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← Childhood Multiple Endocrine Neoplasia (MEN) Syndromes

HEALTH PROFESSIONAL · SOURCE READING

MEN2A syndrome

Source: Childhood Multiple Endocrine Neoplasia (MEN) Syndromes Treatment (PDQ®)–Health Professional Version, National Cancer Institute.

Source updated: April 3, 2025 · Captured 2026-09-09.

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Context: Treatment of Childhood Multiple Endocrine Neoplasia (MEN) Syndromes / MEN Type 2 (MEN2) Syndromes

For children with MEN2A syndrome, thyroidectomy is commonly performed by approximately age 5 years or older if that is when a RET variant is identified.[2-7] The outcomes for patients with MEN2A syndrome are generally good, although medullary thyroid carcinoma and pheochromocytoma can recur.[8-10]

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A retrospective analysis identified 262 patients with MEN2A syndrome.[11] The median age of the cohort was 42 years and ranged from age 6 to 86 years. There was no correlation between the specific RET variant identified and the risk of distant metastasis. Younger age at diagnosis increased the risk of distant metastasis.

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Young children who are relatives of patients with MEN2A syndrome undergo genetic testing before the age of 5 years. Carriers undergo total thyroidectomy as described above, with autotransplant of one parathyroid gland by a certain age.[12-15]

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Preserved source evidence · Not medical advice