Atypical Teratoid

Published Date : Jan 2024
Category : Oncology
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“Atypical Teratoid: “Unraveling the Enigma of Atypical Teratoid Dystrophy"

The rare and aggressive kind of central nervous system neoplasm known as Atypical Teratoid/Rhabdoid Tumor (AT/RT) mainly affects young children. This cancer presents substantial hurdles for diagnosis and treatment because of its unique histological characteristics. Even though the tumor is uncommon, its fast growth and propensity for metastasis highlight the urgent need for thorough research projects aiming at elucidating its underlying molecular pathways and creating focused treatment approaches.

The incidence of AT/RT differs with age, with 8.1 cases per million in children under 1 year old and 0.6 cases per million in children aged 5 to 9 years. Atypical Teratoid tumors account for less than 5% of total pediatric CNS tumors, but they make up 10% to 20% of CNS tumors in children under the age of three. Mortality rate among the AT/RT according to the St. Jude Children's Research Hospital is 70% in children’s.

Embryonal central nervous system (CNS) cancers that are clinically aggressive and have a median overall survival (OS) of 6 to 17 months. Furthermore, the American Brain Tumor Association released the stats of survival rates for ATRT by age group are children with the age of 1-14 years having the 47.8 % and for young adults with 15-39 have 41.5 % and for adults more than 40-year age have only 24.6 % AT/RT survival rates.

The rare high-grade tumor known as a teratoid rhabdoid cancer, or ATRT, primarily affects children three years of age and under. It is the part of broad category of malignant tumors. They have a tendency to grow quickly, is resemble a big, bulky mass, and this spread into the brain and spinal cord. Cancer is a genetic disease, meaning that specific alterations to the genes that regulate the activity of our cells are what cause the disease. Many cancer types have genetic mutations that is accelerate the growth and migration of cancer cells. The majority of ATRTs are brought on by mutations in the SMARCB1 gene (also called INI1). Early identification of ATRT is challenging since AT/RT symptoms vary from individual to individual and rely on the size and location of the tumor, according to the National Cancer Institute. The treatments for this type cancer majorly includes the surgeries, Chemotherapy, Radiation therapy and Chromatin remodeling agents.

DiseaseLandscape Insights helps decision-makers in the fields of medical technology, therapies, and diagnostics where new developments in technology will change the conditions and drive greater growth.


Atypical teratoid/rhabdoid tumors in infants and kids cause symptoms like these:

  • Headaches and nausea
  • Throwing up
  • Sleeping more than normal
  • Seeming lazy
  • Decreasing to a typical level of energy
  • Walking challenges
  • Imbalance or coordination issues
  • Weakening muscles
  • Bigger size of the head
  • Tilt of head posture
  • One side of the body paralyzed; dual vision
  • Weakening of the facial muscles

Diagnostic Analysis

Diagnostic process of the atypical teratoid rhabdoid tumor is quite difficult because the symptoms of the teratoid rhabdoid tumor are varies from the person to persons and features of the tumor and the extent to which it has spread across the central nervous system (CNS) as it became the difficult to diagnose the AT/RT disease. The primary diagnostic categories listed below, which greatly impact the market based on a number of variables such the development of diagnostic technologies. Below are the some majorly used techniques of atypical teratoid rhabdoid tumor:

  • MRI
  • Spinal tap
  • Genetic testing
  • Biopsy


The first step in treating AT/RT is surgery, which requires careful planning and the use of MRI. The risk of morbidity and the degree of tumor removal both ascertained by MRI. Additionally, MRI is used to assess how well a tumor responds to radiation and chemotherapy, which are often administered follow surgery. A tumor's return or advancement needs for more therapy or palliative care, which MRI might help to detect. The MRI machine is the most accurate machine for the detection of the atypical teratoid detection. According to the National Institute of Health reports of Diagnostic accuracy of qualitative MRI in Jan 2022 shows that the accuracy for the embryonal tumor ranges from 63.33–79.59%.

Spinal tap

Spinal tap is the process by which the sample from the spinal cord is extracted with the help of the needle in case to check the presence of the cancer cell.

Genetic testing

The genetic test basically includes the collection of blood sample or the tissue sample is taken to examine the certain change in the SMARCB1 and SMARCA4 gene. The process of genetic testing involves examining a person's or a tumor's DNA to look for mutations or alterations in particular genes. As it helps in the identifying possible targeted therapy is facilitated by knowledge of the tumor's genetic history.


Cutting a tiny portion of the skull in order to do the biopsy, and then they must use a needle to extract cells from the damaged area. During the biopsy procedure, a physician surgically removes as much of the tumor as feasible if cancer is diagnosed.

Top Players of Diagnostic Kits

The market for Atypical teratoid testing is very competitive worldwide, and as a result, there are many major competitors in the industry with a global presence. The leading producers of Atypical teratoid diagnostic kits are included in the table below.

