Sporadic gastrinoma with refractory benign esophageal stricture:A case report

BACKGROUND Gastrinoma is characterized by an excessive release of gastrin,leading to hypersecretion of gastric acid,subsequently resulting in recurrent peptic ulcers,chronic diarrhea,and even esophageal strictures.This case report aims to improve awareness and facilitate early diagnosis and treatment of gastrinoma by presenting a rare case of gastrinoma with refractory benign esophageal stricture(RBES).Additionally,it highlights the persistent challenges that gastroenterologists encounter in managing RBES.CASE SUMMARY This case demonstrates a patient with gastrinoma who developed RBES and complete esophageal obstruction despite management with maximal acid suppressive therapy,multiple endoscopic bougie dilations and endoscopic incisional therapy(EIT).CONCLUSION It is essential to diagnose gastrinoma as early as possible,as inadequately controlled acid secretion over an extended period increases the risk of developing severe esophageal strictures.In patients with esophageal strictures causing complete luminal obstruction,blind reopening EIT presents challenges and carries a high risk of perforation.

Typical Zollinger-Ellison syndrome-atypical location of gastrinoma and absence of hypergastrinemia:A case report and review of literature

BACKGROUND Zollinger–Ellison syndrome(ZES)results from hypersecretion of gastrin from pancreatic or duodenal neuroendocrine tumors,commonly referred to as gastrinomas.The high levels of gastrin lead to a typical presentation involving watery diarrhea and multiple ulcers in the duodenum.Here,we have presented the rare case of a patient with ZES and absence of hypergastrinemia as well as an atypical location of gastrinoma.CASE SUMMARY A 72-year-old woman presented with the typical clinical manifestations of ZES,including upper abdominal pain,significant watery diarrhea,and acidic liquid vomitus.Surprisingly,however,she did not have an increased level of serum gastrin.In addition,there was no evidence of gastrinoma or any other ulcerogenic tumor.Esophagogastroduodenoscopy was conducted to examine the upper digestive tract.Revised diagnoses were considered,and an individualized treatment plan was developed.The patient responded to antacid medication while experiencing intermittent,recurring bouts of ZES.18F-AlF-NOTAoctreotide positron emission tomography(18F-OC PET)/computed tomography(CT)helped locate the tumor.Postoperative pathology and immunohistochemistry results suggested that the tumor was a gastrinoma located at an unconventional site.CONCLUSION This present case study demonstrates the possibility of ZES-like manifestation in patients with absence of hypergastrinemia.18F-OC PET/CT is a relatively new imaging technique that can be applied for diagnosing even tiny gastrinomas that are atypical in terms of location.

Large primary hepatic gastrinoma in young patient treated with trisegmentectomy: A case report and review of the literature

Primary hepatic gastrinoma is a rare disease, with fewer than 40 cases reported in the medical literature. Because it is located in an organ in which metastases are common, its diagnosis is difficult. We report a case of a 19 years old male patient with a history of gastric ulcers since the age of nine. Following gastric surgery, an antrectomy and a vagotomy, there was some alleviation of symptoms. Subsequently, the patient reported various intermittent episodes of diarrhea, diffuse abdominal pain, and vomiting. The patient underwent tomography, which revealed the presence of a hepatic mass measuring 19.5 cm × 12.5 cm × 17 cm. Primary hepatic gastrinoma was diagnosed based on laboratory examinations that indicated hypergastrinemia and a positron emission tomography/magnetic resonance study with somatostatin analogue that confirmed the liver as the primary site. After hepatic trisegmentectomy(Ⅱ, Ⅲ, Ⅳ, Ⅴ, Ⅷ), the patient's symptoms improved. The case is notable for the presence of a rare tumor with uncommon dimensions.

Biliary tree gastrinomas in multiple endocrine neoplasia type 1 syndrome

AIM:To describe our patients affected with ectopic biliary tree gastrinoma and review the literature on this topic.METHODS: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.RESULTS: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.CONCLUSION:These findings suggest the importance of checking for the presence of ectopic gastrinomas in the biliary tree in MEN1 patients undergoing ZES surgery.

