Zollinger-Ellison Research Articles

Complicated Zollinger-Ellison Syndrome: 365 Days in Hospital

Background: Zollinger-Ellison (ZE) syndrome is a neuroendocrine tumor with hypersecretion of gastrin leading to resistant peptic ulcers. We are reporting a case of Z-E syndrome with extremely complex hospitalization course lasted around one year. Case: A 50-year-old lady presented to the emergency department with anemia, melena and diarrhea. Upper endoscopy showed multiple ulcerations at gastroesophageal junction, duodenal blub and second part of the duodenum. H. pylori was negative. The patient was started on optimum proton pump inhibitors (PPI) with improvement. Couple of months later she relapsed and another endoscopy revealed the same findings. Gastrin level was 700 pg\ml. CT, MRI of the abdomen and video capsule endoscopy were inconclusive for any pancreatic or small bowel masses. One month later she had duodenal perforation. Since that time the patient had complicated hospital course that lasted for more than one year. She required 16 surgeries for management of perforations, peritonitis, gastric outlet obstruction and managing fistulas and strictures. She was admitted to the ICU for 3 months in which she developed acute renal failure that needed dialysis for three months. She was also on Total parenteral nutrition (TPN) for few months then on tube feeding later on. She had 8 upper endoscopies during her hospitalization. Her fasting gastrin level was found to be more than 10,000 pg/ml when she had the perforation, hence the diagnosis of ZE syndrome. She was discharged one year after her admission on regular PO diet with gastrostomy- jejunostomy tube in place. Her enterocutaneous fistula was healing well. Discussion: ZE syndrome is a rare cause of PUD. Ulcers' perforation and bleeding are life threating complications. Our patient presented initially with multiple ulcers in the context of negative H.Pylori, Gastrin level initially was indeterminate (700pg/ml) and was treated with Pantoprazole 40mg twice daily. Thereafter she developed multiple complications needed very complicated hospital course extended over one year. ZE syndrome was diagnosed later with gastrin level of more than 10,000pg/ml. There were no evidence of MEN I features. The tumor site was not identified and the patient with these extensive surgeries was not a candidate for another surgical intervention at time of discharge. Conclusion: ZE syndrome can lead to multiple life threatening complications. High index of suspicion in the right clinical context is needed to prevent complications and the associated morbidities.

Primary Lymph Node Gastrinoma Resulting in Zollinger-Ellison Syndrome

Purpose: A 63-year-old man presented with 4 months of non-bloody diarrhea, with 30 bowel movements daily; diarrhea persisted at night while fasting. There was an associated 70-pound unintentional weight loss, along with rare heartburn partially relieved with antacids. Computed tomography of the abdomen revealed a 4.7-cm enhancing mass in the midline adjacent to or arising from the uncinate process of the pancreas. Serum gastrin, obtained off of a proton-pump inhibitor (PPI) was elevated to 1270 pg/mL. Esophagogastroduodenoscopy revealed severe erosive esophagitis and innumerable duodenal bulb and post-bulbar ulcers. Gastric rugae were thickened with large amounts of reaccumulating secretions that had a bedside pH of 3.5. Based on these findings, Zollinger-Ellison syndrome (ZES) was diagnosed. Octreotide scan demonstrated uptake in the lesion seen on CT with no liver lesions. The patient was started on IV PPI with resolution of abdominal pain and diarrhea. Exploratory laparotomy demonstrated, within the gastrinoma triangle, a well-circumscribed 4 x 5-cm mass, which was mobile and not fixed to the pancreas or other adjacent structures. Pancreas and duodenum were palpated extensively with no evidence of occult lesions; the mass was then excised. Pathology with staining determined the tumor to be a primary lymph node (LN) gastrinoma. Post-operatively, diarrhea resolved and there was gradual weight gain. Gastrin level normalized and PPI was eventually discontinued. ZES is caused by the autonomous secretion of gastrin by gastrinomas. Ninety percent of gastrinomas occur in an anatomic area called the gastrinoma triangle, classically in the pancreas or duodenal wall. Surgical resection can result in cure and should be pursued in patients with resectable disease. Not all gastrinomas in LN result from metastatic spread from pancreatic primaries, but rather may be primary LN tumors as shown in our case. This is supported by the absence of gastrinoma in other locations and normalization of gastrin levels postoperatively. Primary LN gastrinomas do not have any distinguishing characteristics compared to other types of gastrinomas. All patients with ZES proceeding to surgery should have detailed exploration to rule out pancreatic or duodenal tumors, as well as routine excision of LNs within the gastrinoma triangle. The existence of primary LN gastrinomas is controversial, although one study suggests they may occur in 10% of ZES cases. They do not occur in MEN 1. They are unique in that they have been cured by lymph node excision alone. This case demonstrates that gastrinomas may develop primarily in LNs rather than the more commonly suspected solid organs.

