• Also known as Wermer syndrome
• Characterized by tumors of the parathyroid, anterior pituitary, and pancreas
• Tumors may develop synchronously or metachronously, may be benign or malignant, and may be hyperplasia, adenomas, or carcinoma
• Pancreatic tumors include functioning and nonfunctioning islet tumors such as gastrinoma, insulinoma, glucagonoma, VIPoma, somatostatinoma, pancreatic polypeptide tumors (PPomas)
• Other tumors in MEN 1 can include adrenocortical tumors, thymic or bronchial carcinoid tumors, multiple lipomas, cutaneous angiofibromas and collagenomas
• Syndrome is transmitted as autosomal dominant trait
• Most common mutation is in the menin gene on 11q13
• Trait has 100% penetrance but variable expressivity
• Syndrome often develops in the third and fourth decade of life
• Male:female ratio is 1:1
• No known racial predilection
• 90-97% of patients have biochemical evidence of hyperparathyroidism
• 30-80% manifest pancreatic islet cell tumors
• 15-50% develop pituitary tumors
• Gastrinomas with MEN 1 account for 20% of all cases of Zollinger-Ellison syndrome
• Presentation depends on endocrine tissue involved and the overproduction of a specific hormone; symptoms may arise from tumor mass itself
• Dyspepsia, abdominal pain, hematemesis
• Syncope, tremulousness, diaphoresis, confusion, dizziness (hypoglycemia)
• Symptoms of hypercalcemia, renal stones
• Headaches, visual field defects, amenorrhea, galactorrhea, hypogonadism, acromegaly (pituitary dysfunction)
• Elevated serum calcium and intact parathyroid hormone
• Elevated gastric acid secretion; elevated fasting serum gastrin level
• Supervised fast with resultant hypoglycemia, elevated serum insulin level, and elevated serum C-peptide level
• Elevated prolactin level
• Direct genetic testing generally offered only for probands with definitive evidence of the MEN 1 syndrome; once the mutation is known, then direct genetic testing of at-risk family members is useful
• CT scan, angiography, or somatostatin receptor scintigraphy to evaluate for pancreatic islet cell tumors
• Sestamibi scan, cervical US, CT, or selective venous sampling to evaluate location of abnormality for persistent or recurrent hyperparathyroidism
• Annual surveillance for adult gene carriers should include:
-History and physical exam
-Calcium and parathyroid hormone measurement for hyperparathyroidism
-Gastrin, pancreatic polypeptide, abdominal CT scan and somatostatin receptor scintigraphy for pancreatic tumor detection
-Prolactin level for pituitary adenoma
-Chest CT scan for thymic carcinoid tumor detection
• Parathyroid disease treated with surgery
• Treat parathyroid disease first
• Pituitary tumors may require surgical ablation or irradiation but usually are managed medically
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