Multiple endocrine neoplasia type 1


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MEN 1

An inherited neoplasia syndrome characterised by: parathyroid hyperplasia (95%), pituitary adenoma (10%), pancreatic islet hyperplasia, adenoma or carcinoma, and/or adrenal cortical hyperplasia, adenoma and carcinoid tumours.

Molecular pathology
Mutation of MEN1 on chromosome 11.

Multiple endocrine neoplasia type 1 (MEN-1)

An inherited condition marked by multiple malignancies of the pituitary gland, parathyroid gland, and islet cells of the pancreas. About half of MEN-1 patients with pancreatic islet cell tumors will have gastrinomas, gastrin-producing tumors that lead to ulcer disease.
Mentioned in: Gastrinoma
References in periodicals archive ?
Asymptomatic children with multiple endocrine neoplasia type 1 mutations may harbor nonfunctioning pancreatic neuroendocrine tumors.
Multiple endocrine neoplasia type 1 gene maps to chromosome 11 and is lost in insulinoma.
MEN-1: Multiple endocrine neoplasia type 1, NECT: Neuroendocrine tumor, GHRH: Growth hormone releasing hormone, GH: Growth hormone, IGF-1: Insulin like growth factor.
Friedman, E., Larsson, C., Amorosi, A., Brandi, M.L., Bale, A., Metz, D., Jensen, R.T., Skarulis, M., Eastman, R.C., Nieman, L., Norton, J.A., Marx, S.J., "Multiple endocrine neoplasia type 1: Pathology, pathophysiology, and differential diagnosis," in The Parathyroids, ed.
Burgess, "Multiple endocrine neoplasia type 1 (MEN 1) is associated with an increased prevalence of diabetes mellitus and impaired fasting glucose," Clinical Endocrinology, vol.
Is the multiple endocrine neoplasia type 1 gene a suppressor for fundic argyrophil tumors in the Zollinger-Ellison syndrome?
Familial isoloated primary parathyroidism- a multiple endocrine neoplasia type 1 variant Eur J Endocrinol 2001; 145: 155-60.
Germline inactivating mutations of MEN1 gene are the main cause of multiple endocrine neoplasia type 1 (MEN1), a syndrome characterized by tumors in the pituitary gland, the parathyroid glands, and the enteropancreatic neuroendocrine tissues.
(8) However, previously reported cases of a concomitant parathyroid adenoma and a leiomyoma occurred only in patients with multiple endocrine neoplasia type 1. (9) Moreover, there has not been any documented case of a concomitant parathyroid adenoma and a laryngeal vascular leiomyoma.
Brandi, M.L., Marx, S.J., Aurbach, G.D., and Fitzpatrick, L.A., "Familial Multiple Endocrine Neoplasia Type 1: A new look at pathophysiology." Endocrine Reviews, vol.
(8) Similar to multiple endocrine neoplasia type 1 (MEN1) syndrome, patients with VHL disease can be associated with precursor lesions including ductuloinsular complexes (nesidioblastosis), islet dysplasia (referring to normal-sized or slightly enlarged islets <0.5 mm that contain neuroendocrine cells showing loss of the normal spatial and quantitative arrangement of the normal [alpha], [beta], [delta], and PP cell types), pancreatic micro-adenomatosis (when islet dysplasia is between 0.5-5 mm, it is termed microadenoma-, multiple microadenomas are termed microadenomatosis), and peliosis of the nontumorous islets (17) (Figure 2, A and B).

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