Endocrine Abstracts

INTRODUCTION: Gastrinomas usually arise in pancreas or duodenum and may be small and difficult to localize. These tumours are multiple in 50% of sporadic cases but in MEN1 this figure rises to 90%.

CASE REPORT: Our patient presented aged 24 with a neck mass. Investigations confirmed primary hyperparathyroidism and four-gland parathyroidectomy confirmed hyperplasia. When normocalcaemic, her fasting plasma gastrin level was noted to be 129 pmol/l (NR<40). Abdominal CT scan revealed 1.5cm lesion the second part of the duodenum. Angiography showed a blush in this region after injection in the superior mesenteric artery (SMA), inferior pancreaticoduodenal artery (IPDA) and gastroduodenenal artery (GDA). Following injection into the GDA, IPDA and SMA, the gastrin level increased from 57pmol/l to >400pmol/l, 97pmol/l to 219pmol/l and from 140 pmol/l to 167 pmol/l respectively. There was no rise in plasma gastrin following injection of the splenic or hepatic arteries. Octreotide scan showed faint uptake in the midline consistent with the duodenal loop. The patient also had an iron deficient anaemia and in view of her high gastrin levels, an endoscopy was arranged which revealed very extensive, multiple ulcerated polypoid lesions. Histology of multiple biopsies revealed a malignant, infiltrative pleomorphic neuroendocrine tumour in the body and fundus. On subsequent review of the octreotide scan, there was dense uptake in the region of the stomach. She underwent a total gastrectomy and removal of the duodenal lesion. Histology of the stomach and duodenum confirmed malignant gastrimoma with multiple positive nodes and APUDoma respectively. Postoperatively she remains with normal gastrin level and repeat octreotide scan shows no abnormal uptake.

Discussion: Gastrinomas in MEN 1 are frequently multiple and usually located in the pancreas and duodenum. We present a case of a malignant gastrinoma initially thought to be duodenal gastrinoma but after several localization methods was found to have multiple gastric foci of malignant gastrinoma. This illustrates the importance of careful preoperative histological diagnosis of ulceration in the stomach for such patients.