SUN-LB057 An Interesting Case of Multiple Pancreatic Neuroendocrine Tumour: Normoglycaemic Glucagonoma and Concurrent Gastrinoma

Abstract

Background: Pancreatic neuro-endocrine tumours (pNETs) are rare tumours, constituting less than 5% of pancreatic tumours. Of these cases, only an estimated 6% present with functional secretion of more than one hormone. Clinical Case: A 72-year-old woman was admitted to the acute frailty ward with symptoms of generalized weakness, fatigue and reduced exercise tolerance with shortness of breath on exertion. She reported unintentional weight loss of 16 kg in the preceding 3 months and widespread migratory rash with epidermal breakdown in the lower legs that was resistant to antibiotic therapy. Initial investigations on admission revealed severe anaemia with a haemoglobin (Hb) of 51 g/L that was refractory to blood transfusion, prompting gastroscopy that showed erosive gastritis. CT/MRI scans revealed a 24 mm solid arterial-enhancing mass lesion in the uncinate process of the pancreas, highly suggestive of a neuroendocrine tumour (NET). Histopathological examination of a skin biopsy showed intraepithelial vacuolisation of the cells in the upper epidermis, neutrophilic infiltration with psoriasiform hyperplasia and confluent parakeratosis; typical features of necrolytic migratory erythema (NME). Fasting serum gut hormone profile prior to omeprazole therapy was consistent with multiple functioning NET with elevated serum glucagon level (>500 pmol/L) and hypergastrinaemia (274 pmol/L). Fasting serum glucose level was 4.5 mmol/L and serum albumin was 24 g/L. Elevated serum fasting serum glucagon and gastrin levels strongly supported diagnosis of neuroendocrine tumour resulting in normoglycaemic glucagonoma syndrome (NME, weight loss without diabetes mellitus) and Zollinger-Ellison syndrome (anaemia and hyperchlorhydria). The rash resolved rapidly less than a week after commencing medical management with octreotide, a somatostatin receptor analogue. She was also commenced on high dose PPI therapy. Somatostatin receptor scintigraphy was performed that confirmed a somatostatin-avid primary tumour in the pancreas without distant metastases. Surveillance will include blood glucose monitoring, interval rescanning with CT abdomen and pelvis with contrast and octreotide scan to assess for metastases. Surgical resection of the tumour will be considered as the patient's performance status improves. Conclusion: We report a rare case of a patient diagnosed with a multiple functioning pNET. The clinician must always consider unconventional rashes as dermatological manifestations of systemic disease. A normal fasting serum glucose level is not a reliable exclusion criterion of glucagonoma syndrome due to functioning insulin secretion from beta pancreatic islet cells. Isolated measurement of fasting serum gastrin level is not appropriate for diagnosis of Zollinger-Ellison syndrome in patients treated with proton pump inhibitors or histamine receptor antagonists. Unless otherwise noted, all abstracts presented at ENDO are embargoed until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. s presented at a news conference are embargoed until the date and time of the news conference. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.