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Endocrine Abstracts (2019) 68 P7 | DOI: 10.1530/endoabs.68.P7

UKINETS2019 Poster Presentations Abstracts (37 abstracts)

pNETs (Pancreatic Neuroendocrine tumours) in MEN1 (Multiple Endocrine Neoplasia): a single centre case series

Hema Venkataraman , Stacey Smith , Suzanne Vickrage , Joanne Kemp-Blake , Ian Geh , Shishir Shetty , Tahir Shah & John Ayuk


University Hospitals Birmingham, Birmingham, UK


Introduction: MEN1 associated pNETs share a unique profile in comparison to sporadic pNETs. The literature on pNETs in MEN1 is evolving with on-going ambiguities in presentation and management. We present a case series of MEN1 associated pNETs from a tertiary centre of excellence for NETs in the UK.

Methods: Institutional NET database was used for retrospective data analysis for all patients with pNETs associated with MEN1.

Results: Of a total of 1773 patients with NETs, 292 had pNETs with 16 of 292 having genetically proven MEN1. The average age of diagnosis of pNET in MEN1 was mean(S.D.): 34.7(11.5) years with an equal distribution among both sexes (8 men & 8 women). 8 pNETs were diagnosed on radiological surveillance for MEN1 related tumours. 6 pNETs were non-functioning (nfPNETs), 7 gastrinomas & 4 insulinomas (1 both gastrinoma and insulinoma) (2 insulinomas followed by nfPNET recurrence). Biochemical analysis of nfPNET revealed elevated gut hormones (GH) in 3/6. Pancreatic polypeptide (PP) and Chromogranin-B (CGB) were elevated in 2/6 patients and Glucagon in 1/6. 6/16 had multiple pNETs with the tail being the most common site of pNETs (12 tail, 7 head, 7 body). 7 had recurrence after a previous resection after a mean(S.D.) of 14.2(11.5) years. Recurrent tumours were gastrinomas(4/7) and nfPNETs (3/7) (2 after previous insulinoma resection and 1 previous nfPNET). 6/16 pNETs were metastatic (4 gastrinoma and 2 nfPNETs). At least one GH was elevated in all metastatic pNETs (4 gastrin, 1 pancreatic polypeptide & 1 chromogrannin B). No patients with MEN1 related pNET had elevated CGA. Histology of 11/16 pNETs revealed well differentiated NETs (7 Grade 1, 1 Grade 2, 3 no Ki67). Grade 2 pNET was associated with metastatic gastrinoma. There were 2 deaths related to a metastatic thymoma and a gastrinoma respectively.

Conclusion: MEN1 related PNETs from our single centre cohort, had a young age of onset and were more likely to be functioning PNETs, more commonly occurring in the tail of the pancreas. 50% of NfPNETs and all metastatic PNETs had elevated levels of at-least one GH. No patients with MEN1 associated PNET had CGA elevation.

Volume 68

17th Annual Meeting of the UK and Ireland Neuroendocrine Tumour Society 2019

Birmingham, UK
02 Dec 2019 - 02 Dec 2019

UK and Ireland Neuroendocrine Tumour Society 

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