Endocrine Abstracts (2018) 55 P03 | DOI: 10.1530/endoabs.55.P03

Non-functional duodenal neuroendocrine carcinoma- a rare cause of diabetes mellitus

Chad Bisambar1, Andrew Collier1 & Fraser Duthie2

1NHS Ayrshire and Arran, Ayr, UK; 2NHS Glasgow and Greater Clyde, Glasgow, UK.

Case history: We present a 40 year old female admitted with hyperglycaemia, polyuria, polydipsia and weight loss of 6 kg over a 1 month period. She had no night sweats or change in bowel habit. There was no personal or family history of malignancy or diabetes mellitus. She denied any alcohol, cigarette or illicit drug use. She took no prescription or OTC medication. On examination, she was jaundiced with pale mucous membranes. The rest of systemic examination was normal. Capillary glucose was 23.1 mmol/l.

Investigations: FBC, LFT, U and E, HbA1c, Urinary ACR, blood film, fasting gut hormone profile, CT- chest, abdomen and pelvis, duodenoscopy and biopsy, MRI liver, Octreotide scan, Endoscopic Ultrasound and biopsy, Screen for MEN 1 syndrome.

Results and treatment: Hb- 64, Wcc- 8.4, platelet count- 346, lab glucose - 21.8 mmol/l, T -bili 48, Alp 687, Ast - 96, Alt - 117, Urea- 2.5, Cr- 52, Na- 136, k - 4.6, Hba1c –79 mmol/mol, Blood film- iron deficiency anaemia, Urinary ACR- 5.4, Pituitary profile, Calcium and PTH normal. Fasting gut hormones: Vip- 4 (<30 pmol/l), pancreatic polypeptide- 12 (<3000 pmol/l), gastrin −8 (< 40 pmol/l), glucagon- 14 (0 to 50 pmol/l), Somatostatin- 174(0 to 150 pmol/l). Chromogranin A- 78 (0 to 60 pmol/l), chromogranin B −49 (0 to 150 pmol/l). Duodenoscopy and biopsy- flat velvet like lesion in anterior wall of 2ndpart of duodenum around ampulla. Biopsy – tubovillous adenoma with low grade dyplasia. CT chest, abdomen, pelvis - significant dilatation of intra and extra hepatic biliary tree including pancreatic duct. Periampullary 30 mm mass lesion projecting into lumen of duodenum. Enlarged nodes around superior mesenteric artery. Confirmed on MRI liver. EUS and biopsy- Mass in medial wall duodenum. Suspicious node over SMA. Fine needle biopsy of duodenal wall and lymph node in keeping with grade 1, well differentiated neuroendocrine tumour. NM octreotide whole body scan and Spect CT- no uptake. Treatment-BD mixed insulin, transfused to Hb>8 g/dl. WHIPPLES PANCREATICO-DUODENECTOMY: R0 pT3 pN1 well differentiated neuroendocrine carcinoma arising in duodenum; Grade G1 (Ki 67: 0.5%); Venous invasion present; Involvement of 4 of 17 lymph nodes.

Conclusions and points for discussion: Duodenal NET with main pancreatic duct obstruction can present with hyperglycaemia and cause diabetes. This is in the absence of gluconeogenic hormones such as somatostatin and glucagon. There was complete resolution of diabetes post Whipple’s procedure and patient is now off insulin. Her last HBA1C was 31 mmol/mol.

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