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Chapter-060 Multiple Endocrine Neoplasia Type 1

BOOK TITLE: ESI Manual of Clinical Endocrinology

Author
1. Thomas Nihal
2. Paul Thomas V
3. Asha HS
ISBN
9789351526476
DOI
10.5005/jp/books/12535_75
Edition
2/e
Publishing Year
2015
Pages
13
Author Affiliations
2. Christian Medical College, Vellore, Tamil Nadu, India
3. Christian Medical College, Vellore, Tamil Nadu, India
Chapter keywords
Multiple endocrine neoplasia-1, autosomal dominant disorder, parathyroid denomas, tumor suppressor gene, hyperparathyroidism, carcinoid tumors, endocrine tumors, cutaneous tumors

Abstract

Multiple endocrine neoplasia-1 is an autosomal dominant disorder that is characterized by the combination of tumors occurrence in the parathyroids, pancreatic, islets, and anterior pituitary. Men-1 syndrome is caused because of the mutations that occurred in MEN-1 tumor suppressor gene located on the 11q13.5 chromosome. Diagnosis for primary hyperparathyroidism, pancreatic endocrine tumors, and pituitary tumors are briefly described. This chapter also describes the treatments for primary hyperparathyroidism (PHPT), pancreatic neuroendocrine tumors (PNETs), pituitary tumors, and associated tumors like carcinoid tumors, adrenal tumors, CNS tumors, thyroid tumors, cutaneous tumors, and screening of relatives. For confirming the diagnosis of those affected and is also for identifying the relatives who are at risk of affecting with MEN-1 can be detected by using genetic testing method.

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