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Multiple endocrine neoplasia type 1

ORPHA:652· ICD-10 D44.8

Definition

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.

Prevalence
1-9 / 100 000
Inheritance
Autosomal dominant, Not applicable
Age of onset
All ages