Clinical features

Endocrine tumours of the duodenum produce symptoms either by virtue of local infiltration causing obstructive jaundice, pancreatitis, haemorrhage, and intestinal obstruction (nonfunctioning tumours) or, less frequently, by secreted peptide hormones (functioning tumours). The prevalent position of somatostatin-cell tumours, gangliocytic paraganglio-mas, and small cell carcinomas in the ampullary region explains their frequent association with obstructive biliary disease. About 20% of the tumours, especially those located in the duodenal bulb, are asymptomatic and often incidentally discovered, e.g. by imaging analysis, endoscopy or pathological examination of gastrectomy and duodenopancreate-ctomy specimens removed for gastric and pancreatic cancers. Zollinger-Ellison Syndrome (ZES) with hypergastrinaemia, gastrin hypersecretion, and refractory peptic ulcer disease, is the only syndrome of endocrine hyper-function consistently observed in association with endocrine tumours of the duodenum and upper jejunum {208, 429, 726, 1780, 2076}. The association with ZES is found in about 15% of duodenal

Fig. 4.12 Gastrin cell tumour with typical gyriform trabecular pattern.

gastrin-cell tumours {1780}. Tumours associated with overt ZES differ from their apparently nonfunctioning counterpart in arising earlier in life and having a higher incidence of metastatic and non-bulbar cases {1780}. Argentaffin, serotonin-producing, carci-noids are unusual in the upper small intestine. It follows that duodenal carci-noids only exceptionally give rise to a clinical carcinoid syndrome, associated with liver metastases of the tumour {233, 1816}. In none of the cases of somato-statin-cell tumours, so far reported, did the patients develop the full 'somato-statinoma' syndrome (diabetes mellitus, diarrhoea, steatorrhoea, hypo- or achlor-hydria, anaemia and gallstones) that has been described in association with some pancreatic somatostatin-cell tumours {1780}.

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Diabetes 2

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