ATRT (Atypical Teratoid Rhabdoid Tumor)

A rare and rapidly progressive brain tumor seen in infants and young children.

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Neuro-Oncology: Brain Tumors

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1Overview

Quick and clear information for patients

What is this Condition?

ATRT is a rare and rapidly progressive brain tumor that occurs most commonly in infants and children under three years of age. It is associated with alterations in specific genes (SMARCB1 or SMARCA4) and may not respond easily to standard treatments. It can be located in both the lower part of the brain (cerebellum, brainstem) and upper regions; it has a tendency to spread through cerebrospinal fluid early on. Although its treatment is challenging, long-term results can be achieved in some children with intensive protocols combining surgery, chemotherapy, and radiation therapy at appropriate ages. This disease should be managed by experienced pediatric neuro-oncology centers by a multidisciplinary team.

Symptoms

Morning headache that worsens; constant restlessness and crying in infants

Projectile vomiting unrelated to feeding

Rapid head growth in infants, tension in the fontanelle

Unsteady gait, loss of sitting balance, clumsiness

Difficulty swallowing, double vision, facial asymmetry

Fatigue, drowsiness, rapidly progressive weakness

Diagnosis Methods

Contrast-enhanced brain MRI is the first-line diagnostic method; spinal cord MRI is also performed to assess for spinal involvement. Definitive diagnosis is established through pathological examination of tissue samples obtained during surgery and genetic testing (SMARCB1/SMARCA4 mutations). Cerebrospinal fluid analysis may also be used to evaluate dissemination.

Treatment Methods

The first and most important step in treatment is surgery aimed at removing the tumor as completely as possible within safe margins. This is followed by intensive chemotherapy; some children receive hematopoietic stem cell support. Radiation therapy may be considered in children over three years of age; in very young children, radiation therapy is deferred as much as possible to protect the developing brain. Families are also informed about appropriate clinical trials.

Maximal safe tumor resection surgery

Intensive multi-drug chemotherapy

Hematopoietic stem cell rescue following high-dose chemotherapy in suitable children

Radiation therapy in children over three years of age (proton therapy preferred when available)

Treatment Path

Related Treatments

The procedures and interventions most commonly explored with this condition.

4 links

Endoscopic Tumor Biopsy

Endoscopic Tumor Biopsy procedure

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Glial tümörler, internal dekompresyon, mikrocerrahi ile

Glial tümörler, internal dekompresyon, mikrocerrahi ile procedure

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Posterior fossa tümörleri

Posterior fossa tümörleri procedure

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Stereotaktik radyocerrahi (SRC) - Gamma Knife, Cyberknife

Stereotaktik radyocerrahi (SRC) - Gamma Knife, Cyberknife procedure

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References

Greenberg MS. Handbook of Neurosurgery. 10th ed. Thieme; 2023.
Di Rocco C, Pang D, Rutka JT, eds. Textbook of Pediatric Neurosurgery. Springer; 2020.
Kamenova M, et al. Human Brain and Spinal Cord Tumors: From Bench to Bedside. Volume 2. Cham: Springer; 2023.
Osborn AG, Hedlund GL, Salzman KL. Osborn's Brain: Imaging, Pathology, and Anatomy. 2nd ed. Elsevier; 2018.

This content is for informational purposes and based on academic sources; consult your physician for diagnosis and treatment.

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ATRT (Atypical Teratoid Rhabdoid Tumor)

Neuro-Oncology: Brain Tumors

1Overview

What is this Condition?

Symptoms

Diagnosis Methods

Treatment Methods

Related Treatments

Endoscopic Tumor Biopsy

Glial tümörler, internal dekompresyon, mikrocerrahi ile

Posterior fossa tümörleri

Stereotaktik radyocerrahi (SRC) - Gamma Knife, Cyberknife

References

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Related Treatments

Endoscopic Tumor Biopsy

Glial tümörler, internal dekompresyon, mikrocerrahi ile

Posterior fossa tümörleri

Stereotaktik radyocerrahi (SRC) - Gamma Knife, Cyberknife

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