Page 226 - Medicine and Surgery

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222 Chapter 5: Hepatic, biliary and pancreatic systems

survival of patients who undergo curative resection is Many pancreatic endocrine tumours express somato-
30%. statin receptors. Radiolabelled octreotide (a somato-
statin analogue) can be used for localisation of the
primary tumour and detection of any metastases.
Islet cell tumours
Surgery is the treatment of choice.
Insulinoma: Ausually benign islet-cell tumour that may Several options are available for the treatment of
occur in the pancreas or at ectopic sites causing the metastatic neuroendocrine tumors including oc-
hypersecretion of insulin. There may be gradual in- treotide, interferon α,chemotherapy and hepatic
tellectual and motor impairment with insidious per- artery embolisation.
sonality changes. Severe attacks of hypoglycaemia can Glucagonoma: This is a very rare tumour of the islet cells
produce sweating, palpitations, tremulousness and a of the pancreas which is often asymptomatic. Patients
rangeofbizarrepsychoneurologicalbehaviours.Patients maypresentwithnecrolyticmigratoryerythema,painful
may present with a hypoglycaemic coma. Histology glossitis, stomatitis, gastrointestinal upset, weight loss,
shows encapsulated yellow/brown nodules containing diabetes mellitus and anaemia. Plasma glucagon levels
cords and nests of well-differentiated β-cells. are raised. Imaging and systemic treatment are as for
insulinoma. Resection of the tumour is usually curative.
Investigations/management Other islet cell tumours: Very rarely islet-cell tumours
Fasting hypoglycaemia with inappropriately high in- secrete VIP or gastrin. VIPomas cause a profuse wa-

sulin secretion (exogenous insulin can be excluded by tery diarrhoea, hypokalaemia and achlorhydria. The pa-
measuring C-peptide levels). tient may present as an acute abdomen, with ileus and
The tumour is found by conventional imaging in less abdominal distension suggestive of intestinal obstruc-

than 50% of cases. Selective venous sampling of con- tion. Treatment is by resection where possible, or sys-
centrations of insulin may be helpful, endoscopic ul- temic treatment as for insulinoma. Gastrinomas cause
trasound is increasingly being used. the Zollinger–Ellison syndrome (see page 161).