Management of multiple endocrine neoplasia type 1 (MEN1) and sporadic pancreatic neuroendocrine tumours (PNETS) in relation to the clinical guidelines: a single centre audit

Research output: Contribution to conference (unpublished)Posterpeer-review

Colleges, School and Institutes

Abstract

Introduction and aim
Pancreatic neuroendocrine tumours (PNETs) may occur sporadically (sPNETs) or
as part of the multiple endocrine neoplasia type 1(MEN1) syndrome, which is
characterised by occurrence of PNETs, parathyroid and anterior pituitary
tumours. Our aim was to review the management of these patients in relation
to the clinical practice MEN1 guidelines, and the ENETS and UKINETS
guidelines for PNETs.

Patients and methods
Patients attending with MEN1 or sporadic PNETs, during 2011–2013, were
ascertained. All patients were reviewed at NET multidisciplinary team meetings.
All PNETs were characterised using the WHO 2010 classification, TNM and
ENETs staging system.

Results
Of 94 individuals (49 males and 45 females) with MEN1-associated tumours or a
family history of MEN1, 67% had genetic testing to identify the MEN1 mutation,
and a diagnosis of MEN1 was established in 81 (i.e. 86%) patients by genetic and
clinical criteria; and the remaining 13 patients were unaffected relatives. Ninetyone percent of the MEN1 patients had primary hyperparathyroidism (PHPT); 67% had PNETs; and 36% had a pituitary tumour. Screening frequencies of 1, 2 and 3 times, during the 3 years were, respectively, as follows: for PHPT, using plasma calcium and parathyroid hormone (PTH) measurements, 20, 25 and 30%; for PNETs, using fasting gastro-intestinal hormones, 22.5, 30 and 27.5%, and using pancreatic-duodenal imaging, 15, 25 and 37.5%; and for pituitary tumours, using plasma prolactin and insulin-like growth factor 1 (IGF1), 15, 28 and 25%, and using MRI, 26, 6 and 2%. Of 59 patients (34 males and 25 females) with sPNETs, 39 had a non-functioning and 25 a functioning tumour (17 insulinomas, three glucagonomas, two gastrinomas, one hyperplasia of islet Langerhans, and two somatostatinomas).

Conclusions
Our audit shows compliance with guidelines, with MEN1 patients being regularly
screened for the development of PHPT, PNETs and pituitary tumours.

Details

Original languageEnglish
Publication statusPublished - Nov 2016
EventSociety for Endocrinology BES 2016 - Brighton, United Kingdom
Duration: 7 Nov 20169 Nov 2016

Conference

ConferenceSociety for Endocrinology BES 2016
CountryUnited Kingdom
CityBrighton
Period7/11/169/11/16