Diagnostic Market Players

Diagnostic Market Players

Diagnostic Product

Biocare Medicals



Super SensitiveTM 1-Step Polymer HRP Detection Kit ISH


Multiplex IHC Detection kit

Santa Cruz Biotechnology

ImmunoCruz® ABC Kit: sc-516216

Bio-Rad Laboratories

QX600 Droplet Digital PCR System

Promega Corporation

qPCR Probe Kits: GoTaq® Probe qPCR and RT-qPCR Systems

Bioneer Corporation

AccuPower® PCR PreMix


Taq DNA Polymerase

Illumina, Inc.

MiSeq Reagent Kit v3

GE Healthcare

MRI Scan Machines SIGNA™

Siemens Healthineers


Philips Healthcare,

MR 7700

Canon Medical Systems

ONE Aquilion


Treatment Analysis:

Atypical teratoid is treated with several therapies. Therapeutic developments in the field of the Atypical teratoid treatment is continuously making the changes as the diseases is also getting mutations in it. According to a statement by the medicine's developer, Kazia Therapeutics Limited, Paxalisib has been given an orphan drug designation by the FDA in June 20, 2022 for use as a possible therapeutic option for children with atypical rhabdoid and teratoid tumors, a rare and extremely aggressive pediatric brain cancer. By the study of Dana-Farber/Boston Children's, the recovery rate is improved from the 10% to the 50% in Atypical teratoid cancers.

Basically, the treatments for atypical teratoid diseases includes the Surgery, Chemotherapy, stem-cell transplant, Radiation therapy and other medications.


Most of the tumor that is found will be removed by the neurosurgeons, typically during the biopsy. Whether a significant portion of the tumor is surgically removed depends on a number of criteria, including the tumor's size, location, and whether or not it has spread to other parts of the body. The relevant survival rate after completion of the surgeries is presented by the American Brain Tumor Association in children are 48.7%, in young adults it is 41.8% and in adults it is 24.6 %.

High-dose chemotherapy with stem-cell transplant

This type of treatment procedure includes the replacement of the bone marrow stem cells that the chemotherapy destroyed with fresh cells using larger dosages of chemotherapy. After that the stem start the producing new RBC. High dosages of potent chemotherapy drugs damages bone marrow's blood-forming cells; therefore, a stem-cell transplant restore the body's capacity to fight the tumor and successfully restore blood supplies. Younger individuals who are not good candidates for radiation therapy is offered stem-cell transplants by medical professionals.

Radiation therapy

To attempt to eradicate any cancer cells that is still present, doctors employs radiation therapy on the brain and spinal cord following surgery, either with or without chemotherapy. Children under the age of 3 are typically not eligible for this therapy because brain radiation stunt a baby's or young child's growth and development. According to the National Institutes of Health the survival rate after the completion of the radiotherapy is found to be 56% in patients.

1) ‘Anticancer’ Drugs Used for Treatment

The following table lists the three anticancer medications presently used to treat atypical teratoid syndrome along with details on their typical dosage, formulation, mechanism of action, and adverse effects:


Mode of Action


Typical Dosing


Side Effects


By damaging DNA cell stop them from dividing

50 mg/kg per dose once a day


·       Anxiety

·       Nausea

·       Fainting

·       Pale Skin

·       Vomiting



By damaging DNA cell stop them from dividing

Carboplatin injection300 mg/m² IV on day 1 every 4 weeks for 6 cycles



·       Tingling Feeling in Hands or Feet

·       Sore Lips

·       Ulcers In the Mouth


Topoisomerase inhibitors stop DNA from copying itself by inhibiting the activity of an enzyme called topoisomerase. In addition to preventing cancer cells from proliferating, blocking this enzyme also harm the DNA of the cells.

Adult dose rangle: 100-200mg/m2/day for 5 days


  • feeling weak (asthenia)
  • nausea and vomiting
  • generally feeling unwell (malaise)
  • abdominal pain
  • damage to the liver (hepatotoxicity)



The development of cancer cells is inhibited by altretamine, leading to their eventual destruction. There will also be other impacts since altretamine also have an impact on the development of healthy body cells. After using the medication for months or years, certain side effects might not show up.

Treatment Market Player

Some of the major player of the treatments of the atypical teratoid are listed below:

Treatment Market Player

Treatment Product


CARBOWEL 150MG INJ(Carboplatin)



Carboplatin Injection


Xediton pharmaceutical Inc.

Vepesid® (Etoposide) Capsules


Dr Reddy



CARBOPLATIN (carboplatin)


U & V Cancure Private Limited


Accord Healthcare


Aetos Pharma

Carboplatin Injections BP 10mg/ml








Services Offered by DiseaseLandscape Insights in Atypical Teratoid Diseases Industry to grow exponentially.

Insights from the DiseaseLandscape Insights are useful for guiding and establishing commercial services for major international companies. It assists them with market analysis of product launch expansion, drug discovery and launch phase research and development, resource allocation, risk assessment of drug efficacy and safety, supply chain collaboration services, and long-term planning through insights into disease dynamics. In addition to providing comprehensive services for handling information related to clinical and medication safety problems, DiseaseLandscape Insight also specializes in medical writing, which entails producing scientific papers for a range of purposes, including documents pertaining to legislation and research, diseases, or pharmaceuticals. Educational and marketing materials, journal articles and publication abstracts, and content for websites catering to the healthcare sector.

