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Start free with EleplanMultiple endocrine neoplasia type 1
ORPHA:652Disease
Also called MEN1 · Wermer syndrome
What it is
A rare inherited cancer syndrome, characterized by the development of multiple neuroendocrine tumors of the parathyroids, gastro-entero-pancreatic tract, and anterior pituitary gland, and less commonly the adrenal cortical gland, thymus and bronchi, with other non-endocrine tumors in some patients.
Key facts
- Prevalence
- 1-9 / 100 000 (Europe)
- Age of onset
- All ages
- Inheritance
- Autosomal dominant, Not applicable
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Very common80–99%
5Common30–79%
18- Abdominal pain
- Adrenocortical abnormality
- Decreased male libido
- Diarrhea
- Galactorrhea
- Gastroesophageal reflux
- Hypercalciuria
- Hypergastrinemia
- Impotence
- Large cafe-au-lait macules with irregular margins
- Multiple lipomas
- Neoplasm of the pancreas
- Peptic ulcer
- Pituitary adenoma
- Pituitary prolactin cell adenoma
- Reduced bone mineral density
- Weight loss
- Zollinger-Ellison syndrome
Sometimes5–29%
37- Abnormal circulating aldosterone
- Adrenocortical carcinoma
- Amenorrhea
- Anorexia
- Carcinoid tumor
- Confetti-like hypopigmented macules
- Confusion
- Constipation
and 29 more in this range
Rare1–4%
14- Coma
- Elevated calcitonin
- Ependymoma
- Glucagonoma
- Increased serum serotonin
- Obtundation status
- Parathyroid carcinoma
- Pheochromocytoma
and 6 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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