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Multiple endocrine neoplasia type 1 (MEN-1 aka Wermer Syndrome) is one of a group of disorders, the multiple endocrine neoplasias, that affect the endocrine system through development of neoplastic lesions in pituitary, parathyroid gland and pancreas. [1]
Multiple Endocrine Neoplasia type 1 (MEN1) is a rare hereditary endocrine cancer syndrome characterized primarily by tumors of the parathyroid glands (95% of cases), endocrine gastroenteropancreatic (GEP) tract (30–80% of cases), and anterior pituitary (15–90% of cases). [19]
Menin is a putative tumor suppressor associated with multiple endocrine neoplasia type 1 (MEN-1 syndrome) and has autosomal dominant inheritance. [6] Variations in the MEN1 gene can cause pituitary adenomas, hyperparathyroidism, pancreatic neuroendocrine tumors, gastrinoma, and adrenocortical cancers.
1:30,000 Multiple endocrine neoplasia type 1 (Wermer's syndrome) MEN1: dominant Multiple endocrine neoplasia type 2: RET: dominant Muscular dystrophy: multiple AR, AD, X-linked Muscular dystrophy, Duchenne and Becker type: Myostatin-related muscle hypertrophy: MSTN: Myotonic dystrophy: DMPK, CNBP: dominant or T 1:8,000 Natowicz syndrome: HYAL1 ...
Multiple endocrine neoplasia type 1: Neurofibromatosis type 1: Neurofibromatosis type 1-like syndrome: Noonan syndrome: Peutz–Jeghers syndrome: Silver–Russell syndrome: Tuberous sclerosis: Von Hippel–Lindau disease
Familial isolated pituitary adenoma (FIPA) is a term that is used to identify a condition that displays an autosomal dominant inheritance and is characterised by the presence of two or more related patients affected by adenomas of the pituitary gland only, with no other associated symptoms that occur in multiple endocrine neoplasia type 1 (MEN ...
Most gastrinomas are sporadic (75–80%), whereas approximately 20–25% are associated with multiple endocrine neoplasia type 1 (MEN-1). [5] Over 50% of gastrinomas are malignant and can metastasize to regional lymph nodes and liver.
Nearly 25 percent of patients with gastrinomas have multiple tumors as part of a condition called multiple endocrine neoplasia type 1 (MEN 1). MEN I patients have tumors in their pituitary gland and parathyroid glands, in addition to tumors of the pancreas. [9]