MEN2B 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

Patients with MEN2B syndrome have worse outcomes than those with MEN2A syndrome, primarily because medullary thyroid carcinoma is more aggressive. Because of the increased severity of medullary thyroid carcinoma in children with MEN2B syndrome and in those with RET variants in codons 883, 918, and 922, it is recommended that these children undergo prophylactic thyroidectomy in infancy.[3,16,17]; [18][Level of evidence C2] This therapy can improve outcomes in patients with MEN2B syndrome.[19] Complete removal of the thyroid gland is recommended because of a high incidence of bilateral disease.

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