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

  • Clinical Management of Endocrine Diseases
  • Published:
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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

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Author information

Authors and Affiliations

  1. Department of Endocrinology, Catholic University of the Sacred Heart, Rome, Italy

    S. Chiloiro, A. Bianchi, A. Giampietro, A. Pontecorvi & L. De Marinis

  2. Department of Radiology, Catholic University of the Sacred Heart, Rome, Italy

    F. Lanza & M. G. Brizi

  3. Department of Oncology, Catholic University of the Sacred Heart, Rome, Italy

    G. Schinzari

  4. Department of Nuclear Medicine, Catholic University of the Sacred Heart, Rome, Italy

    V. Rufini & A. Giordano

  5. Department of Anatomic Pathology, Catholic University of the Sacred Heart, Rome, Italy

    F. Inzani & G. Rindi

Authors
  1. S. Chiloiro
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  2. F. Lanza
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  3. A. Bianchi
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  12. L. De Marinis
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Corresponding author

Correspondence to L. De Marinis.

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Conflict of interest

The authors declare that they have no competing interests.

Ethical approval

All procedures performed in this study were conducted in accordance with the good clinical practice, ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments.

Informed consent

Informed consent was obtained from all individual participants included in the study.

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Chiloiro, S., Lanza, F., Bianchi, A. et al. Pancreatic neuroendocrine tumors in MEN1 disease: a mono-centric longitudinal and prognostic study. Endocrine 60, 362–367 (2018). https://doi.org/10.1007/s12020-017-1327-0

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  • DOI: https://doi.org/10.1007/s12020-017-1327-0

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