Pancreatic neuroendocrine tumors in MEN1 disease: a mono-centric longitudinal and prognostic study
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CLINICAL MANAGEMENT OF ENDOCRINE DISEASES
Pancreatic neuroendocrine tumors in MEN1 disease: a monocentric longitudinal and prognostic study S. Chiloiro1 F. Lanza2 A. Bianchi1 G. Schinzari3 M. G. Brizi2 A. Giampietro1 V. Rufini4 F. Inzani5 A. Giordano4 G. Rindi5 A. Pontecorvi1 L. De Marinis1 ●
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Received: 17 January 2017 / Accepted: 12 May 2017 © Springer Science+Business Media New York 2017
Abstract Purpose Multiple endocrine neoplasia type 1 (MEN1) is an inherited endocrine neoplastic syndrome associated with a greater risk of endocrine tumor development like pancreatic neuroendocrine tumors (p-NET), with different clinical characteristics from sporadic ones. This paper aims to compare clinical, hystological and morphological aspects of p-NET in patients affected from MEN1 (MEN1+) and notaffected ones (MEN1−). Methods We performed a retrospective observational study. Data was collected between December 2010 and December 2015, including patients with a histological diagnosis of pNET and radiological imaging. We compared clinical, histological, radiological, and prognostic aspects of MEN+ pNET with MEN−1 p-NET. Results Of the 45 patients enrolled, 13 MEN1+ and 21 MEN1− cases were analyzed. Frequency of not secreting pNETs and insulin secreting p-NETs, histopathological grades and Ki67 expression were superimposable between MEN1+ and MEN1− patients. MEN1+ pNETs are more
* L. De Marinis [email protected] 1
Department of Endocrinology, Catholic University of the Sacred Heart, Rome, Italy
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Department of Radiology, Catholic University of the Sacred Heart, Rome, Italy
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Department of Oncology, Catholic University of the Sacred Heart, Rome, Italy
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Department of Nuclear Medicine, Catholic University of the Sacred Heart, Rome, Italy
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Department of Anatomic Pathology, Catholic University of the Sacred Heart, Rome, Italy
often multicentric compared to MEN1− pNETs. Frequency of liver and nodes metastatic spread was higher in MEN1− p-NET compared to MEN1+ p-NET. Analyzing p-NET according to the disease outcome, we found that recovered and stable p-NETs in MEN1+ patients, compared to MEN1− cases, are diagnosed at lower age (p = 0.04/p = 0.002) and that are more frequently multifocal lesions (p = 0.009/ p = 0.002). Conclusions In our study pNETs in MEN1+ and pNETs in MEN1− don’t significantly differ for prognosis but only for clinical features. p-NET stage disease and prognosis can be positively influenced by early diagnosis and screening in index patients’ first-degree relatives Keywords Neuroendocrine tumor MEN1 Pancreas pNET ●
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Introduction Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominantly inherited endocrine neoplastic syndrome characterized by higher risk of tumor development in endocrine organs [1, 2]. Clinical characteristics of gastrointestinal neuroendocrine tumors (NETs) in MEN1 syndrome differ from sporadic ones [3, 4]. Natural history of MEN-1 related NETs isn’t completely known as few patients were studied prospectively with a standardized protoc
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