Multiple endocrine neoplasia is a group of inherited syndromes in which tumors grow in several endocrine glands. MEN1 involves the parathyroids, pituitary and pancreas, while MEN2 involves medullary thyroid cancer, pheochromocytoma and, in MEN2A, the parathyroids.
Key symptoms
- Kidney stones, bone pain or fatigue from high calcium
- Headache, vision changes or nipple discharge from a pituitary tumor
- Stomach ulcers, diarrhea or low blood sugar from pancreatic tumors
- Spells of headache, sweating and palpitations from a pheochromocytoma
- Neck lump; family history of these tumors
What you find on exam
- Thyroid nodule or neck lymph nodes
- Blood pressure, often high or in spells
- Visual fields
- Tall, thin build and bumps on the lips or tongue in MEN2B
- Detailed family history across generations
Tests
- Calcium and PTH
- MEN1: prolactin, IGF-1, gastrin and fasting glucose
- MEN2: calcitonin, CEA and plasma metanephrines
- Genetic testing for the MEN1 or RET gene; imaging guided by the results
Treatment basics
- Remove a pheochromocytoma first, before any other surgery
- Parathyroid surgery for hyperparathyroidism
- Preventive thyroidectomy in RET carriers, timed by mutation risk
- Proton pump inhibitor and surgery for gastrinoma; drugs or surgery for pituitary tumors
- Screen and counsel family members
Red flags
- Hypertensive crisis, mainly during surgery or anesthesia
- Very high calcium with confusion
- Severe episodes of low blood sugar
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Sources: NCCPA PANCE Content Blueprint; NBOME COMLEX-USA clinical presentations; AAMC Core Entrustable Professional Activities (Core EPAs). Written by Making Synapses. Making Synapses is not affiliated with these organizations.