Sporadic versus hereditary gastrinomas of the duodenum and pancreas: Distinct clinico-pathological and epidemiological features

Gastrinomas are defined as gastrin secreting tumors that are associated with Zollinger-Ellison syndrome （ZES）. ZES is characterized by elevated fasting gastrin serum levels, positive secretin stimulation test and clinical symptoms such as recurrent peptic ulcer disease, gastroesophageal reflux disease and occasional diarrhea. Genetically, nonhereditary （sporadic） gastrinomas are distinguished from hereditary gastrinomas, which are associated with multiple endocrine neoplasia type 1 （MEN1） syndrome. In general, duodenal gastrinomas are small and solitary if they are sporadic and multiple as well as hereditary. The sporadic gastrinomas occur in the duodenum or in the pancreas while the hereditary gastrinomas almost all occur in the duodenum. Our series of 77 sporadic duodenal neuroendocrine tumors （NETs） includes 18 patients （23.4%） with gastrinomas and ZES. Of 535 sporadic NETs in the pancreas collected from the NET archives of the departments of pathology in Zürich, Switzerland, and Kiel, Germany, 24 patients （4.5%） suffered from sporadic pancreatic gastrinomas and ZES. These NETs have to be distinguished from tumors with immunohistochemical positivity for gastrin but without evidence of ZES. An additional 19 patients suffered from MEN1 and ZES. These patients showed exclusively duodenal gastrinomas, but not pancreatic gastrinomas. The prognosis of sporadic and MEN1- associated duodenal gastrinomas is better than that of pancreatic gastrinomas, since they progress slowly to liver metastasis. In summary, sporadic and MEN1- associated gastrinomas in the duodenum and pancreas show different clinico-pathological and genetic features. The incidence of sporadic duodenal gastrin-producing tumors is increasing, possibly due to optimized diagnostic procedures. In contrast, pancreatic MEN1- associated gastrinomas seem to be extremely rare. A considerable subset of tumors with immunohistochemical expression of gastrin but without evidence of ZES should be designated as functionally inactive NETs expressing gastrin, but not as gastrinomas.

Biochemically curative surgery for gastrinoma in multiple endocrine neoplasia type 1 patients

AIM： To search for the optimal surgery for gastrinoma and duodenopancreatic neuroendocrine tumors in patients with multiple endocrine neoplasia type 1. METHODS： Sixteen patients with genetically confirmed multiple endocrine neoplasia type 1 （MEN 1） and Zollinger-Ellison syndrome （ZES） underwent resection of both gastrinomas and duodenopancreatic neuroendocrine tumors （NETs） between 1991 and 2009. For localization of gastrinoma, selective arterial secretagogue injection test （SASI test） with secretin or calcium solution was performed as well as somatostatin receptor scintigraphy （SRS） and other imaging methods such as computed tomography （CT） or magnetic resonance imaging （MRI）. The modus of surgery for gastrinoma has been changed over time, searching for the optimal surgery： pancreaticoduodenectomy （PD） was first performed guided by localization with the SAST test, then local resection of duodenal gastrinomas with dissection of regional lymph nodes （LR）, and recently pancreas-preserving total duodenectomy （PPTD） has been performed for multiple duodenal gastrinomas. RESULTS： Among various types of preoperative localizing methods for gastrinoma, the SASI test was the most useful method. Imaging methods such as SRS or CT made it essentially impossible to differentiate functioning gastrinoma among various kinds of NETs. However, recent imaging methods including SRS or CT were useful for detecting both distant metastases and ectopic NETs; therefore they are indispensable for staging of NETs. Biochemical cure of gastrinoma was achieved in 14 of 16 patients （87.5%）; that is, 100% in 3 patients who underwent PD, 100% in 6 patients who underwent LR （although in 2 patients （33.3%） second LR was performed for recurrence of duodenal gastri- noma）, and 71.4% in 7 patients who underwent PPTD. Pancreatic NETs more than 1 cm in diameter were resected either by distal pancreatectomy or enucleations, and no hepatic metastases have developed postoperatively. Pathological study of the resected specimens revealed co-existence of pancreatic gastrinoma with duodenal gastrinoma in 2 of 16 patients （13%）, and G cell hyperplasia and/or microgastrinoma in the duodenal Brunner＇s gland was revealed in all of 7 duodenal specimens after PPTD. CONCLUSION： Aggressive resection surgery based on accurate localization with the SASI test was useful for biochemical cure of gastrinoma in patients with MEN 1.Imamura Metal. Curative resection of gastrinoma in MEN-1