Beyond the GIST: Mesenchymal Tumors of the Stomach

Intramural gastric masses arise in the wall of the stomach (generally within the submucosa or muscularis propria), often with intact overlying mucosa. These tumors are typically mesenchymal in origin and have overlapping radiologic appearances. A combination of features such as location, attenuation, enhancement, and growth pattern may suggest one diagnosis over another. Gastrointestinal stromal tumors (GISTs) account for the majority of intramural tumors and can vary widely in appearance, from small intraluminal lesions to exophytic masses that protrude into the peritoneal cavity, commonly with areas of hemorrhage or necrosis. A well-circumscribed mass measuring -70 to -120 HU is a lipoma. Leiomyomas usually manifest as low-attenuation masses at the gastric cardia. Homogeneous attenuation is a noteworthy characteristic of schwannomas, particularly for larger lesions that might otherwise be mistaken for GISTs. A hypervascular mass in the antrum is a common manifestation of glomus tumors. Hemangiomas are also hypervascular but often manifest in childhood. Inflammatory fibroid polyps usually arise as a polypoid mass in the antrum. Inflammatory myofibroblastic tumors are infiltrative neoplasms with a propensity for local recurrence. Plexiform fibromyxomas are rare, usually antral tumors. Carcinoid tumors are epithelial in origin, but often submucosal in location, and therefore should be distinguished from other intramural lesions. Multiple carcinoid tumors are associated with hypergastrinemia, either in the setting of chronic atrophic gastritis or Zollinger-Ellison syndrome. Sporadic solitary carcinoid tumors not associated with hypergastrinemia have a higher rate of metastasis. Histopathologic analysis, including immunohistochemistry, is usually required for diagnosis of intramural masses.

FORMULATION AND IN VITRO EVALUATION OF OMEPRAZOLE FAST DISSOLVING TABLETS

Omeprazole is a proton pump inhibitor used in the treatment of dyspepsia, peptic ulcer disease (PUD), Gastroesophageal reflux disease (GERD), laryngopharyngeal reflux disease (LPR) and Zollinger-Ellison syndrome. The present study deals with the formulation of omeprazole fast dissolving tablets utilising cross linked alginic acid and calcium silicate as super disintegrates and a total of 8 formulation batches were prepared. All the pre compression and post compression parameters are studied and the results comply with in the limits. In vitro dissolution studies explained that the optimised F7 formulation with super disintegrants Cross linked alginic acid at low ratio and Calcium silicate at high ratio showed a cumulative release of 99.6 % of drug at the end of 12 minutes and also exhibited first order kinetics with Higuchi mechanism of drug release.

Hypochlorhydria and Achlorhydria Are Associated With False-Positive Secretin Stimulation Testing for Zollinger-Ellison Syndrome

Secretin stimulation testing (SST) is used to evaluate patients with hypergastrinemia in the diagnosis of Zollinger-Ellison syndrome. Case series have documented false-positive SST in patients with achlorhydria. This study reviews our experience with SST in hypochlorhydric and achlorhydric patients. We examined 27 patients with hypochlorhydria or achlorhydria based on a predefined basal acid output (BAO) measurement of less than 5.0 mEq/h who also underwent SST for diagnosis of Zollinger-Ellison syndrome. We report the frequency of false-positive SST results in this setting. Three hundred thirty patients underwent gastric analysis of which 27 had BAO of less than 5.0 mEq/h and SST conducted. The mean (SD) fasting gastrin level was 247 (304) pg/mL, and the mean (SD) BAO measurement was 1.6 (1.8) mEq/h. Twenty patients were off, and 7 were on antisecretory therapy at time of testing. Four patients had false-positive SST results: 3 with gastric atrophy (BAO = 0 mEq/h) and 1 with drug-induced hypochlorhydria (BAO = 0.5 mEq/hr). These false-positive test results were confirmed by structural and functional imaging studies. We have identified a 14.8% false-positive rate in SST in patients with hypochlorhydria or achlorhydria. Growing literature has identified severe consequences associated with discontinuing antisecretory treatment for testing; therefore, SST will require interpretation in the setting of gastric acid suppression and needs to be interpreted in this context.

