Pancreatic neuroendocrine tumors in MEN1 disease: a mono-centric longitudinal and prognostic study

Giovanni Schinzari, Maria Gabriella Brizi, Vittoria Rufini, Alessandro Giordano, Guido Rindi, Alfredo Pontecorvi, Sabrina Chiloiro, Francesca Lanza, Antonio Bianchi, Antonella Giampietro, Frediano Inzani, Laura De Marinis Grasso

Research output: Contribution to journalArticle

9 Citations (Scopus)

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 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
Original languageEnglish
Pages (from-to)362-367
Number of pages6
JournalENDOCRINE
Volume60
DOIs
Publication statusPublished - 2018

Keywords

  • Endocrinology
  • Endocrinology, Diabetes and Metabolism
  • MEN1
  • Neuroendocrine tumor
  • Pancreas
  • pNET

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