Onkologie up2date, Table of Contents Onkologie up2date 2020; 2(01): 19-29DOI: 10.1055/a-1023-5221 Tumoren der endokrinen Organe Georg Thieme Verlag KG Stuttgart · New York Multiple endokrine Neoplasie Ludwig Schaaf , Friedhelm Raue Recommend Article Abstract Buy Article All articles of this category Die multiple endokrine Neoplasie (MEN) gehört zu den autosomal-dominant vererbbaren Tumorsyndromen. Wegen Mutationen im MEN-1-Gen bzw. im RET-Protoonkogen entwickeln MEN-Patienten benigne und maligne endokrine Tumoren – teilweise bereits im Kindesalter. Dieser Beitrag schildert, wie der gezielte Einsatz der molekulargenetischen Untersuchung und der spezifischen Tumormarker eine Frühdiagnose und ggf. Heilung der malignen Tumoren ermöglicht. Full Text References Literatur 1 Thakker RV, Newey PJ, Walls GV. et al. Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1). J Clin Endocrinol Metab 2012; 97: 2990-3011 2 Machens A, Schaaf L, Karges W. et al. Age-related penetrance of endocrine tumours in multiple endocrine neoplasia type 1 (MEN1): a multicentre study of 258 gene carriers. Clin Endocrinol (Oxf) 2007; 67: 613-622 3 Pieterman CR, Conemans EB, Dreijerink KM. et al. Thoracic and duodenopancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1: natural history and function of menin in tumorigenesis. Endocr Relat Cancer 2014; 21: R121-R142 4 Thosani S, Ayala-Ramirez M, Palmer L. et al. The characterization of pheochromocytoma and its impact on overall survival in multiple endocrine neoplasia type 2. J Clin Endocrinol Metab 2013; 98: E1813-E1819 5 Castagna MG, Fugazzola L, Maino F. et al. Reference range of serum calcitonin in pediatric population. J Clin Endocrinol Metab 2015; 100: 1780-1784 6 Manoharan J, Raue F, Lopez CL. et al. Is routine screening of young asymptomatic MEN1 patients necessary?. World J Surg 2017; 41: 2026-2032 7 Falconi M, Eriksson B, Kaltsas G. et al. ENETS Consensus Guidelines Update for the Management of Patients with Functional Pancreatic Neuroendocrine Tumors and Non-Functional Pancreatic Neuroendocrine Tumors. Neuroendocrinology 2016; 103: 153-171 8 Thakker RV. Multiple endocrine neoplasia type 1 (MEN1) and type 4 (MEN4). Mol Cell Endocrinol 2014; 386: 2-15 9 Wells jr. SA, Asa SL, Dralle H. et al. Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma. Thyroid 2015; 25: 567-610 10 Raue F, Frank-Raue K. Update multiple endocrine neoplasia type 2. Fam Cancer 2010; 9: 449-457 11 Krampitz GW, Norton JA. RET gene mutations (genotype and phenotype) of multiple endocrine neoplasia type 2 and familial medullary thyroid carcinoma. Cancer 2014; 120: 1920-1931 12 Frank-Raue K, Rondot S, Raue F. Molecular genetics and phenomics of RET mutations: Impact on prognosis of MTC. Mol Cell Endocrinol 2010; 322: 2-7 13 Wells jr. SA, Pacini F, Robinson BG. et al. Multiple endocrine neoplasia type 2 and familial medullary thyroid carcinoma: an update. J Clin Endocrinol Metab 2013; 98: 3149-3164 14 Kratzsch J, Petzold A, Raue F. et al. Basal and stimulated calcitonin and procalcitonin by various assays in patients with and without medullary thyroid cancer. Clin Chem 2011; 57: 467-474 15 Links TP, Verbeek HH, Hofstra RM. et al. Endocrine tumours: progressive metastatic medullary thyroid carcinoma: first- and second-line strategies. Eur J Endocrinol 2015; 172: R241-R251