Gastrinoma and Zollinger Ellison syndrome:A roadmap for the management between new and old therapies

Zollinger-Ellison syndrome(ZES)associated with pancreatic or duodenal gastrinoma is characterized by gastric acid hypersecretion,which typically leads to gastroesophageal reflux disease,recurrent peptic ulcers,and chronic diarrhea.As symptoms of ZES are nonspecific and overlap with other gastrointestinal disorders,the diagnosis is often delayed with an average time between the onset of symptoms and final diagnosis longer than 5 years.The critical step for the diagnosis of ZES is represented by the initial clinical suspicion.Hypergastrinemia is the hallmark of ZES;however,hypergastrinemia might recognize several causes,which should be ruled out in order to make a final diagnosis.Gastrin levels>1000 pg/mL and a gastric pH below 2 are considered to be diagnostic for gastrinoma;some specific tests,including esophageal pH-recording and secretin test,might be useful in selected cases,although they are not widely available.Endoscopic ultrasound is very useful for the diagnosis and the local staging of the primary tumor in patients with ZES,particularly in the setting of multiple endocrine neoplasia type 1.Some controversies about the management of these tumors also exist.For the localized stage,the combination of proton pump inhibitory therapy,which usually resolves symptoms,and surgery,whenever feasible,with curative intent represents the hallmark of gastrinoma treatment.The high expression of somatostatin receptors in gastrinomas makes them highly responsive to somatostatin analogs,supporting their use as anti-proliferative agents in patients not amenable to surgical cure.Other medical options for advanced disease are super-imposable to other neuroendocrine neoplasms,and studies specifically focused on gastrinomas only are scant and often limited to case reports or small retrospective series.The multidisciplinary approach remains the cornerstone for the proper management of this composite disease.Herein,we reviewed available literature about gastrinoma-associated ZES with a specific focus on differential diagnosis,providing potential diagnostic and therapeutic algorithms.

Primary hepatic gastrinoma:Report of a case and review of literature

Primary hepatic gastrinoma is a very rare ectopic gastrinoma with less than 20 cases reported worldwide. We report the case of a patient with hypergastrinemia who was subjected to exhaustive preoperative and intraoperative imaging and also careful surgical exploration of the duodenum and pancreas which failed initially to identify the primary tumour. Eventually the patient was subjected to left liver lobectomy,as a small palpable lesion was noted intraoperatively. The diagnosis of gastrinoma requires a high index of clinical suspicion and the flawless cooperation of many specialties.

THE DIAGNOSIS AND TREATMENT OF GASTRINOMA:REPORT OF 17 CASES

Objective.To study the experience of diagnosis and treatment of gastrinoma. Methods.A retrospective study of 17 patients with gastrinoma seen from 1978 through 1998 in PUMC hospital. Results.Three of the 17 cases were associated with multiple endocrine neoplasia type I(MEN I)syndrome.Tumors dispersed extensively in pancreas(31%),duodenum(25%),lymphnode(19%)and other place(25%).Ultrasonography and computed tomography(CT)were sensitive in localizing tumor in pancreas and liver,but were not so well for small tumor in duodenum and lymph node.The angiography,percutaneous transhepatic portal sampling(PTPS),scintigraphy and endoscopic ultrasonography(EUS)also can be used.Sixteen of 17 patients underwent 38 operations and 56 percent of the 16 cases underwent several operations.Seven patients at last performed total gastrectomy that was still considered as a choice of treatment.Tumor resection was rare because the advancing of tumor in most cases. Conclusions.Diagnosing gastrinoma in its early stage and striving for the tumor resection and reducing the metastasis of liver were the cruxes for good prognosis.

