All of the following are seen in MEN 2b EXCEPT:
Correct Answer: Hyperparathyroidism
Description: Hyperparathyroidism REF: Harrison's Internal Medicine 17th ed Chapter 345 Table 345-1, http://en.wikipedia.org/ wiki/Multiple_endocrine_neoplasia Feature MEN 1 MEN 2A MEN 2B Eponym Wermer syndrome Sipple syndrome Williams-Pollock syndrome, Gorlin-Vickers syndrome, and Wagenmann-Froboese syndrome Entero-Pancreatic tumors gastrinoma (50%), insulinoma (20%), vipoma, glucagonoma, PPoma ? Pituitary adenoma 66% - ? Parathyroid hyperplasia 90% 50% ? Medullary thyroid carcinoma 100% 85% Pheochromocytoma >33% 50% Marfanoid body habitus - 80% Mucosal neuroma 100% Gene(s) MEN1 RET RET Approx. prevalence 1 in 35,000 1 in 40,000 1 in 40,000 (1 in 20,000 to 1 in 40,000) Primary hyperparathyroidism is the most common manifestation of MEN1 Hyperparathyroidism is the earliest manifestation of the syndrome in most MEN1 patients. Enteropancreatic tumors are the second most common manifestation of MEN1 Gastrinomas are the most common enteropancreatic tumors observed in MEN1 patients Insulinomas are the second most common enteropancreatic tumors in patients who suffer from MEN1. Unlike gastrinomas, most insulinomas originate in the pancreas bed, becoming the most common pancreatic tumor in MEN1.
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