What's multiple endocrine neoplasia type 1?
Multiple endocrine neoplasia type 1 is an inherited syndrome that predisposes to tumours of the parathyroid glands, the pituitary gland, and the hormone-producing cells of the pancreas and gut.
| Also indexed as | ORPHA:652, MONDO:0007540 |
|---|---|
| Features mapped | 8 |
| Treatments mapped | 1 |
| Published sources | 8 |
| Last reviewed | 2026-08-04 |
Signs and symptoms
Primary hyperparathyroidism
Primary hyperparathyroidism, in which the parathyroid glands become overactive, is a feature of multiple endocrine neoplasia type 1.
Hypergastrinemia
Higher-than-normal gastrin in the blood, called hypergastrinemia, occurs in multiple endocrine neoplasia type 1 in people who develop Zollinger-Ellison syndrome.
Pituitary adenoma
Multiple endocrine neoplasia type 1 has a classic triad of features: overactive parathyroid glands, neuroendocrine tumours of the digestive tract, and pituitary adenomas, which are growths in the pituitary gland. Most people who inherit the condition go on to develop these features.
Zollinger-Ellison syndrome
Zollinger-Ellison syndrome can occur in multiple endocrine neoplasia type 1, and some people who develop it go on to have surgery.
Insulinoma
Insulinomas, insulin-producing tumours of the pancreas, occur in multiple endocrine neoplasia type 1. In one surgical group they accounted for about a quarter of the pancreatic neuroendocrine tumours removed.
Thyroid carcinoma
Papillary thyroid carcinoma, a cancer of the thyroid gland, has been found in women with multiple endocrine neoplasia type 1, alongside breast and kidney cancers.
Adrenocortical carcinoma
Adrenocortical carcinoma, a cancer of the outer layer of the adrenal gland, is rare in multiple endocrine neoplasia type 1. Most adrenal growths in the condition are benign.
Hypercalcemia
High calcium in the blood occurs in multiple endocrine neoplasia type 1. It is usually addressed with surgery, most often before the age of 50.
Treatment and management
What the research describes, not a recommendation. Treatment decisions belong with your clinician.
This covers treatments that appear in the published research mapped here. Investigational and experimental therapies are not included, so their absence is a boundary of this map, not a sign they do not exist.
parathyroidectomy
Surgery to remove overactive parathyroid glands (parathyroidectomy) is a standard part of care for the primary hyperparathyroidism of MEN1.
Used to help with: Primary hyperparathyroidism.
“Cervical thymectomy should, therefore, be considered a standard component of care for multiple endocrine neoplasia type 1 patients undergoing parathyroidectomy for primary hyperparathyroidism.”
How to read the evidence labels
Where this comes from
This guide is built from 8 published source(s). Every claim above links back to one of them. Click any source ID to read the original on PubMed.
Take it further
Printed, source-linked documents built from this condition's graph — ready to bring to an appointment or attach to a coverage request. Every claim carries its published source, the same as this guide.