Percutaneous radiofrequency ablation approach through the spleen:initial case report for pancreatic tail gastrinoma

Gastrinoma has a low incidence, and the pancreas-originated gastrinoma is rare.Pancreatic gastrinoma patients with liver metastases have poor prognosis and short survival.Local treatment to reduce the tumor burden helps to improve symptoms and slows down tumor progression for patients with unresectable tumors.We report a case of pancreatic tail gastrinoma with unresectable liver metastases.The patient received a comprehensive minimally invasive interventional treatment, that is, chemoembolization and radiofrequency ablation for liver metastases, and percutaneous transplenic radiofrequency ablation combined with radioactive 125I seed implantation for pancreatic tail gastrinoma.The patient was followed up for more than 20 months, and showed no clear evidence of tumor recurrence.We explored the safety and feasibility of percutaneous transplenic radiofrequency ablation for unresectable pancreatic tail gastrinoma.This transplenic approach allow more indications for minimally invasive therapy and provides a new treatment option not only for patients with unresectable pancreatic tail tumor but also for patients refusing surgery.

Alterations of chromosome 3p in 24 cases of gastrinomas and their correlations with clinicopathological and prognostic features

Purpose::The pathogenesis of gastrinomas is largely unknown,and there is a lack of reliable genetic determinants that are useful to distinguish malignant and benign forms of this tumor or predict the prognosis of patients with this disease.Loss of heterozygosity(LOH)on chromosome 3p is reported to occur in pancreatic neuroendocrine tumors(PNETs)as well as in non-PNETs and its presence is reported to correlate with tumor prognosis in non-endocrine tumors.However,little data are available from prospective studies on gastrinomas.Experimental design::We assessed occurrence of 3p LOH in 24 gastrinomas and correlated its presence with tumor biological behavior and other clinicopathological features of gastrinomas.Results::Either 3p LOH or microsatellite instability involving 3p occurred in 11 of 24 tumors(46%).Seven(29%)gastrinomas had 3p LOH.Of the 7 gastrinomas with 3p LOH,5(71%)had 3p12 LOH with the marker D3S2406,which was the shortest region of highest overlap(SRO).Chromosome 3p LOH was not associated with aggressive biological behavior of gastrinomas or with poor prognosis of patients with gastrinoma.Similarly,3p12 LOH(SRO)was not correlated with aggressive growth of tumors and/or liver metastases.Conclusion::Gastrinomas have a relative high frequency of 3p12 LOH suggesting this area may harbor putative tumor suppressor gene(s),which may play a role in the tumorigenesis,but not aggressiveness,of a subset of these tumors.

胃泌素瘤的临床特点分析和诊断方法比较

背景:胃泌素瘤为一少见的神经内分泌肿瘤,临床诊断困难。目的:总结胃泌素瘤的临床特点,以提高其诊断水平。方法:回顾性分析北京协和医院1983年1月～2006年12月收治的23例胃泌素瘤患者的临床资料。结果:本组患者从出现症状到确诊平均需(5.5±0.9)年,确诊时合并肝转移者占60.9%。临床表现以腹痛(91.3%)、腹泻(78.3%)为主。胃泌素瘤腹泻多为水泻(88.9%),抑酸剂可缓解症状(94.4%)。胃液分析和空腹血清胃泌素测定的诊断敏感性分别为76.9%和45.5%。生长抑素受体显像对胃泌素瘤诊断率较高(90.0%)。胃泌素瘤可同时分泌其他肽类激素,本组胃泌素瘤80%分泌2种以上激素,50%分泌3种激素。结论:胃泌素瘤的腹泻发生率近80%,仅次于最常见的腹痛症状,临床上不容忽视,其特点为水泻,且抑酸剂治疗有效。生长抑素受体显像对胃泌素瘤诊断率较高。

胃泌素瘤的诊治经验

【目的】总结胃泌素瘤诊断和治疗经验。【方法】回顾北京协和医院自1978年至2004年3月收治的胃泌素瘤28例,作了分析和总结。【结果】原发病灶位于胰而多发的病灶11例,位于十二指肠3例,胰和十二指肠均有3例,胃窦部2例,三角区淋巴结2例,多发性内分泌肿瘤7例。治疗主要为切除原发灶及尽量切除肝转移灶。在大多数胰的胃泌素瘤,只有胃次全或全切除有助。【结论】从预后观点看,胃泌素瘤的早期诊断是关键。切除靶器官以及内科治疗如应用H2受体阻滞剂、生长抑素(善宁)亦有效。胃泌素瘤如为MENⅠ型的组成部分,预后远较散发的胃泌素瘤要好。