Selective Arterial Embolization of Liver Metastases from Gastrinomas: A Single-Centre Experience.

Background. Gastrinomas are rare functional neuroendocrine tumors causing the Zollinger-Ellison syndrome (ZES). At presentation, up to 25% of gastrinomas are metastasized, predominantly to the liver. Embolization of liver metastases might reduce symptoms of ZES although a postembolization syndrome can occur. In this study, the results of embolization are presented, and the literature results are described. Methods. From a prospective database of pancreatic neuroendocrine tumors, all patients with liver metastatic gastrinomas were selected if treated with arterial embolization. Primary outcome parameters were symptom reduction, complications, and response rate. The literature search was performed with these items. Results. Three patients were identified; two presented with synchronous liver metastases. All the three patients had symptoms of ZES before embolization. Postembolization syndrome occurred in two patients. Six months after embolization, all the 3 patients had a clinical and complete radiological response; a biochemical response was seen in 2/3 patients. From the literature, only a small number of gastrinoma patients treated with liver embolization for liver metastases were found, and similar results were described. Conclusion. Selective liver embolization is an effective and safe therapy for the treatment of liver metastatic gastrinomas in the reduction of ZES. Individual treatment strategies must be made for the optimal success rate.

Primary Lymph Node Gastrinoma

Gastrinoma is a hormone-secreting tumor associated with the Zollinger-Ellison syndrome. It is quite rare among children. The discovery of gastrinomas in unusual locations such as lymph nodes, bones, ovaries, and the liver poses a diagnostic dilemma as to whether the tumor is primary or metastatic. Here, we present a case of a primary gastrinoma within a lymph node.

Wireless Telemetry and Cystic Fibrosis: Just the pHacts

Wireless Telemetry and Cystic Fibrosis: Just the pHacts

Gastric Neuroendocrine Tumors

Gastric neuroendocrine tumors (NETs) are rare and can occur in patients with chronic atrophic gastritis (type I), multiple endocrine neoplasia 1 syndrome, or Zollinger–Ellison syndrome (type II), or sporadically (type III). Types I and II gastric NETs usually present as multiple small sessile lesions and are usually found coincidentally. Type III lesions are usually larger, isolated, and responsible for iron deficiency anemia and abdominal pain. This article is part of an expert encyclopedia.

A Modified Technique for Esophagojejunostomy or Esophagogastrostomy After Laparoscopic Gastrectomy

Reconstruction of the digestive tract involving esophageal anastomosis after laparoscopic gastrectomy is a surgically difficult procedure. In this study, a newly developed transoral pretilted circular anvil, a "the oral to the abdomen" method, was proven to be effective. A total of 34 consecutive patients underwent esophageal anastomosis using the OrVil in our hospital from July 2009 to February 2011. The esophagus was transected and a small hole was then made in the esophageal stump through which the nasogastric tube of the OrVil was passed to insert the anvil into the abdominal cavity. After fixation with a stapler and a glove at the jejunal loop or the remnant stomach, the abdominal cavity was entered through the minilaparotomy. Pneumoperitoneum and airtightness were reestablished after the glove edge was turned over to seal off the protector. Eventually, intracorporeal esophagojejunostomy or esophagogastrostomy was accomplished under the guidance of laparoscopy. There were 34 patients in the study: 1 with Zollinger-Ellison syndrome, 7 with stromal tumors in cardia, 23 with adenocarcinoma in the stomach, and 3 with cardia adenocarcinoma involving the lower esophagus. The surgical margins for all tumor patients were negative for tumor cells. The mean operative time was 175.0 minutes (90 to 240 min) and the mean intraoperative blood loss was 195.6 mL (50 to 800 mL). The 34 patients underwent successful laparoscopic surgeries with no open conversions. For 32 patients, there were no technological complications in the transoral insertion of the anvil to the esophageal stump. There were no anastomotic leaks after the surgery. The use of the OrVil device, a "the oral to the abdomen" method, changes the direction of the anvil insertion and significantly decreases both difficulty and duration of the laparoscopic surgery. More importantly, if the mass is at a higher position, this approach can achieve a higher surgical margin compared with the hand-sewn purse-string suture technique, thus avoiding the need to undergo a thoracotomy (Supplemental Digital Content 1, http://links.lww.com/SLE/A83).

