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multiple endocrine neoplasia

MEN stands for: multiple endocrine neoplasia
MEN is: disorders of endocrine excess; group of hereditary syndromes characterized by aberrant growth of malignant and benign tumors in multiple endocrine glands
MEN syndromes include: Wermer syndrome (MEN-1); Sipple syndrome (MEN-2a); Mucosal neuroma syndrome (MEN-2b)
Wermer syndrome (MEN-1) 3 P's: pituitary, parathyroid, pancreas (also facial angiofibromas)
Sipple syndrome (MEN-2a): medullary thyroid carcinoma, parathyroid, pheochromocytoma
Mucosal neuroma syndrome (MEN-2b): medullary thyroid carcinoma, pheochromocytoma, mucosal and GI ganglioneuromas, (parathyroid tumor is rare)
Which is more common: MEN-1 or MEN-2? MEN-1
Which is more common: MEN-2a or MEN-2b? MEN-2a
Which of the 3 MEN forms is more aggressive with a worse prognosis? MEN-2b
MEN-1 treatment hypercalcemia (surgery); gastrinoma (PPIs); insulinoma (surgery, diazoxide); glucagonoma (surgery); prolactinoma (dopamine agonist); GH (trans-sphenoidal surgery)
Most common clinical manifestation of MEN-2: medullary thyroid carcinoma
Second most common clinical manifestation of MEN-2: pheochromocytoma
Common in MEN-2a but not in MEN-2b: parathyroid tumors
MEN-2 treatment total thyroidectomy with radical lymph node dissection (for medullary thyroid carcinoma); surgical excision under alpha-adrenergic blockade (for pheochromocytoma)
Created by: Carrie D.