TY - JOUR
T1 - Pancreatic neuroendocrine tumors in MEN1 disease: a mono-centric longitudinal and prognostic study
AU - Chiloiro, Sabrina
AU - Lanza, Francesca
AU - Bianchi, Antonio
AU - Schinzari, Giovanni
AU - Brizi, Maria Gabriella
AU - Giampietro, Antonella
AU - Rufini, Vittoria
AU - Inzani, Frediano
AU - Giordano, Alessandro
AU - Rindi, Guido
AU - Pontecorvi, Alfredo
AU - De Marinis Grasso, Laura
PY - 2018
Y1 - 2018
N2 - 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 not-affected ones (MEN1â). Methods: We performed a retrospective observational study. Data was collected between December 2010 and December 2015, including patients with a histological diagnosis of p-NET and radiological imaging. We compared clinical, histological, radiological, and prognostic aspects of MEN+ p-NET with MENâ1 p-NET. Results: Of the 45 patients enrolled, 13 MEN1+ and 21 MEN1â cases were analyzed. Frequency of not secreting p-NETs and insulin secreting p-NETs, histopathological grades and Ki67 expression were superimposable between MEN1+ and MEN1â patients. MEN1+ pNETs are more often multicentric compared to MEN1â pNETs. Frequency of liver and nodes metastatic spread was higher in MEN1â 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 MEN1â 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 MEN1â donât 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 patientsâ first-degree relatives
AB - 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 not-affected ones (MEN1â). Methods: We performed a retrospective observational study. Data was collected between December 2010 and December 2015, including patients with a histological diagnosis of p-NET and radiological imaging. We compared clinical, histological, radiological, and prognostic aspects of MEN+ p-NET with MENâ1 p-NET. Results: Of the 45 patients enrolled, 13 MEN1+ and 21 MEN1â cases were analyzed. Frequency of not secreting p-NETs and insulin secreting p-NETs, histopathological grades and Ki67 expression were superimposable between MEN1+ and MEN1â patients. MEN1+ pNETs are more often multicentric compared to MEN1â pNETs. Frequency of liver and nodes metastatic spread was higher in MEN1â 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 MEN1â 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 MEN1â donât 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 patientsâ first-degree relatives
KW - Endocrinology
KW - Endocrinology, Diabetes and Metabolism
KW - MEN1
KW - Neuroendocrine tumor
KW - Pancreas
KW - pNET
KW - Endocrinology
KW - Endocrinology, Diabetes and Metabolism
KW - MEN1
KW - Neuroendocrine tumor
KW - Pancreas
KW - pNET
UR - http://hdl.handle.net/10807/113967
UR - http://www.springer.com/humana+press/journal/12020
U2 - 10.1007/s12020-017-1327-0
DO - 10.1007/s12020-017-1327-0
M3 - Article
SN - 1355-008X
VL - 60
SP - 362
EP - 367
JO - Endocrine
JF - Endocrine
ER -