434P - Differential clinical and pathological characteristics of hereditary neuroendocrine pancreatic tumours (NEPT)

Date 08 October 2016
Event ESMO 2016 Congress
Session Poster Display
Topics Neuroendocrine Cancers
Presenter Gema Marín Zafra
Citation Annals of Oncology (2016) 27 (6): 136-148. 10.1093/annonc/mdw369
Authors G. Marín Zafra1, J. Tebar Masso2, J.M. Rodriguez Gonzalez1, P. Segura Luque1, J.L. Alonso Romero1, T. García3, P. Sanchez Henarejos1, A. Soto1, M.J. Martinez4
  • 1Medical Oncology, Hospital Universitario Virgen de la Arrixaca, 30120 - Murcia/ES
  • 2Endocrinology, Hospital Universitario Virgen de la Arrixaca, 30120 - Murcia/ES
  • 3Medical Oncology, Hospital Universitario Morales Meseguer, 30008 - Murcia/ES
  • 4Medical Oncology Unit, Hospital Universitario Santa Lucia, 30120 - Cartagena/ES

Abstract

Background

NEPT include a complex spectrum of neoplasm with diverse biology and clinical course. Multiple Endocrine Neoplasm type 1 (MEN1) syndrome is an autosomal dominant disease associated with a high frequency of duodenal and pancreatic neuroendocrine tumours. Due to its very low prevalence, the biological and clinical behaviour of hereditary NEPT is poorly characterized. Since they are underrepresented in clinical trials, the therapeutic approach is usually extrapolated from sporadic NEPT guidelines. The aim of this work was to define the clinical and pathological characteristics of hereditary NEPT.

Methods

We retrospectively analysed a large cohort (1983-2015) of MEN1-associated NEPT, corresponding to all incident cases in a high-prevalence geographical area. Clinical and pathological characteristics of hereditary and sporadic NEPT were compared. Chi-2 test and T-tests were used for comparison of qualitative and quantitative variables. Survival analysis was performed with Kaplan-Meier curves and log-rank test.

Results

A total of 85 patients were included: 58 (68%) patients with sporadic and 27 (31%) with hereditary NEPT (77%: c.1546delC). In comparison with sporadic cases, hereditary NEPT more frequently presented as non-metastatic tumors (96% vs. 36%, p = 0.001), with multiple lesions (74% vs. 25%, p 

Conclusions

Hereditary NEPT associated to MEN1 syndrome exhibit a very good prognosis, with no disease-related deaths observed in a large cohort of patients. Although genetic-driven early diagnose may have contributed to this outcome, the low grade and low proliferation found in our series, suggest a different biological behaviour for hereditary NEPT.

Clinical trial identification

Legal entity responsible for the study

Hospital Universitario Virgen de la Arrixaca, Murcia, Spain

Funding

None

Disclosure

All authors have declared no conflicts of interest.