multiple endocrine neoplasia type-2B (MEN-2B)
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Introduction
Also see MEN-2.
Pathology
- features of MEN 2A except parathyroid disease
- medullary thyroid carcinoma may be present in the first year of life & commonly causes death in the 2nd or 3rd decade of life
- pheochromocytoma, often multiple
- ganglioneuromas (neural cell hyperplasia) of the lips, tongue & colon
- mucosal neuroma
- sometimes skeletal (Marfanoid) & eye abnormalities
Genetics
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]
More general terms
Additional terms
References
- ↑ Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 2052-55
- ↑ Jump up to: 2.0 2.1 Medical Knowledge Self Assessment Program (MKSAP) 11, 17, 18. American College of Physicians, Philadelphia 1998, 2015, 2018.
- ↑ Jump up to: 3.0 3.1 NEJM Knowledge+ Endocrinology
Patient information
multiple endocrine neoplasia type-2B patient information