Children with a confirmed germline RET codon C634 or A883F mutation represent a distinct, high-risk subgroup of medullary thyroid cancer that requires age-directed surgical planning guided by specific clinical findings.
This protocol applies to a paediatric patient carrying a RET codon C634 or RET codon A883F mutation. These mutations define the ATA-H risk category — separate from both the highest-risk ATA-HST group and the lower-risk ATA-MOD group — and carry specific implications for when and how surgical management should be undertaken.
Management in this ATA-H risk category is surgical. The timing of the procedure is determined by the child's age, and the extent of surgery may be influenced by biochemical and imaging findings present at evaluation. The full decision criteria and sequencing are detailed in the complete 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.
Children in the ATA-H category should have a thyroidectomy performed at age 5 years, or earlier based on the detection of elevated serum Ctn levels.
A central neck dissection should be performed in children with serum Ctn levels above 40 pg/mL, or with evidence on imaging or direct observation of lymph node metastases.
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