Gastrinomas are the most common symptomatic, malignant PET in patients with or without MEN1 and sufficient numbers of these patients have been entered into our protocols to allow systematic assessment. Studies are now underway, evaluating the natural history of these tumors including the a definition of factors determining prognosis and cause of death. Our studies have identified a cohort of 25% of patients in whom the gastrinomas have aggressive growth and 25% who have multiple endocrine neoplasia type 1 (MEN1). During this year information from our prospective studies was used to write a number of guideline and position papers with the North American Neuroendocrine Tumor Network and the European Neuroendocrine tumor network. In addition two surgical studies were completed including: a study of the effect of surgery in patients with negative preoperative imaging studies. This study demonstrated that tumors can be found in all patients, a proportion cured long-term;leading to the recommendation all of these patients should under exploration if no contraindications. A second study demonstrated for the first time that the presence of lymph node metastases only was associated with a poorer prognosis in patients with gastrinomas and that an increasing number of positive lymph nodes had proportional increased prognostic effect. Furthermore, a number of consensus conferences and position papers on classification/treatment of various disease aspects were published as well as a collaborative study of the need for preoperative neck imaging in patients with hyperparathyroidism and MEN1.

Project Start
Project End
Budget Start
Budget End
Support Year
21
Fiscal Year
2012
Total Cost
$333,486
Indirect Cost
City
State
Country
Zip Code
Ito, Tetsuhide; Jensen, Robert T (2016) Imaging in multiple endocrine neoplasia type 1: recent studies show enhanced sensitivities but increased controversies. Int J Endocr Oncol 3:53-66
Falconi, M; Eriksson, B; Kaltsas, G et al. (2016) ENETS Consensus Guidelines Update for the Management of Patients with Functional Pancreatic Neuroendocrine Tumors and Non-Functional Pancreatic Neuroendocrine Tumors. Neuroendocrinology 103:153-71
Ito, Tetsuhide; Lee, Lingaku; Jensen, Robert T (2016) Treatment of symptomatic neuroendocrine tumor syndromes: recent advances and controversies. Expert Opin Pharmacother :1-15
Nilubol, Naris; Weinstein, Lee S; Simonds, William F et al. (2016) Limited Parathyroidectomy in Multiple Endocrine Neoplasia Type 1-Associated Primary Hyperparathyroidism: A Setup for Failure. Ann Surg Oncol 23:416-23
Ito, Tetsuhide; Hijioka, Susumu; Masui, Toshihiko et al. (2016) Advances in the diagnosis and treatment of pancreatic neuroendocrine neoplasms in Japan. J Gastroenterol :
Oberg, Kjell; Krenning, Eric; Sundin, Anders et al. (2016) A Delphic consensus assessment: imaging and biomarkers in gastroenteropancreatic neuroendocrine tumor disease management. Endocr Connect 5:174-87
Lee, Lingaku; Igarashi, Hisato; Fujimori, Nao et al. (2015) Long-term outcomes and prognostic factors in 78 Japanese patients with advanced pancreatic neuroendocrine neoplasms: a single-center retrospective study. Jpn J Clin Oncol :
Norton, Jeffrey A; Krampitz, Geoffrey; Jensen, Robert T (2015) Multiple Endocrine Neoplasia: Genetics and Clinical Management. Surg Oncol Clin N Am 24:795-832
Norton, Jeffrey A; Krampitz, Geoffrey; Zemek, Allison et al. (2015) Better Survival But Changing Causes of Death in Patients With Multiple Endocrine Neoplasia Type 1. Ann Surg 261:e147-8
Lesurtel, Mickael; Nagorney, David M; Mazzaferro, Vincenzo et al. (2014) When should a liver resection be performed in patients with liver metastases from neuroendocrine tumours? A systematic review with practice recommendations. HPB (Oxford) :

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