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C R C Pieterman, E B Conemans, K M A Dreijerink, J M de Laat, H Th M Timmers, M R Vriens, and G D Valk

Introduction Thoracic and duodenopancreatic neuroendocrine tumors (dpNETs) can occur either sporadically or as a manifestation of an inherited syndrome, most importantly the multiple endocrine neoplasia type 1 (MEN1) syndrome. This is an autosomal

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A A Pannett and R V Thakker

Combined clinical and laboratory investigations of multiple endocrine neoplasia type 1 (MEN1) have resulted in an increased understanding of this disorder which may be inherited as an autosomal dominant condition. Defining the features of each disease manifestation in MEN1 has improved patient management and treatment, and has also facilitated a screening protocol to be instituted. The application of the techniques of molecular biology has enabled the identification of the gene causing MEN1 and the detection of mutations in patients. The function of the protein encoded by the MEN1 gene has been shown to be in the regulation of JunD-mediated transcription but much still remains to be elucidated. However, these recent advances provide for the identification of mutant MEN1 gene carriers who are at a high risk of developing this disorder and thus require regular and biochemical screening to detect the development of endocrine tumours.

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Xiao-Hua Jiang, Jie-Li Lu, Bin Cui, Yong-Ju Zhao, Wei-qing Wang, Jian-Min Liu, Wen-Qiang Fang, Ya-Nan Cao, Yan Ge, Chang-xian Zhang, Huguette Casse, Xiao-Ying Li, and Guang Ning

Introduction Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant inherited disorder characterized by frequent occurrence of tumours of parathyroid, pancreatic islet and anterior pituitary, sometimes in combination with other rare

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P H Kann, E Balakina, D Ivan, D K Bartsch, S Meyer, K-J Klose, Th Behr, and P Langer

T, Celik I, Rothmund M & Nies C 2000 Pancreaticoduodenal endocrine tumors in multiple endocrine neoplasia type 1: surgery or surveillance? Surgery 128 958 –966. Bolondi L , Li Bassi S, Gaiani S, Campione O, Marrano

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Brian Harding, Manuel C Lemos, Anita A C Reed, Gerard V Walls, Jeshmi Jeyabalan, Michael R Bowl, Hilda Tateossian, Nicky Sullivan, Tertius Hough, William D Fraser, Olaf Ansorge, Michael T Cheeseman, and Rajesh V Thakker

Introduction Multiple endocrine neoplasia type 1 (MEN1), in man, is characterised by the combined occurrence of tumours of parathyroids, pancreatic islets and anterior pituitary ( Marx 2005 , Thakker 2006 ). Some patients may also develop adrenal

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Nader Hussein, JieLi Lu, Huguette Casse, Sandra Fontanière, Anne-Marie Morera, Séverine Mazaud Guittot, Alain Calender, Nathalie Di Clemente, and Chang X Zhang

Introduction The patients with multiple endocrine neoplasia type 1 (MEN1), a hereditary syndrome transmitted with an autosomal dominant trait, predispose to the occurrence of multiple endocrine tumours of the parathyroids, pancreas, anterior

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Jerena Manoharan, Max B Albers, and Detlef K Bartsch

PH Bartsch DK 2017 Limited value of Ga-68-DOTATOC-PET-CT in routine screening of patients with multiple endocrine neoplasia Type 1 . World Journal of Surgery 41 1521 – 1527 . ( doi:10.1007/s00268-017-3907-9 ) 10.1007/s00268

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Elizabeth Grubbs, Daniel Halperin, Steven G Waguespack, and Robert F Gagel

Overview The multiple endocrine neoplasia syndromes are a varied group of disorders that include multiple endocrine neoplasia types 1 and 2 (MEN 1, MEN 2), von Hippel-Lindau (VHL) disease , Carney complex (CC), hereditary pheochromocytoma

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S M Sadowski, G Cadiot, E Dansin, P Goudet, and F Triponez

with thymic carcinoids and multiple endocrine neoplasia type 1 syndrome at MD Anderson Cancer Center, volume 7, 2016, pages 279–287, Christakis I, Qiu W, Silva Figueroa AM, Hyde S, Cote GJ, Busaidy NL, Williams M, Grubbs E, Lee JE & Perrier ND. With

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Rachel S van Leeuwaarde, Joanne M de Laat, Carolina R C Pieterman, Koen Dreijerink, Menno R Vriens, and Gerlof D Valk

Introduction Multiple endocrine neoplasia type 1 (MEN1) (OMIM 131100) is a rare autosomal inherited disorder associated with a high risk for patients to simultaneously develop tumors of the parathyroid glands, duodenopancreatic neuroendocrine