MEN 1 syndrome is associated with all EXCEPT:
Correct Answer: Medullary carcinoma of thyroid
Description: ANSWER: (D) Medullary carcinoma of thyroidREF: Harrisons Internal Medicine 17th ed Chapter 345 Table 345-1, http://en.wikipedia.org/wiki/Multiple_endocrine_neoplasiaSome people think that option (D) was Pheochromocytoma not Medullary carcinoma of thyroid. However Table 345-1 of Harrison enumerates Pheochromocytoma as a rare possibility of MEN 1 but its text does not describes it like most other references.FeatureMEN 1MEN 2AMEN 2BEponymWermer syndromeSipplesyndromeWilliams-Pollock syndrome, Gorlin-Vickers syndrome, and Wagenmann-Froboese syndromeEntero-Pancreatic tumorsgastrinoma (50%',insulinoma (20%), vipoma, glucagonoma, PPoma--Pituitary adenoma66%--Parathyroid hyperplasia90%50%-Medullary thyroid carcinoma-100%85%Pheochromocytoma->33%50%Marfanoid body habitus--80%Mucosal neuroma--100%Gene(s)MEN1RETRETApprox. prevalence1 in 35.000 (1 in 20,000 to1 in 40,000)1 in 40,0001 in 40,000Primary hyperparathyroidism is the most common manifestation of MEN1Hyperparathyroidism is the earliest manifestation of the syndrome in most MEN1 patients.Enteropancreatic tumors are the second most common manifestation of MEN1Gastrinomas are the most common enteropancreatic tumors observed in MEN1 patientsInsulinomas are the second most common enteropancreatic tumors in patients who suffer from MENl. Unlike gastrinomas, most insulinomas originate in the pancreas bed, becoming the most common pancreatic tumor in MEN1.
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