胃泌素瘤临床病理分析

目的 为提高临床对胃泌素瘤的诊断与治疗水平。方法 对本院近 30年收治的 1 2例胃泌素瘤病例进行总结分析。结果 本组胃泌素瘤患者除具有典型的高胃酸症状外 ,还具有以下有别于一般消化性溃疡的特点 :( 1 )腹泻 :多数病例 ( 7/1 2 )伴有严重腹泻 ,其中 1例出现休克 ;( 2 )多发性肿瘤 :3例患者伴有其它神经内分泌肿瘤 ;( 3)十二指肠多发结节 ( 3/1 2 ) :活检虽无异常 ,但有 2例经手术证实为粘膜下胃泌素瘤 ;( 4)多中心发生 :9例胃泌素瘤为多中心性 ,7例累及不同器官 ,以胰腺、胃、十二指肠多见 ;( 5 )多激素分泌 :6例患者中 5例分泌 2种以上激素 ;( 6 )部分病例对化疗相对敏感。结论 胃泌素瘤可表现为多中心发生、多激素分泌的特性 ,应力争做到不漏掉肿瘤并积极治疗。

Recent standardization of treatment strategy for pancreatic neuroendocrine tumors

Recent advances in localization techniques,such as the selective arterial secretagogue injection test(SASI test) and somatostatin receptor scintigraphy have promoted curative resection surgery for patients with pancreatic neuroendocrine tumors(PNET).For patients with sporadic functioning PNET,curative resection surgery has been established by localization with the SASI test using secretin or calcium.For curative resection of functioning PNET associated with multiple endocrine neoplasia type 1(MEN 1) which are usually multiple and sometimes numerous,resection surgery of the pancreas and/or the duodenum has to be performed based on localization by the SASI test.As resection surgery of PNET has increased,several important pathological features of PNET have been revealed.For example,in patients with Zollinger-Ellison syndrome(ZES),duodenal gastrinoma has been detected more frequently than pancreatic gastrinoma,and in patients with MEN 1 and ZES,gastrinomas have been located mostly in the duodenum,and pancreatic gastrinoma has been found to co-exist in 13% of patients.Nonfunctioning PNET in patients with MEN 1 becomes metastatic to the liver when it is more than 1 cm in diameter and should be resected after careful observation.The most important prognos-tic factor in patients with PNET is the development of hepatic metastases.The treatment strategy for hepatic metastases of PNET has not been established and aggressive resection with chemotherapy and trans-arterial chemoembolization have been performed with significant benefit.The usefulness of octreotide treatment and other molecular targeting agents are currently being assessed.

Clinically detected gastroenteropancreatic neuroendocrine tumors are on the rise: Epidemiological changes in Germany

AIM:To study the epidemiologic changes of gastroenteropancreatic neuroendocrine tumors(GEP-NET)in Germany,we analyzed two time periods 1976-1988 and1998-2006.METHODS:We evaluated epidemiological data of GEP-NET from the former East German National Cancer Registry(DDR Krebsregister,1976-1988)and its successor,the Joint Cancer Registry(GKR,1998-2006),which was founded after German reunification.Due to a particularly substantial database the epidemiological data from the federal states of Mecklenburg-Western Pomerania,Saxony,Brandenburg and Thuringia,covering a population of more than 10.8 million people,were analyzed.Survival probabilities were calculated using life table analysis.In addition,GEP-NET patients were evaluated for one or more second(non-GEP-NET)primary malignancies.RESULTS:A total of 2821 GEP neuroendocrine neoplasms were identified in the two registries.The overall incidence increased significantly between 1976 and2006 from 0.31(per 100.000 inhabitants per year)to2.27 for men and from 0.57 to 2.38 for women.In the later period studied(2004-2006),the small intestine was the most common site.Neuroendocrine(NE)neoplasms of the small intestine showed the largest absolute increase in incidence,while rectal NE neoplasms exhibited the greatest relative increase.Only the incidence of appendiceal NET in women showed little change between 1976 and 2006.Overall survival of patients varied for sex,tumor site and the two periods studied but improved significantly over time.Interestingly,about 20%of the GEP-NET patients developed one or more second malignancies.Their most common location was the gastrointestinal tract.GEP-NET patients without second malignancies fared better than those with one or more of them.CONCLUSION:The number of detected GEP-NET increased about 5-fold in Germany between 1976 and2006.At the same time,their anatomic distribution changed,and the survival of GEP-NET patients improved significantly.Second malignancies are common and influence the overall survival of GEP-NET patients.Thus,GEP-NET warrant our attention as well as intensive research on their tumorigenesis.

