PMID- 28567607
OWN - NLM
STAT- MEDLINE
DCOM- 20181114
LR  - 20181114
IS  - 1559-0100 (Electronic)
IS  - 1355-008X (Linking)
VI  - 60
IP  - 2
DP  - 2018 May
TI  - Pancreatic neuroendocrine tumors in MEN1 disease: a mono-centric longitudinal and 
      prognostic study.
PG  - 362-367
LID - 10.1007/s12020-017-1327-0 [doi]
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 (MEN1-). 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 MEN-1 p-NET. RESULTS: Of the 45 patients enrolled, 13 MEN1+ 
      and 21 MEN1- cases were analyzed. Frequency of not secreting p-NETs and insulin 
      secreting p-NETs, histopathological grades and Ki67 expression were 
      superimposable between MEN1+ and MEN1- patients. MEN1+ pNETs are more 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.
FAU - Chiloiro, S
AU  - Chiloiro S
AD  - Department of Endocrinology, Catholic University of the Sacred Heart, Rome, 
      Italy.
FAU - Lanza, F
AU  - Lanza F
AD  - Department of Radiology, Catholic University of the Sacred Heart, Rome, Italy.
FAU - Bianchi, A
AU  - Bianchi A
AD  - Department of Endocrinology, Catholic University of the Sacred Heart, Rome, 
      Italy.
FAU - Schinzari, G
AU  - Schinzari G
AD  - Department of Oncology, Catholic University of the Sacred Heart, Rome, Italy.
FAU - Brizi, M G
AU  - Brizi MG
AD  - Department of Radiology, Catholic University of the Sacred Heart, Rome, Italy.
FAU - Giampietro, A
AU  - Giampietro A
AD  - Department of Endocrinology, Catholic University of the Sacred Heart, Rome, 
      Italy.
FAU - Rufini, V
AU  - Rufini V
AD  - Department of Nuclear Medicine, Catholic University of the Sacred Heart, Rome, 
      Italy.
FAU - Inzani, F
AU  - Inzani F
AD  - Department of Anatomic Pathology, Catholic University of the Sacred Heart, Rome, 
      Italy.
FAU - Giordano, A
AU  - Giordano A
AD  - Department of Nuclear Medicine, Catholic University of the Sacred Heart, Rome, 
      Italy.
FAU - Rindi, G
AU  - Rindi G
AD  - Department of Anatomic Pathology, Catholic University of the Sacred Heart, Rome, 
      Italy.
FAU - Pontecorvi, A
AU  - Pontecorvi A
AD  - Department of Endocrinology, Catholic University of the Sacred Heart, Rome, 
      Italy.
FAU - De Marinis, L
AU  - De Marinis L
AD  - Department of Endocrinology, Catholic University of the Sacred Heart, Rome, 
      Italy. laurademarinis@yahoo.it.
LA  - eng
PT  - Journal Article
PT  - Observational Study
DEP - 20170531
PL  - United States
TA  - Endocrine
JT  - Endocrine
JID - 9434444
SB  - IM
MH  - Adult
MH  - Aged
MH  - Female
MH  - Humans
MH  - Longitudinal Studies
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/*pathology
MH  - Neuroendocrine Tumors/etiology/*pathology
MH  - Pancreas/*pathology
MH  - Pancreatic Neoplasms/etiology/*pathology
MH  - Retrospective Studies
OTO - NOTNLM
OT  - MEN1
OT  - Neuroendocrine tumor
OT  - Pancreas
OT  - pNET
EDAT- 2017/06/02 06:00
MHDA- 2018/11/15 06:00
CRDT- 2017/06/02 06:00
PHST- 2017/01/17 00:00 [received]
PHST- 2017/05/12 00:00 [accepted]
PHST- 2017/06/02 06:00 [pubmed]
PHST- 2018/11/15 06:00 [medline]
PHST- 2017/06/02 06:00 [entrez]
AID - 10.1007/s12020-017-1327-0 [pii]
AID - 10.1007/s12020-017-1327-0 [doi]
PST - ppublish
SO  - Endocrine. 2018 May;60(2):362-367. doi: 10.1007/s12020-017-1327-0. Epub 2017 May 
      31.