Causes of Death and Prognostic Factors in Multiple Endocrine Neoplasia Type 1

Multiple endocrine neoplasia type 1 (MEN1) is classically characterized by the development of functional or nonfunctional hyperplasia or tumors in endocrine tissues (parathyroid, pancreas, pituitary, adrenal). Because effective treatments have been developed for the hormone excess state, which was a major cause of death in these patients in the past, coupled with the recognition that nonendocrine tumors increasingly develop late in the disease course, the natural history of the disease has changed. An understanding of the current causes of death is important to tailor treatment for these patients and to help identify prognostic factors; however, it is generally lacking.To add to our understanding, we conducted a detailed analysis of the causes of death and prognostic factors from a prospective long-term National Institutes of Health (NIH) study of 106 MEN1 patients with pancreatic endocrine tumors with Zollinger-Ellison syndrome (MEN1/ZES patients) and compared our results to those from the pooled literature data of 227 patients with MEN1 with pancreatic endocrine tumors (MEN1/PET patients) reported in case reports or small series, and to 1386 patients reported in large MEN1 literature series. In the NIH series over a mean follow-up of 24.5 years, 24 (23%) patients died (14 MEN1-related and 10 non-MEN1-related deaths). Comparing the causes of death with the results from the 227 patients in the pooled literature series, we found that no patients died of acute complications due to acid hypersecretion, and 8%–14% died of other hormone excess causes, which is similar to the results in 10 large MEN1 literature series published since 1995. In the 2 series (the NIH and pooled literature series), two-thirds of patients died from an MEN1-related cause and one-third from a non-MEN1-related cause, which agrees with the mean values reported in 10 large MEN1 series in the literature, although in the literature the causes of death varied widely. In the NIH and pooled literature series, the main causes of MEN1-related deaths were due to the malignant nature of the PETs, followed by the malignant nature of thymic carcinoid tumors. These results differ from the results of a number of the literature series, especially those reported before the 1990s. The causes of non-MEN1-related death for the 2 series, in decreasing frequency, were cardiovascular disease, other nonendocrine tumors > lung diseases, cerebrovascular diseases. The most frequent non-MEN1-related tumor deaths were colorectal, renal > lung > breast, oropharyngeal. Although both overall and disease-related survival are better than in the past (30-yr survival of NIH series: 82% overall, 88% disease-related), the mean age at death was 55 years, which is younger than expected for the general population.Detailed analysis of causes of death correlated with clinical, laboratory, and tumor characteristics of patients in the 2 series allowed identification of a number of prognostic factors. Poor prognostic factors included higher fasting gastrin levels, presence of other functional hormonal syndromes, need for >3 parathyroidectomies, presence of liver metastases or distant metastases, aggressive PET growth, large PETs, or the development of new lesions.The results of this study have helped define the causes of death of MEN1 patients at present, and have enabled us to identify a number of prognostic factors that should be helpful in tailoring treatment for these patients for both short- and long-term management, as well as in directing research efforts to better define the natural history of the disease and the most important factors determining long-term survival at present.

Multiple gastrinomas of the duodenum in a patient with sporadic Zollinger-Ellison syndrome

Multiple gastrinomas of the duodenum in a patient with sporadic Zollinger-Ellison syndrome

Pancreatic Adenocarcinoma in a Patient With Multiple Endocrine Neoplasia 1 Syndrome

To the Editor We present the rare occurrence of a concurrent pancreatic neuroendocrine tumor (pNET) and pancreatic ductal adenocarcinoma (PDAC) in a patient with multiple endocrine neoplasia 1 (MEN1) syndrome. It is important for clinicians to consider the possibility of PDAC in patients with MEN1 because aggressive early surgical intervention provides the only chance of cure. Multiple endocrine neoplasia 1 is characterized by parathyroid adenomas, pNETs, and anterior pituitary tumors.1 Multiple endocrine neoplasia 1–associated pNETs are usually slowly progressive and associated with low malignant potential. In the context of MEN1, pancreatic NET size correlates with prognosis and the presence of metastases,2 and lesions of more than 2 cm are associated with greater genetic instability and malignant behaviour.3 The European Neuroendocrine Tumour Society recommendations for management of pancreatic lesions in a patient with MEN1 state that early diagnosis and surgical excision of MEN1-related pNET improve survival, preventing or delaying the development of distant metastases.4 It is mandatory to operate on MEN1-related nonfunctioning pancreatic tumors with metastases, size of more than 2 cm, or yearly increased size of more than 0.5 cm. Management of pNETs of less than 2 cm is controversial, current recommendation being intensive surveillance to avoid repeated intervention where lesions are typically multiple and behave in an indolent fashion.

