Presentation and outcome of pancreaticoduodenal endocrine tumors in multiple endocrine neoplasia type 1 syndrome

Maria Vittoria Davì, Letizia Boninsegna, Luca Dalle Carbonare, Marco Toaiari, Paola Capelli, Aldo Scarpa, Giuseppe Francia, Massimo Falconi

Research output: Contribution to journalArticle

Abstract

Aim: To assess presentation and outcome of pancreaticoduodenal endocrine tumors (PDETs) in a single center series of multiple endocrine neoplasia type 1 (MEN1) patients. Methods: Retrospective analysis of prospectively collected data of MEN1 patients observed at the University of Verona. Results: Thirty-one MEN1 patients had PDETs, including 16 nonfunctioning (NF), 6 insulinomas and 9 Zollinger-Ellison syndrome (ZES). In 16 of these patients (52%), PDET was the manifestation which led to the diagnosis of MEN1; among this group, 15 patients (94%) previously had unidentified primary hyperparathyroidism (PHPT), which was asymptomatic in 9 cases (60%). Of the 31 patients, 19 (61%) underwent curativesurgery and 13 (68%, 7 NF-PDETs, 4 insulinomas and 2 ZES) were disease-free after a median follow-up of 3 years (range: 0.5-15). One patient had debulking surgery with stable disease after 2 years of follow-up. Eight patients with NF-PDETs ≤20 mm and 2 with ZES, treated with a conservative approach, showed stable disease. One patient with insulinoma was lost to follow-up. Conclusions: PDET may be the manifestation that leads to MEN1 diagnosis since the almost constant presence of PHPT is very often unrecognized or considered sporadic. Conversely, the presence of PDETs should be looked for in all patients presenting PHPT, even if asymptomatic, particularly before age 50. Surgery may be curative in the majority of insulinomas and can prolong disease-free survival in NF-PDET, but is not proven to be effective in ZES. A conservative approach can be safely reserved for patients with NF-PDETs ≤20 mm.

Original languageEnglish
Pages (from-to)58-65
Number of pages8
JournalNeuroendocrinology
Volume94
Issue number1
DOIs
Publication statusPublished - Jul 2011

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Multiple Endocrine Neoplasia
Multiple Endocrine Neoplasia Type 1
Zollinger-Ellison Syndrome
Insulinoma
Neoplasms
Primary Hyperparathyroidism
Lost to Follow-Up
Disease-Free Survival

Keywords

  • Multiple endocrine neoplasia type 1
  • Pancreaticoduodenal endocrine tumors
  • Primary hyperparathyroidism

ASJC Scopus subject areas

  • Endocrinology
  • Endocrinology, Diabetes and Metabolism
  • Endocrine and Autonomic Systems
  • Cellular and Molecular Neuroscience

Cite this

Presentation and outcome of pancreaticoduodenal endocrine tumors in multiple endocrine neoplasia type 1 syndrome. / Davì, Maria Vittoria; Boninsegna, Letizia; Dalle Carbonare, Luca; Toaiari, Marco; Capelli, Paola; Scarpa, Aldo; Francia, Giuseppe; Falconi, Massimo.

In: Neuroendocrinology, Vol. 94, No. 1, 07.2011, p. 58-65.

Research output: Contribution to journalArticle

Davì, MV, Boninsegna, L, Dalle Carbonare, L, Toaiari, M, Capelli, P, Scarpa, A, Francia, G & Falconi, M 2011, 'Presentation and outcome of pancreaticoduodenal endocrine tumors in multiple endocrine neoplasia type 1 syndrome', Neuroendocrinology, vol. 94, no. 1, pp. 58-65. https://doi.org/10.1159/000326164
Davì, Maria Vittoria ; Boninsegna, Letizia ; Dalle Carbonare, Luca ; Toaiari, Marco ; Capelli, Paola ; Scarpa, Aldo ; Francia, Giuseppe ; Falconi, Massimo. / Presentation and outcome of pancreaticoduodenal endocrine tumors in multiple endocrine neoplasia type 1 syndrome. In: Neuroendocrinology. 2011 ; Vol. 94, No. 1. pp. 58-65.
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AU - Capelli, Paola

AU - Scarpa, Aldo

AU - Francia, Giuseppe

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AB - Aim: To assess presentation and outcome of pancreaticoduodenal endocrine tumors (PDETs) in a single center series of multiple endocrine neoplasia type 1 (MEN1) patients. Methods: Retrospective analysis of prospectively collected data of MEN1 patients observed at the University of Verona. Results: Thirty-one MEN1 patients had PDETs, including 16 nonfunctioning (NF), 6 insulinomas and 9 Zollinger-Ellison syndrome (ZES). In 16 of these patients (52%), PDET was the manifestation which led to the diagnosis of MEN1; among this group, 15 patients (94%) previously had unidentified primary hyperparathyroidism (PHPT), which was asymptomatic in 9 cases (60%). Of the 31 patients, 19 (61%) underwent curativesurgery and 13 (68%, 7 NF-PDETs, 4 insulinomas and 2 ZES) were disease-free after a median follow-up of 3 years (range: 0.5-15). One patient had debulking surgery with stable disease after 2 years of follow-up. Eight patients with NF-PDETs ≤20 mm and 2 with ZES, treated with a conservative approach, showed stable disease. One patient with insulinoma was lost to follow-up. Conclusions: PDET may be the manifestation that leads to MEN1 diagnosis since the almost constant presence of PHPT is very often unrecognized or considered sporadic. Conversely, the presence of PDETs should be looked for in all patients presenting PHPT, even if asymptomatic, particularly before age 50. Surgery may be curative in the majority of insulinomas and can prolong disease-free survival in NF-PDET, but is not proven to be effective in ZES. A conservative approach can be safely reserved for patients with NF-PDETs ≤20 mm.

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