Competitive Analysis

  • Leading businesses in the global market have been concentrating on new product releases and enhancing their testing skills in order to meet the increasing demand for early detection and strengthen their competitive position. Also, they have adopted several strategies like merger acquisition, collaboration and partnerships enhance and increase their product portfolio.
  • Roche a leader in the oncology product made a $250 million cash upfront payment, Roche has a final merger agreement to acquire Good Therapeutics, a biopharmaceutical business located in the United States.
  • Also, on the August 25, 2021, Eli Lilly and Company have made the strategic collaboration with Lycia Therapeutics to enter in the new market and to increases their portfolio which is directly impact on the revenue of companies also improve the standing in the market competition.

Market Trends Analysis

Several technical advancements and the adoption of synthetic biomarkers technologies for testing are driving the overall market growth. According to the National Institutes of Health a new type of cancer diagnostic tool called synthetic biomarkers employs an internal biosensor sensor to detect phenotypic changes in tumours early on and to enhance cancer-related signals to a very high level that is simple to measure. However, this diagnostic technology will help in early detection of the various cancers.

Clinical Assessment

The thrilling adventure into the field of Atypical Teratoid therapy begins in the DiseaseLandscape insights, where cutting-edge discoveries and extraordinarily exciting new opportunities will completely change the state of medicine.

DiseaseLandscape Insights helps in setting up and conducting clinical trials for new drugs and therapies, patient recruiting tactics, regulatory compliance, guaranteeing successful trial results, etc. The table below lists the study names of the ongoing clinical trials along with the phases in which they are presently being conducted.



A Phase 1/2 Study of Tiragolumab (NSC# 827799) and Atezolizumab (NSC# 783608) in Patients With Relapsed or Refractory SMARCB1 or SMARCA4 Deficient Tumors

A Phase 1/2 Study of Tiragolumab (NSC# 827799) and Atezolizumab (NSC# 783608) in Patients With Relapsed or Refractory SMARCB1 or SMARCA4 Deficient Tumors

TAZNI: A Phase I/II Combination Trial of Tazemetostat With Nivolumab and Ipilimumab for Children With INI1-Negative or SMARCA4-Deficient Tumors

TAZNI: A Phase I/II Combination Trial of Tazemetostat With Nivolumab and Ipilimumab for Children With INI1-Negative or SMARCA4-Deficient Tumors

Loc3CAR: Locoregional Delivery of B7-H3-specific Chimeric Antigen Receptor Autologous T Cells for Pediatric Patients With Primary CNS Tumors

Phase 2 Proof of Concept Study of Nivolumab and Ipilimumab in Children and Young Adults With Relapsed or Refractory INI1-negative Cancers

Phase 1 Study of B7-H3-Specific CAR T Cell Locoregional Immunotherapy for Diffuse Intrinsic Pontine Glioma/Diffuse Midline Glioma and Recurrent or Refractory Pediatric Central Nervous System Tumors

Phase 1/2 study to evaluate palbociclib (ibrance®) in combination with irinotecan and temozolomide or in combination with topotecan and cyclophosphamide in pediatric patients with recurrent or refractory solid tumors

Interleukin-15 Armored Glypican-3-specific Chimeric Antigen Receptor Expressing Autologous T Cells as Immunotherapy for Children With Solid Tumors

A Multi-Center Phase II Study of Selinexor in Treating Recurrent or Refractory Wilms Tumor and Other Pediatric Solid Tumors

Phase I Study of Autologous T Lymphocytes Expressing GD2-specific Chimeric Antigen and Constitutively Active IL-7 Receptors for the Treatment of Patients With GD2-expressing Brain Tumors (GAIL-B)

AflacST1502: A Phase II Study of Sirolimus in Combination With Metronomic Chemotherapy in Children With Recurrent and/or Refractory Solid and CNS Tumors

Phase I Study of Autologous T Lymphocytes Expressing GD2-specific Chimeric Antigen and Constitutively Active IL-7 Receptors for the Treatment of Patients With GD2-expressing Brain Tumors (GAIL-B)

A Randomized Phase I/II Study of Talazoparib or Temozolomide in Combination With Onivyde in Children With Recurrent Solid Malignancies and Ewing Sarcoma

A Randomized Phase I/II Study of Talazoparib or Temozolomide in Combination With Onivyde in Children With Recurrent Solid Malignancies and Ewing Sarcoma

NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) Screening Protocol



There are some obstacles in the market for atypical teratoid illnesses, but DiseaseLandscape Insights helps by offering solutions to these obstacles for industry participants. Diseases Landscape Insights provide the healthcare sector with insightful information that has been carefully chosen.

Important services for the business segment will be offered by Disease Landscape Insights. The help offered by Disease Landscape Insights makes it easier to set up and conduct clinical trials for new medications and therapies, as well as for patient recruitment strategies, regulatory compliance, ensuring successful trial outcomes, etc. 

DLI services are a driving force behind effective medication launches that provide positive results. The whole range of services provided by Disease Landscape Insights Services goes beyond information on treatment trends and illness prevalence.

Vishal SawantBusiness Development

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