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Peptic ulcer due to Zollinger-Ellison syndrome is a rare entity. In this case report a 55-year-old man had a medical history of esophageal reflux, vomiting, and diarrhea for 10 years. Despite continuous medication with a proton pump inhibitor, no complete recovery from symptoms was achieved. A diagnosis of gastrinoma was at first not considered. After discontinuation of the proton pump inhibitor for only a few days, the strong stimulation of the gastrinoma led to fulminant hydrochloric acid burn of the distal esophagus with iatrogenic or spontaneous perforation at the esophagogastral junction. We describe the operative treatment as a two-stage reconstruction with colon interposition and resection of the primary tumor in the duodenum.

Background: Zollinger Ellison syndrome (ZES), an ulcerative disease of the upper gastrointestinal tract that involves the production of high levels of gastrin and gastric acid, is a rare, symptomatic, endocrine neoplastic disease., Case: We report a rare case of gastrinoma that was first diagnosed during pregnancy in which the primary tumor was located in the liver. The ZES was well controlled with Zoton (Lansoprazole) following surgery. The patient had an uneventful pregnancy and delivery without significant complications., Conclusions: The present case suggests that treatment with Zoton for ZES during pregnancy is safe and effective.

Aims/hypothesis: Type 1 and type 2 diabetes are characterised by a beta cell deficit. Islet hyperplasia has been described in patients with Zollinger-Ellison syndrome secondary to gastrin-producing tumours (gastrinomas), and gastrin therapy has increased beta cell mass in rodents and human islets in vitro. In the present studies we addressed the following questions: (1) In pancreas specimens from gastrinoma cases, is the fractional beta cell area increased? (2) If so, is this restricted to tumour-adjacent islets or also present in tumour-distant islets? (3) Is new beta cell formation (beta cell replication and islet neogenesis) increased and beta cell apoptosis decreased in pancreas specimens from gastrinoma cases?, Methods: Pancreas was obtained at surgery from four patients with Zollinger-Ellison syndrome caused by pancreatic gastrinomas and 15 control subjects at autopsy., Results: Islet fractional beta cell area (p<0.001), islet size (p<0.001) and beta cell replication (Ki67 staining) (p<0.05) were increased in islets adjacent to the tumours, but not in tumour-distant pancreas, compared with control subjects. We did not observe any differences in beta cell apoptosis or in the number of insulin-positive cells in ducts either adjacent to or distant from the tumour., Conclusions/interpretation: One or more factors released by human gastrinomas increase beta cell replication in islets immediately adjacent to the tumour, but not in tumour-distant islets. While these findings demonstrate that adult human beta cells can be driven into the cell cycle, they caution against the therapeutic usefulness of gastrin, since islets located >1 cm away from the gastrinomas did not exhibit changes in beta cell turnover, despite markedly elevated systemic gastrin levels sufficient to cause severe gastrointestinal symptoms.

The preoperative localization of gastrinomas often fails despite all modern imaging methods. Therefore, after biochemical confirmation of the diagnosis and exclusion of diffuse metastases, a meticulous surgical exploration including intraoperative ultrasound (IOUS) and duodenal exploration after duodenotomy should be performed. The experienced surgeon will be able to identify more than 90% of the primary tumors. Depending on the localization, excision of the tumor in the duodenal wall or enucleation from the pancreatic head should be performed. If the tumor is localized in the tail of the pancreas, distal pancreatectomy is the treatment of choice. Complete resection of the tumor is the only curative approach for the patients. For MEN-1 gastrinomas a spleen-preserving distal pancreatectomy with enucleation of tumors of the pancreatic head and duodenotomy with excision of duodenal gastrinomas should be performed. If the source of gastrin secretion can be regionalized to the pancreatic head by a preoperative SASI angiography, a pylorus-preserving partial pancreaticoduodenectomy might be the treatment of choice.

Benign tumors of the pancreatic head are normally treated by a partial duodenopancreatectomy. This operation has been developed for the treatment of malignant alterations in the pancreatic head and includes resection of the gastric bowel, duodenum and common bile duct. The aim of this study was to evaluate whether the less radical duodenum-preserving pancreatic head resection is a suitable surgical procedure in the treatment of benign pancreatic head tumors. From May 1982 to December 1996, seven patients underwent surgical treatment for benign pancreatic head tumors. Two patients suffered from gastrinoma of the pancreatic head, four exhibited a serous or mucinous cystadenoma, and one patient suffered from an intraductal papillary-mucinous tumor in this region. All patients were treated by duodenum-preserving pancreatic head resection. The operation was easily performed with little blood loss and a low rate of complications. None of the patients had to be reoperated upon due to postoperative surgical complications. After a follow-up period of a median 3 years, six of seven patients were had no recurrence of the disease and were symptom-free. One patient who had initially been operated on for gastrinoma still exhibited high gastrin values postoperatively. The endocrine and exocrine pancreatic function was not impaired in the early and late postoperative phase as compared to the preoperative assessment. From our results, it is concluded that duodenum-preserving pancreatic head resection is an adequately radical, yet organ-preserving procedure for the treatment of benign tumors of the pancreatic head without compromising endocrine and exocrine function.

During the past 10 years (1987-1996), 842 laparotomies were performed for pancreatic or periampullary neoplasms; in 25 patients (2.9%) a neuroendocrine tumor was diagnosed. In 19 of these 25 patients (76%) a non-functioning endocrine tumor and in 6 patients (24%) a hormone-active tumor (four insulinoma, one gastrinoma, one VIPoma) was found. Of 19 non-functioning neuroendocrine tumors, 14 were malignant. The resection rate of these malignant tumors was 78.6% (11 of 14; 3 resections were palliative); in comparison, the resection rate of ductal pancreatic carcinoma in our hospital was 28.1%. The probability of 5-year survival amounts to 73% after surgical resection in malignant endocrine tumors and to 19% in ductal pancreatic carcinoma (including palliative resections). As it is not always clear whether non-functioning endocrine tumors are benign or malignant, oncological resection is recommended. Adjuvant chemotherapy seems not to be necessary.

We report on a case of malignant gastrinoma located on the posterior surface of the descending duodenum, presenting with Zollinger-Ellison syndrome. The tumor was not evident on preoperative imaging studies, metastasis was not present and there was no coincidence with multiple endocrine neoplasia type-I. As the gastrinoma was located on the posterior surface of the pancreatic head, to obtain a sufficient safety margin, partial excision of this region was necessary. Under preservation of the Oddi's sphincter, the reconstruction was completed by direct suturing of the duodenal wall to the pancreatic surface without need for enteral diversion procedures. This technique represents a possible non-invasive resection modality for benign and malignant duodenal gastrinomas located close to the pancreatic head region.

Percutaneous aspiration to measure hormone levels in suspected functioning endocrine adenomas has been performed in 23 suspected cases of parathyroid adenomas, 7 cases of ACTH-producing thoracic carcinoid tumors, and 1 case of gastrinoma. When tumors secrete hormone or peptide markers, the biochemical assay can replace cytology to identify the lesion with greater specificity and at less expense.