotherapy Planning: Coordinating intensive chemotherapy cycles with stem cell support
  • CNS-Directed Intrathecal Therapy: Managing cerebrospinal fluid (CSF) prophylaxis to prevent spinal spread
  • Post-Treatment Surveillance: Long-term follow-up with MRI reviews, blood work, and developmental support
  • Family Counseling & Genetic Testing: Supporting families with education, BRCA/SMARCB1 testing, and emotional care

Why Families Trust Dr. Bahl

  • Over 20 years of clinical experience in treating rare pediatric CNS tumors
  • Globally trained with expertise in the latest oncology innovations
  • Access to cutting-edge diagnostics (INI1/SMARCB1 mutation testing, PET-CT, MR Spectroscopy)
  • Integral part of FMRI’s Pediatric Tumor Board, ensuring evidence-based, multidisciplinary decisions
  • Fluent in coordinating international patient care with dedicated visa, interpreter, and virtual consult services

AT/RT Treatment Cost Comparison: India vs. Turkey vs. USA

Treatment Component India (USD) Turkey (USD) USA (USD)
Brain Surgery (Craniotomy) $4,000 – $8,000 $7,000 – $12,000 $40,000 – $70,000
Awake Craniotomy (if applicable) $7,000 – $10,000 $10,000 – $15,000 $60,000 – $90,000
Radiation Therapy (IMRT) $5,000 $15,000 $30,000 – $50,000
Radiation IGRT $6,000 $15,000 $30,000 – $50,000
Radiation V-MAT $6,500 $15,000 $30,000 – $50,000
Chemotherapy (Multi-agent cycles) $700 – $1,500 $1,000 – $2,000 $10,000 – $50,000
High-Dose Chemo + Stem Cell Rescue (HDC-ASCR) $18,000 – $25,000 $30,000 – $45,000 $150,000 – $250,000
Diagnostics (MRI, Biopsy, IHC, Genetic Tests) $500 – $1,000 $800 – $1,200 $3,000 – $6,000

What are the available Support for International Patients Coming to India?

India's hospitals offer dedicated services for international patients, including:

  • Visa Assistance: Guidance through the medical visa application process.
  • Travel and Accommodation: Help with booking flights and lodging near the hospital.
  • Language Support: Interpreter services to facilitate communication.
  • Post-Treatment Care: Coordination of follow-up appointments and remote consultations.

What are the Survival Rate, Prognosis, and Follow-Up Care?

AT/RT is an aggressive tumor with a generally poor prognosis. However, early detection and a comprehensive treatment plan can improve outcomes.

Risk Category Estimated 2-Year Survival
Gross total resection + Radiation 60–70%
Subtotal resection, <3 years age 20–40%
Disseminated disease at diagnosis <20%

Follow-Up Care:

Regular monitoring through imaging and clinical evaluations is crucial to detect any recurrence. Long-term follow-up also addresses potential late effects of treatment, especially in children.

Frequently Asked Questions

The average hospitalization period ranges from 3 to 5 days, depending on the treatment plan and patient recovery.

A stay of 7 to 14 days is generally recommended to accommodate pre-treatment evaluations, the procedure, and initial recovery.

Yes, India offers world-class cancer care with internationally trained oncologists and state-of-the-art facilities, ensuring treatment quality on par with Western standards.

Yes, most hospitals have dedicated international patient services that assist with travel logistics, accommodation bookings, and other necessary arrangements.
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