A thymic carcinoid tumour causing Zollinger-Ellison and Cushing’s syndromes due to ectopic ACTH and gastrin secretion

Cushings’s syndrome due to ectopic adrenocorticotrophin (ACTH) secretion was first described nearly a century ago by Hurst Brown. Bronchial carcinomas, usually bronchial carcinoids, were the first to be described and continue to be the leading cause of the ectopic ACTH syndrome; however, thymic carcinoids account for approximately 10% of cases. The Zollinger Ellison syndrome, excessive gastrin secretion from a neuroendcorine tumour, has been described as exclusively due to neuroendocrine tumours of entero-pancreatic origin, while secretion of gastrin from a thymic carcinoid tumour has not been reported to our knowledge. We report a unique case of a 34-year-old woman with ACTH-dependent Cushing’s syndrome and Zollinger-Ellison syndrome due to a thymic carcinoid tumour. caSe report

Sporadic duodenal macrogastrinoma: a rare case report

Gastrinomas are rare neuroendocrine tumors characterized by the secretion of gastrin, which causes hyperchlorhydria, thereby producing the Zollinger-Ellison syndrome. In most cases this syndrome manifests as severe peptic ulcer disease. We are presenting an extremely rare clinical case of sporadic duodenal macrogastrinoma. The patient underwent investigation due to six-year history of epigastric pain, heartburn and episodic diarrhea. Endoscopy, endosonoscopy and histologic examination of biopsy specimens indicated the presence of duodenal gastrinoma with no signs of peptic ulcers. Pyloroduodenal segment including 3.5 cm macrogastrinoma was resected. This case is unique as duodenal gastrinomas are usually very small, up to 1 cm. During the follow up period we observed slowly decreasing hypergastrinemia. Somatostatin receptor scintigraphy, CT and upper GI endoscopy were performed to reveal the reasons, though did not find any abnormalities. 8 months of follow-up did not reveal any progression of the disease. The etiology of slowly decreasing hypergastrinemia remains unclear, controversial and is under investigation.

A case of Zollinger-Ellison syndrome diagnosed by duodenal ulcer perforation into the gallbladder

A 79-year-old woman presented with abdominal pain. Because of epigastric discomfort she had taken rabeprazole (10 mg/day) for 7 years and had discontinued it 3 weeks previously. She had no history of peptic ulcer disease, nonsteroidal anti-inflammatory drug ingestion, or Helicobacter pylori infection. Laboratory examination showed a white blood cell count of 8150/mm3, hemoglobin of 12.7 g/dL, platelet count of 260,000/mm3, and C-reactive protein of 5 mg/L (<4 mg/L). EGD revealed multiple ulcers in the second part of the duodenum, including a perforation with surrounding yellow mucus (A, arrow).

Biliary tree gastrinomas in multiple endocrine neoplasia type 1 syndrome

To describe our patients affected with ectopic biliary tree gastrinoma and review the literature on this topic. Between January 1992 and June 2012, 28 patients affected by duodenopancreatic endocrine tumors in multiple endocrine neoplasia type 1 (MEN1) syndrome underwent surgery at our institution. This retrospective review article analyzes our experience regarding seventeen of these patients subjected to duodenopancreatic surgery for Zollinger-Ellison syndrome (ZES). Surgical treatment consisted of duodenopancreatectomy (DP) or total pancreatectomy (TP). Regional lymphadenectomy was always performed. Any hepatic tumoral lesions found were removed during surgery. In MEN1 patients, removal of duodenal lesions can sometimes lead to persistence or recurrence of hypergastrinemia. One possible explanation for this unfavorable outcome could be unrecognized ectopic localization of gastrin-secreting tumors. This study described three cases among the seventeen patients who were found to have an ectopic gastrinoma located in the biliary tree. Seventeen MEN1 patients affected with ZES were analyzed. The mean age was 40 years. Fifteen patients underwent DP and two TP. On histopathological examination, duodeno pancreatic endocrine tumors were found in all 17 patients. Eighty-one gastrinomas were detected in the first three portions of the duodenum. Only one gastrinoma was found in the pancreas. The mean number of gastrinomas per patient was 5 (range 1-16). Malignancy was established in 12 patients (70.5%) after lymph node, liver and omental metastases were found. Three patients exhibited biliary tree gastrinomas as well as duodenal gastrinoma(s). In two cases, the ectopic gastrinoma was removed at the same time as pancreatic surgery, while in the third case, the biliary tree gastrinoma was resected one year after DP because of recurrence of ZES. These findings suggest the importance of checking for the presence of ectopic gastrinomas in the biliary tree in MEN1 patients undergoing ZES surgery.

