MYCN amplification
MYCN amplification, more than four times the normal copy number of the gene on FISH, marks the most aggressive fifth of neuroblastomas and puts a child in the high-risk group whatever their age or stage. No drug targets it; it decides how much treatment is given.
Overview
The INRG and COG definitions call MYCN amplified when the signal count is more than four times that of the chromosome 2 reference (or more than 10 copies), by FISH on tumour or bone marrow. Amplification is present in about 20 percent of neuroblastomas and assigns high risk in the INRG classification irrespective of other features (except some stage L1 tumours), triggering induction chemotherapy, surgery, tandem transplant, radiotherapy and anti-GD2 immunotherapy with dinutuximab. Dinutuximab's label describes high-risk neuroblastoma without naming MYCN. Amplification also defines a spinal ependymoma subtype and is found in some medulloblastomas.
- Target · the protein and the cell it sits on
- Drug · antibody, small molecule, cell or radioligand
- Effect · signal, damage or kill
In plain words · MYCN is a growth-driving gene that some neuroblastomas copy many times over; that amplification is one of the strongest signs the tumour is aggressive and sets the intensity of treatment.
If your child's neuroblastoma is MYCN-amplified it is treated as high risk with the full programme of chemotherapy, surgery, stem cell rescue, radiotherapy and immunotherapy, even when the tumour is small or the child is young. There is no drug aimed at MYCN itself yet, though trials of indirect approaches are open.
Written only from the label or guideline text cited on this page. Not medical advice; your own report and the reading your team gives it come first.
MYCN signal count more than four times the reference (chromosome 2 centromere or a control probe) by FISH, or more than 10 copies by molecular methods; gain below that is not amplification.
“MYCN status: amplified versus not amplified is a criterion of the International Neuroblastoma Risk Group (INRG) classification”
Cohn et al., The International Neuroblastoma Risk Group (INRG) classification system, J Clin Oncol 2009No approval uses this readout as a threshold. It is defined by INRG classification system (Cohn et al., J Clin Oncol 2009).
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