Pancreatic neuroendocrine tumors in MEN1 disease: a mono-centric longitudinal and prognostic study
- 253 Downloads
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−).
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.
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).
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
KeywordsNeuroendocrine tumor MEN1 Pancreas pNET
Compliance with ethical standards
Conflict of interest
The authors declare that they have no competing interests.
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 was obtained from all individual participants included in the study.
- 8.G. Rindi, R. Arnold, C. Capella, et al. in Nomenclature and Classification of Neuroendocrine Neoplasms of the Digestive System, ed. by F. Bosman, F. Carneiro. World Health Organization Classification of Tumors, Pathology and Genetics of Tumors of the Digestive System (Lyon, IARC Press, 2010), p. 10–12.Google Scholar
- 11.A. Zerbi, M. Falconi, G. Rindi, G. Delle Fave, P. Tomassetti, C. Pasquali, V. Capitanio, L. Boninsegna, V. Di Carlo; the members of the AISP-Network Study Group, Clinicopathological features of pancreatic endocrine tumors: a prospective multicenter study in Italy of 297 Sporadic Cases. Am. J. Gastroenterol. 105, 1421–1429 (2010)CrossRefPubMedGoogle Scholar
- 12.A. Zerbi, V. Capitanio, L. Boninsegna, G. Delle Fave, C. Pasquali, G. Rindi, D. Campana, M. Falconi; the members of the AISP-Network Study Group, Treatment of malignant pancreatic neuroendocrine neoplasms: middle-term (2-year) outcomes of a prospective observational multicentre study. HPB 15, 935–943 (2013)CrossRefPubMedPubMedCentralGoogle Scholar
- 14.B. Skögseid, J. Rastad, G. Akerström. Pancreatic Endocrine Tumors in Multiple Endocrine Neo- plasia Type 1. Surgical Endocrinology, ed. by G.M. Doherty, B. Skögseid (Lippincott, Williams & Wilkins, Philadelphia, 2001) p. 510–524Google Scholar
- 15.R.T. Jensen. Zollinger-Ellison syndrome. Surgical Endocrinology, ed. by G.M. Doherty, B. Skögseid (Lippincott, Williams & Wilkins, Philadelphia, 2001) 291–344Google Scholar
- 17.F. Tonelli, F. Giudici, G. Fratini, M.L. Brandi, Pancreatic endocrine tumors in multiple endocrine neoplasia type 1 syndrome: review of literature. Endocr. Pract. 17(suppl 3), 33–40 (2011)Google Scholar
- 19.M.L. Brandi, R.F. Gagel, A. Angeli, J.P. Bilezikian, P. Beck-Peccoz, C. Bordi, B. Conte-Devolx, A. Falchetti, R.G. Gheri, A. Libroia, C.J.M. Lips, G. Lombardi, M. Mannelli, F. Pacini, B.A.J. Ponder, F. Raue, B. Skogseid, G. Tamburrano, R.V. Thakker, N.W. Thompson, P. Tomassetti, F. Tonelli, S.A. Wells, Jr., S.J. Marx, Guidelines for diagnosis and therapy of MEN type 1 and type 2. J. Clin. Endocrinol. Metab. 86(12), 5658–5671 (2001)Google Scholar
- 27.M.S. Hausman, Jr., N.W. Thompson, P.G. Gauger, G.M. Doherty, The surgical management of MEN-1 pancreatoduodenal neuroendocrine disease. Surgery 136, 1205–1211 (2004)Google Scholar
- 30.D. Granberg, M. Stridsberg, R. Seensalu, B. Eriksson, G. Lundqvist, K.O ̈ Berg, B. Skogseid, Plasma chromogranin A in patients with multiple endocrine neoplasia type 1. CE & M. 84, (8), 2712–2717 (1999)Google Scholar
- 33.E. Bajetta, L. Ferrari, A. Martinetti, L. Celio, G. Procopio, S. Artale, N. Zilembo, M. Di Bartolomeo, E. Seregni, E. Bombardieri, Chromogranin A., neuron specific enolase, carcinoembryonic antigen, and hydroxyindole acetic acid evaluation in patients with neuroendocrine tumors. Cancer, 86(5), 858–865 (1999)Google Scholar
- 35.Ita.NET Guidelines if Neuroendocrine Neoplasia. 2015 EditionGoogle Scholar