This protocol applies to paediatric patients with MEN2B who carry the RET codon M918T mutation — the variant that places them in the American Thyroid Association's highest-risk (ATA-HST) category. This genetic profile is associated with a particularly aggressive disease course, and the timing of intervention is a defining factor in management.
In children with MEN2B and a RET codon M918T mutation (ATA-HST category), medullary thyroid carcinoma is characteristically highly aggressive. The ATA-HST designation reflects this recognised urgency and directly shapes the threshold and timing of surgical decision-making in this population.
Management in this highest-risk paediatric category centres on early surgical intervention, with the approach to the thyroid and associated structures guided by the child's age and the operative findings. The complete criteria, procedural sequence, and decision points — including considerations for adjacent structures — are detailed in the full protocol.
DOI: 10.1089/thy.2014.0335
In patients with MEN2B and a RET codon M918T mutation (ATA-HST category), the MTC is usually highly aggressive and if possible thyroidectomy should be performed early in life.
Children in the ATA-HST category with a RET codon M918T mutation should have a thyroidectomy in the first year of life, perhaps even in the first months of life.
In the absence of suspicious lymph nodes the performance of a central neck dissection should be based on whether the parathyroid glands can be identified and left in situ or autotransplanted.
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