A Case of Zollinger-Ellison Syndrome in Multiple Endocrine Neoplasia Type 1 with Urolithiasis as the Initial Presentation

Zollinger-Ellison syndrome (ZES) is characterized by gastrinoma and resultant hypergastrinemia, which leads to recurrent peptic ulcers. Because gastrinoma is the most common pancreatic endocrine tumor seen in multiple endocrine neoplasia type I (MEN 1), the possibility of gastrinoma should be investigated carefully when patients exhibit symptoms associated with hormonal changes. Ureteral stones associated with hyperparathyroidism in the early course of MEN 1 are known to be its most common clinical manifestation; appropriate evaluation and close follow-up of patients with hypercalcemic urolithiasis can lead to an early diagnosis of gastrinoma. We report a patient with ZES associated with MEN 1, and urolithiasis as the presenting entity. A 51-year-old man visited the emergency department with recurrent epigastric pain. He had a history of calcium urinary stone 3 years ago, and 2 years later he had 2 operations for multiple jejunal ulcer perforations; these surgeries were 9 months apart. He was taking intermittent courses of antiulcer medication. Multiple peripancreatic nodular masses, a hepatic metastasis, parathyroid hyperplasia, and a pituitary microadenoma were confirmed by multimodal imaging studies. We diagnosed ZES with MEN 1 and performed sequential surgical excision of the gastrinomas and the parathyroid adenoma. The patient received octreotide injection therapy and close follow-up.

Combined Biological Therapy is Effective to Control all Neuroendocrine Tumor Manifestations in a Patient with MEN1 Syndrome

Multiple endocrine neoplasia type 1 (MEN1) is a hereditary syndrome predisposing to the development of many endocrine tumors. Mainly pituitary, parathyroids and pancreas are involved although a proliferative state interests all neuroendocrine system. MEN1 pancreatic neuroendocrine tumors (pNET) are multiples and can secrete different hormones resulting in specific endocrine syndrome. The therapeutic approach is based in surgery which usually is followed by tumor relapse or persistence unless to be highly aggressive. Biotherapy with somatostatin analogs (SSAs) and dopamine agonists could be of great benefit to manage these patients without altering their life quality. We report a case of a 36-year-old MEN1 man affected with multicentric PNETs associate with insulinoma and Zollinger-Ellison syndrome, pituitary prolactinoma and primary hyperparathyroidism. Therapy with symptomatic agents (proton pump inhibitors, diazoxide) as well as biotherapy (lanreotide, cabergoline) was started. At six month follow-up, symptomatic agents were stopped and disease control was only based on lanreotide plus cabergoline. This combined biotherapy was able to control endocrine syndromes and tumor growth, resulting in the possibility to perform a safer and selective surgical intervention on pNETs. Somatostatin and dopamine receptor expression has been evaluated and related to clinical response to biotherapy. Therapy with lanreotide and cabergoline was able to control multiple secreting tumors in a patient with MEN1, due to high expression of specific receptors. The combined use of somatostatin analogues and dopamine agonists is suggested in patients with MEN1.

Multiple endocrine neoplasia type 1 with upper gastrointestinal hemorrhage and perforation: A case report and review

Multiple endocrine neoplasia type 1 (MEN1) is a rare hereditary syndrome known to predispose subjects to endocrine neoplasms in a variety of tissues such as the parathyroid glands, pituitary gland, pancreas and gastrointestinal tract. We herein report a patient with a past history of pituitary adenoma, presenting with symptoms of chronic diarrhea for nearly one year and a sudden upper gastrointestinal hemorrhage as well as perforation without signs. Nodules in the duodenum and in the uncinate process and tail of pancreas and enlargement of the parathyroid glands were detected on preoperative imaging. Gastroscopy revealed significant ulceration and esophageal reflux diseases. The patient underwent subtotal parathyroidectomy and autotransplantation, pylorus-preserving pancreaticoduodenectomy and pancreatic tail resection and recovered well. The results observed in our patient suggest that perforation and bleeding of intestine might be symptoms of Zollinger-Ellison Syndrome in patients with MEN1.