Question | Answer |
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) |