Children carrying a germline RET codon M918T mutation in the setting of MEN2B face a particularly aggressive form of medullary thyroid cancer. This clinical scenario requires a specific, time-sensitive management approach that differs from other RET mutation categories.
Clinical scenario: A child with MEN2B and a confirmed RET codon M918T germline mutation. This mutation places patients in the highest-risk category (ATA-HST), where medullary thyroid cancer is typically highly aggressive and early intervention is critical.
The structured protocol for this scenario centres on surgical intervention early in life — with timing considerations that go beyond standard paediatric oncology timelines. Neck management is included in the protocol, with specific criteria guiding the extent of dissection. The complete sequence, surgical decision criteria, and post-operative management are detailed in the full protocol.
DOI: 10.1089/thy.2014.0335
The ATA-HST category includes patients with MEN2B and the RET codon M918T mutation, the ATA-H category includes patients with RET codon C634 mutations and the RET codon A883F mutation, and the ATA-MOD category includes patients with RET codon mutations other than M918T, C634, and A883F.
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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