Management of patients and subjects at risk for multiple endocrine neoplasia type 1: MEN 1. GENEM 1. Groupe d'Etude des Néoplasies Endocriniennes Multiples de type 1

Hormone Research
P ChansonA Murat

Abstract

Multiple endocrine neoplasia type 1 (MEN 1) is characterized by the combined occurrence, to variable degree, of hyperparathyroidism (HPT) (85.7% of cases according to the French Registry of GENEM 1), tumors of the endocrine pancreas (49.6%), pituitary adenomas (38.4%) and, less frequently, adrenal tumors (9.6%) and neuroendocrine tumors (5.8%). Currently, diagnosis of MEN 1 is done in the fourth decade of life, but familial screening (using genetic tools whose diagnostic accuracy approaches 100%) has lowered the age of diagnosis. Screening for MEN 1 in a patient harboring an apparently sporadic tumor will depend on the endocrine gland involved. Extensive screening for MEN 1 in the presence of HPT will be conducted only when the familial history is suggestive, when parathyroid glands are hyperplastic or when multiple parathyroid adenomas have been found at surgery. All patients with an endocrine pancreas tumor need to be investigated for the presence of other endocrine lesions of MEN 1. Extensive screening for MEN 1 is only recommended when a patient with a pituitary tumor or an adrenal tumor has a familial history suggestive of MEN 1. Otherwise regular measurement of blood calcium and PTH levels seem sufficient. Extensive scree...Continue Reading

Citations

Feb 15, 2000·Clinical Endocrinology·L B JohnstonM O Savage
Nov 16, 2001·The British Journal of Radiology·M P DebrayE Schouman-Claeys
Feb 19, 2005·Journal of Surgical Oncology·Maria A KouvarakiDouglas B Evans
Feb 4, 2006·The American Journal of Gastroenterology·Laurence Thomas-MarquesUNKNOWN Groupe des Tumeurs Endocrines (GTE)
Jan 1, 2012·Cancers·Francesco TonelliMaria Luisa Brandi
Apr 22, 2017·World Journal of Surgery·Francesco GiudiciMaria Luisa Brandi
Apr 29, 2006·The Japanese Journal of Thoracic and Cardiovascular Surgery : Official Publication of the Japanese Association for Thoracic Surgery = Nihon Kyōbu Geka Gakkai Zasshi·Yasuyuki KojimaKenji Inui
May 10, 2011·Endocrine Practice : Official Journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists·Francesco TonelliMaria Luisa Brandi
Jun 10, 2000·European Journal of Clinical Investigation·J F RoijersC J Lips

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