Alpha thalassemia/mental retardation syndrome X-linked (ATRX)
Alpha-thalassemia/mental retardation syndrome X-linked (ATRX) gene is an important genomic marker of gliomas and is either intact (ATRX wild-type) or mutated (ATRX-mutant) and is correlated with other important genomic markers including IDH, 1p19q codeletion and p53 expression 1,2.
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ATRX and IDH
Tumors with ATRX mutation (ATRX-mt) are usually associated with IDH positive (mutated) tumors, and when both are present a better prognosis can be expected over tumors with IDH1 positive but intact ATRX (ATRX-wt) 1-3.
ATRX and 1p19q co-deletion
Mutation of ATRX is almost never seen in patients with 1p/19q co-deletion (i.e. they are essentially mutually exclusive). Oligodendrogliomas will, therefore, have intact ATRX and 1p19q co-deletion whereas IDH-mt astrocytomas will usually, but not always, have ATRX-mt but no 1p19q co-deletion 1-3.
ATRX and p53 expression
ATRX-mt is usually associated with increased p53 expression 2.
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Astrocytic tumour
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astrocytic tumors
- WHO classification of CNS tumors
- WHO grading of CNS tumors
- VASARI MRI feature set
- diffuse astrocytoma grading
- grade I:
- grade II:
- chordoid glioma of the third ventricle
-
low-grade diffuse astrocytoma
- fibrillary astrocytoma (no longer recognised)
- protoplasmic astrocytoma (no longer recognised)
- gemistocytic astrocytoma
- oligoastrocytoma
- pilomyxoid astrocytoma
- pleomorphic xanthoastrocytoma
- grade III
- grade IV:
-
glioblastoma (GBM)
- glioblastoma IDH wildtype
- glioblastoma IDH mutant
- glioblastoma NOS
- variants
- diffuse midline glioma H3 K27M–mutant
-
glioblastoma (GBM)
- glioblastoma vs cerebral metastasis
- radiation-induced gliomas
- gliomatosis cerebri (growth pattern)
- specific locations
- treatment response
- prognostic genetic markers