J Korean Neurosurg Soc.  2018 May;61(3):302-311. 10.3340/jkns.2018.0061.

Atypical Teratoid Rhabdoid Tumour : From Tumours to Therapies

• Richardson EA
• Ho B
• Huang A

• KMID: 2417355
• DOI: http://doi.org/10.3340/jkns.2018.0061

Abstract

Atypical teratoid rhabdoid tumours (ATRTs) are the most common malignant central nervous system tumours in children ≤1 year of age and represent approximately 1-2% of all pediatric brain tumours. ATRT is a primarily monogenic disease characterized by the bi-allelic loss of the SMARCB1 gene, which encodes the hSNF5 subunit of the SWI/SNF chromatin remodeling complex. Though conventional dose chemotherapy is not effective in most ATRT patients, high dose chemotherapy with autologous stem cell transplant, radiotherapy and/or intrathecal chemotherapy all show significant potential to improve patient survival. Recent epigenetic and transcriptional studies highlight three subgroups of ATRT, each with distinct clinical and molecular characteristics with corresponding therapeutic sensitivities, including epigenetic targeting, and inhibition of tyrosine kinases or growth/lineage specific pathways.

Keyword

Brain neoplasms; Rhabdoid tumour; SMARCB1 protein, Human; Antineoplastic agent; Epigenomics; Protein-tyrosine kinases

MeSH Terms

Brain
Brain Neoplasms
Central Nervous System
Child
Chromatin Assembly and Disassembly
Drug Therapy
Epigenomics
Humans
Phosphotransferases
Protein-Tyrosine Kinases
Radiotherapy
Stem Cells
Tyrosine
Phosphotransferases
Protein-Tyrosine Kinases
Tyrosine

Figure

• 

  Fig. 1. Summary of clinical, epigenetic and therapeutic sensitivity of ATRT subgroups. ATRT-SHH : sonic hedgehog subgroup, ATRT-TYR : tyrosinase subgroup, ATRT-MYC : MYC subgroup.

Reference

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