Endocrine Abstracts

Introduction

Multiple Endocrine Neoplasia type-1 (MEN-1) is an inherited autosomal dominant condition with a high degree of penetrance characterized by tumour occurrence in the form of pancreatic neuroendocrine (pNET), parathyroid and anterior pituitary gland tumours, among others. Treatment for MEN-1 associated endocrine tumour, particularly pancreatic, is more challenging than in those without MEN-1, given its multicentric nature and aggressive behaviour. Hence early detection through targeted surveillance is paramount.

Aim

The aim of the audit is to compare our surveillance of MEN-1 patients and benchmark against published guidelines.

Methods

We performed a search for patients with a diagnosis of MEN-1 using our hospital electronic records (notes, laboratory and radiology) and collected data retrospectively. We looked at the radiological and biochemical surveillance and their frequency.

Results

A record of 15 patients with a diagnosis of MEN-1 was found (males 4/15 (27%) and females 11/15 (73%). 13/15 (87%) had annual calcium profile and one at 3 years. For parathyroid hormone (PTH) 11/15(73%) had annual measurements, 3/15(20%) had 3 yearly profile. Screening frequency for fasting gut profile for 1 year, 2 years, 3 years were 9/15(60%), 2/15(13%) and 3/15(20%), pancreatic cross-sectional imaging were 7/15 (47%), 2/15 (13%) and 2/15(13%) respectively. The majority of patients had plasma prolactin and insulin-like growth factor 1 (IGF-1) checks annually 11/15 (73%) and 3/15 (20%) once every 3 years. For pituitary MRI surveillance: 10/15 (67%) were screened within the recommended 3–5 years timeline while 33% had longer interval between scans.

Conclusions

Overall our audit shows good compliance with existing MEN-1 guidelines, with our cohort of patients being regularly screened for the development of primary hyperparathyroidism, pNET and pituitary tumours. To improve this further our MEN-1 patients are now being reviewed in specialised clinics.

Reference

1. Rajesh V. Thakker, Paul J. Newey, Gerard V. Walls, John Bilezikian etc. Clinical Practice Guidelines for Multiple Endocrine Neoplasia Type 1 (MEN1). The Journal of Clinical Endocrinology & Metabolism, Volume 97, Issue 9, 1 September 2012, Pages 2990–3011.