Neuroendocrine tumors of the small bowels are on the rise:Early aspects and management

Neuroendocrine tumors of the small bowel are on the rise. In the US they have increased by 300%-500% in the last 35 years. At the same time their prognosis is much improved. Today,most neuroendocrine tumors (NETs) of the duodenum are detected "incidentally" and therefore recognized at an early stage. Duodenal NETs which are well differentiated,not larger than 10 mm and limited to the mucosa/submucosa can be endoscopically resected. The management of duodenal NETs ranging between 10 and 20 mm needs an interdisciplinary discussion. Endoscopic ultrasound is the method of choice to determine tumor size and depth of infiltration. Surgery is recommended for well-differentiated duodenal NET tumors greater than 20 mm,for localized sporadic gastrinomas (of any size) and for localized poorly differentiated NE cancers. Surgery is recommended for any ileal NET. Advanced ileal NETs with a carcinoid syndrome are treated with longacting somatostatin analogs. This treatment significantly improves (progression-free) survival in patients with metastatic NETs of the ileum. For optimal NET management,tumor biology,type,localization and stage of the neoplasm,as well as the patient's individual circumstances have to be taken into account.

Surgical management of Zollinger-Ellison syndrome: Classical considerations and current controversies

Zollinger-Ellison syndrome(ZES)is characterized by gastric acid hypersecretion causing severe recurrent acid-related peptic disease.Excessive secretion of gastrin can now be effectively controlled with powerful proton pump inhibitors,but surgical management to control gastrinoma itself remains controversial.Based on a thorough literature review,we design a surgical algorithm for ZES and list some significant consensus findings and recommendations:(1)For sporadic ZES,surgery should be routinely undertaken as early as possible not only for patients with a precisely localized diagnosis but also for those with negative imaging findings.The surgical approach for sporadic ZES depends on the lesion location(including the duodenum,pancreas,lymph nodes,hepatobiliary tract,stomach,and some extremely rare sites such as the ovaries,heart,omentum,and jejunum).Intraoperative liver exploration and lymphadenectomy should be routinely performed;(2)For multiple endocrine neoplasia type 1-related ZES(MEN1/ZES),surgery should not be performed routinely except for lesions>2 cm.An attempt to perform radical resection(pancreaticoduodenectomy followed by lymphadenectomy)can be made.The ameliorating effect of parathyroid surgery should be considered,and parathyroidectomy should be performed first before any abdominal surgery for ZES;and(3)For hepatic metastatic disease,hepatic resection should be routinely performed.Currently,liver transplantation is still considered an investigational therapeutic approach for ZES.Well-designed prospective studies are desperately needed to further verify and modify the current considerations.

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 ZollingerEllison Syndrome in patients with MEN1.

Diagnosis of Zollinger-Ellison syndrome:Increasingly difficult

In the present paper the increasing difficulty of diag-nosis of Zollinger-Ellison syndrome (ZES) due to issues raised in two recent papers is discussed. These issues involve the difficulty and need to withdraw patients suspected of ZES from treatment with Proton Pump Inhibitors (omeprazole, esomeprazole, lansoprazole, rabeprazole, pantoprazole) and the unreliability of many gastrin radioimmunoassays. The clinical context of each of these important issues is reviewed and the conclusions in these articles commented from the per-spective of clinical management.