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multiple endocrine neoplasia type-2B (MEN-2B)

Also see MEN-2. Pathology: 1) features of MEN 2A except parathyroid disease 2) medullary thyroid carcinoma may be present in the first year of life & commonly causes death in the 2nd or 3rd decade of life 3) pheochromocytoma, often multiple 4) ganglioneuromas (neural cell hyperplasia) of the lips, tongue & colon 5) mucosal neuroma 6) sometimes skeletal (Marfanoid) & eye abnormalities Genetics: - associated with defects in RET, exon 16, codon 918 [2] Laboratory: - serum calcium: hypercalcemia is rare - ret gene mutation Management: - GLP-1 receptor agonists (incretin mimetics, glutides) are contraindicated in patients with medullary thyroid cancer or MEN2 [3]

Related

medullary thyroid carcinoma

General

multiple endocrine neoplasia type-2 (MEN-2)

Properties

ASSOCIATED-NEOPLASM[S]: pheochromocytoma ganglioneuroma medullary thyroid carcinoma

Database Correlations

OMIM correlations MORBIDMAP 164761

References

  1. Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 2052-55
  2. Medical Knowledge Self Assessment Program (MKSAP) 11, 17, 18. American College of Physicians, Philadelphia 1998, 2015, 2018.
  3. NEJM Knowledge+ Endocrinology