Koexistenz von zwei unterschiedlichen neuroendokrinen Tumoren des oberen Gastrointestinaltraktes und des Pankreas

HISTORY AND CLINICAL FINDINGS: A 41-year-old obese patient presented with cramp-like abdominal pain, watery diarrhoea with partly digested food particles, projectile vomiting and newly diagnosed diabetes mellitus. For the preceding 6 years he had been treated for recurrent gastric and duodenal ulcers. Although the fasting gastrin level was raised and Zollinger-Ellison syndrome suspected, computed tomography (CT), magnetic resonance imaging (MRI) and coeliac angiography at another hospital had failed to discover a tumor. INVESTIGATIONS: Biochemical tests were unremarkable except for an increased GPT concentration, slight fasting hyperglycemia and hypertriglyceridemia. The gastrin and chromogranin A levels were markedly elevated (15,590 pg/ml and 584.2 U/l, respectively). Gastroscopy revealed, in addition to multiple small duodenal ulcers, a round polypoid mass (diameter of 0.7 cm) lateral to the papilla of Vater, histologically an APUDoma. Endoscopic retrograde cholangiopancreatography (ERCP) revealed a 0.5 cm long compression of the duct of Wirsung in the region of the head of the pancreas. Liver metastases were excluded by magnetic resonance imaging and computed tomography. Endosonography showed a ca. 4 mm space-occupying lesion in the region of the body of the pancreas. Octreotide scintigraphy demarcated two foci at the level of the head of the pancreas (somatostatin-receptor positive). TREATMENT AND COURSE: After a pylorus-preserving partial duodenopancreatectomy with lymph node dissection N1/N2, histology confirmed a gastrinoma of the duodenum and a glucagonoma of the pancreas (pT3pN1pMx). Postoperatively the patient became symptom-free and both the blood sugar level and the tumor marker were normal. CONCLUSION: Combined ERCP, endosonography and scintigraphy are more sensitive than other radiological examinations (CT and MRI) in diagnosing and localizing neuroendocrine tumours of the gastrointestinal tract. Despite the low incidence of such tumours, the possible synchronous occurrence of several such tumour should not be ignored.