Multiple Endocrine Neoplasia: Genetics and Clinical Management.

Surgical oncology clinics of North America

PubMedID: 26363542

Norton JA, Krampitz G, Jensen RT. Multiple Endocrine Neoplasia: Genetics and Clinical Management. Surg Oncol Clin N Am. 2015;24(4):795-832.
Early diagnosis of multiple endocrine neoplasia (MEN) syndromes is critical for optimal clinical outcomes; before the MEN syndromes can be diagnosed, they must be suspected. Genetic testing for germline alterations in both the MEN type 1 (MEN1) gene and RET proto-oncogene is crucial to identifying those at risk in affected kindreds and directing timely surveillance and surgical therapy to those at greatest risk of potentially life-threatening neoplasia. Pancreatic, thymic, and bronchial neuroendocrine tumors are the leading cause of death in patients with MEN1 and should be aggressively considered by at least biannual computed tomography imaging.