This protocol covers Multiple endocrine neoplasia type 2A (MEN2A) in paediatric patients who carry a RET codon C634 germline mutation and are classified in the ATA high-risk (ATA-H) category. Risk stratification by mutation type is central to determining the timing and scope of management in this population.
Children in the ATA-H category should have a thyroidectomy performed at age 5 years, or earlier based on the detection of elevated serum calcitonin levels. The ATA-H designation reflects the elevated risk conferred by the RET C634 mutation and directly governs the recommended intervention timeline.
The primary intervention for children in this risk category is surgical. The precise extent of surgery is guided by specific biochemical and clinical criteria — the complete decision criteria and full protocol are available via the link below.
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.
DOI: 10.1089/thy.2014.0335
View source ↗