SFEBES2016 Poster Presentations Clinical biochemistry (28 abstracts)
Management of multiple endocrine neoplasia type 1 (MEN1) and sporadic pancreatic neuroendocrine tumours (PNETS) in relation to the clinical guidelines: a single centre audit
Georgia Ntali 1 , Paul J Newey 1, , Victoria Stokes 1 , Denis Talbot 3 , Zahir Soonawalla 4 , Greg Sadler 5 , Niki Karavitaki 6, , Ashley B Grossman 6 & Rajesh V Thakker 1
1Academic Endocrine Unit, Oxford Centre for Diabetes, Endocrinology and Metabolism, Radcliffe Department of Medicine, University of Oxford, Oxford, UK; 2Division of Cardiovascular and Diabetes Medicine, Level 5, Mailbox 12, Ninewells Hospital and Medical School, Dundee, UK; 3Department of Oncology, University of Oxford, Cancer and Haematology Center, Churchill Hospital, Oxford, UK; 4Department of Hepatobiliary and Pancreatic Surgery, Oxford University Hospitals NHS Trust, Oxford, UK; 5Department of Endocrine Surgery, Oxford University Hospitals NHS Trust, University of Oxford, Oxford, UK; 6Department of Endocrinology, Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, Oxford, UK; 7Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.
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. Ninety-one 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.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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