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Start free with EleplanMultiple endocrine neoplasia type 4
ORPHA:276152Disease
Also called MEN4
What it is
Multiple endocrine neoplasia type 4 (MEN4) is a very rare form of MEN, an inherited cancer syndrome, characterized by parathyroid and anterior pituitary tumors, possibly associated with adrenal, renal, and reproductive organ tumors.
Key facts
- Age of onset
- Adult
- Inheritance
- Autosomal dominant, Not applicable
- Classified as
- Disease
Recorded for the broader condition
- Prevalence
- <1 / 1 000 000 (annual incidence, Ireland)Multiple endocrine neoplasia
Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.
Signs and symptoms
Very common80–99%
6Common30–79%
21- Abnormality of pancreas physiology
- Adrenocortical adenoma
- Angiofibromas
- Diarrhea
- Elevated circulating growth hormone concentration
- Episodic abdominal pain
- Esophagitis
- Fasting hyperinsulinemia
- Hyperinsulinemic hypoglycemia
- Insulinoma
- Neuroendocrine neoplasm
- Peptic ulcer
- Pituitary adenoma
- Pituitary growth hormone cell adenoma
- Pituitary null cell adenoma
- Pituitary prolactin cell adenoma
- Pulmonary carcinoid tumor
- Renal angiomyolipoma
- Subcutaneous lipoma
- Thyroid adenoma
- Zollinger-Ellison syndrome
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.
Gene
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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