What Is the Appropriate Management of Nonfunctioning Pancreatic Neuroendocrine Tumours Disclosed on Screening in Adult Patients With Multiple Endocrine Neoplasia Type 1?

Benjamin G. Challis; Ruth T. Casey; Ashley Grossman; John Newell-Price; Paul Newey; Rajesh V. Thakker


Clin Endocrinol. 2019;91(6):708-715. 

In This Article


NF-pNETs remain a major cause of premature mortality in patients with MEN1. Due to a limited understanding of tumour natural history and an absence of prognostic biomarkers, expert opinions are divided on their optimal clinical management. Although many questions regarding MEN1 associated NF-pNETs remain, since publication of the MEN1 clinical practice guidelines in 2012, a more refined understanding of tumour behaviour and the radiological techniques used for their detection have been established. This knowledge, coupled with emerging data relating to the morbidity associated with surgical resection of NF-pNETs in MEN1 and potential safety concerns associated with surveillance strategies, suggests that it may be timely to revise the current follow-up recommendations. However, a thorough systematic review of the currently available data is required to provide an evidence-based approach, and in its absence we advocate continuation of the guidelines recommended annual radiological surveillance with MRI for MEN1 patients. However, if surveillance yields a completely normal result, imaging every 2–3 years is likely to be sufficient. Currently available data suggest that screening for pNETs should commence between the ages of 10 and 16 years; however, further research is required to establish the optimal age to start pNET surveillance in MEN1.[13] Surgical resection is warranted for NF-pNETs >2 cm, whereas conservative management is generally appropriate for tumours <2 cm, accepting that a small proportion of patients may develop advanced disease. Given that NF-pNETs have distinct biological behaviours compared with non-MEN1 tumours, we discourage against the generalisation of findings from non-MEN1 clinical studies to the MEN1 population. Instead, we believe that it is vital that future MEN1 research focuses on the identification of prognostic biomarkers and surrogate clinical end-points that will facilitate the delivery of innovative, multicentre prospective clinical trials that evaluate the effectiveness of antitumour therapies to guide the optimal management of NF-pNETs in MEN1.