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Zollinger-Ellison Syndrome HELP
Based on 125 articles published since 2010

These are the 125 published articles about Zollinger-Ellison Syndrome that originated from Worldwide during 2010-2020.
+ Citations + Abstracts
Pages: 1 · 2 · 3 · 4 · 5
1 Editorial Secretin stimulation test for gastrin release in Zollinger-Ellison syndrome: to do or not to do? 2013

Poitras, Pierre / Gingras, Marie-Hélène / Rehfeld, Jens F. · ·Pancreas · Pubmed #23851427.

ABSTRACT: -- No abstract --

2 Editorial [Advances in the diagnosis and treatment of Zollinger-Ellison syndrome]. 2012

Krysiak, Robert / Okopień, Bogusław / Herman, Zbigniew Stanisław. · ·Pol Merkur Lekarski · Pubmed #22993903.

ABSTRACT: Zollinger-Ellison syndrome is a rare disorder caused by tumor secretion of gastrin, which results in gastric acid hypersecretion and secondarily in complicated peptic ulcer and diarrhea. ZES might be associated with multiple endocrine neoplasia type 1. The two main principal therapeutic strategies are to control both the gastric acid hypersecretion and the growth of the tumor. Surgery often fails to be curative and therefore many patients requires medical therapy, mainly treatment with proton pump inhibitors. The aim of this paper was to critically discuss contemporary diagnosis and treatment of this neoplasm on the basis of progress made in recent years.

3 Editorial Digestive manifestations of parathyroid disorders. 2011

Abboud, Bassam / Daher, Ronald / Boujaoude, Joe. · ·World J Gastroenterol · Pubmed #22039319.

ABSTRACT: The parathyroid glands are the main regulator of plasma calcium and have a direct influence on the digestive tract. Parathyroid disturbances often result in unknown long-standing symptoms. The main manifestation of hypoparathyroidism is steatorrhea due to a deficit in exocrine pancreas secretion. The association with celiac sprue may contribute to malabsorption. Hyperparathyroidism causes smooth-muscle atony, with upper and lower gastrointestinal symptoms such as nausea, heartburn and constipation. Hyperparathyroidism and peptic ulcer were strongly linked before the advent of proton pump inhibitors. Nowadays, this association remains likely only in the particular context of multiple endocrine neoplasia type 1/Zollinger-Ellison syndrome. In contrast to chronic pancreatitis, acute pancreatitis due to primary hyperparathyroidism is one of the most studied topics. The causative effect of high calcium level is confirmed and the distinction from secondary hyperparathyroidism is mandatory. The digestive manifestations of parathyroid malfunction are often overlooked and serum calcium level must be included in the routine workup for abdominal symptoms.

4 Editorial Zollinger-Ellison syndrome: still a diagnostic challenge in the 21st century? 2011

Pritchard, D Mark. · ·Gastroenterology · Pubmed #21443889.

ABSTRACT: -- No abstract --

5 Editorial The new concept of therapeutic strategy for neuroendocrine tumors: important information from a case report of gastrinoma. 2010

Igarashi, Hisato / Ito, Tetsuhide / Takayanagi, Ryoichi. · ·Intern Med · Pubmed #20823641.

ABSTRACT: -- No abstract --

6 Review Surgical management of Zollinger-Ellison syndrome: Classical considerations and current controversies. 2019

Shao, Qian-Qian / Zhao, Bang-Bo / Dong, Liang-Bo / Cao, Hong-Tao / Wang, Wei-Bin. ·Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences/Peking Union Medical College, Beijing 100730, China. · Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences/Peking Union Medical College, Beijing 100730, China. wwb_xh@163.com. ·World J Gastroenterol · Pubmed #31528093.

ABSTRACT: 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.

7 Review Contemporary surgical management of the Zollinger-Ellison syndrome in multiple endocrine neoplasia type 1. 2019

Albers, Max B / Manoharan, Jerena / Bartsch, Detlef K. ·Department of Visceral, Thoracic, and Vascular Surgery, Philipps University Marburg, Baldingerstr, 35037 Marburg, Germany. Electronic address: albersm@med.uni-marburg.de. · Department of Visceral, Thoracic, and Vascular Surgery, Philipps University Marburg, Baldingerstr, 35037 Marburg, Germany. ·Best Pract Res Clin Endocrinol Metab · Pubmed #31521501.

ABSTRACT: About 30% of patients with MEN1 develop a Zollinger-Ellison syndrome. Meanwhile it is well established that the causative gastrinomas are almost exclusively localized in the duodenum and not in the pancreas, MEN1 gastrinomas occur multicentric and are associated with hyperplastic gastrin cell lesions and tiny gastrin-producing micro tumors in contrast to sporadic duodenal gastrinomas. Regardless of the high prevalence of early lymphatic metastases, the survival is generally good with an aggressive course of disease in only about 20% of patients. Symptoms can be controlled medically. The indication, timing, type, and extent of surgery are highly controversial and are discussed in detail in this article by a thorough and critical review of literature. More radical procedures, like partial pancreaticoduodenectomy, are weighed against less aggressive local excision of gastrinomas and the pros and cons of both approaches are discussed in terms of long-term morbidity, biochemical cure, and survival.

8 Review Gastrinomas: Medical or Surgical Treatment. 2018

Norton, Jeffrey A / Foster, Deshka S / Ito, Tetsuhide / Jensen, Robert T. ·Department of Surgery, Stanford University School of Medicine, 291 campus Drive, Stanford, CA 94305-5101, USA. · Neuroendocrine Tumor Centra, Fukuoka Sanno Hospital, International University of Health and Welfare, 3-6-45 Momochihama, Sawara-Ku, Fukuoka 814-0001, Japan. · Digestive Diseases Branch, NIDDK, National Institutes of Health, Building 10, Room 9C-103, Bethesda, MD 20892-1804, USA. Electronic address: robertj@bdg10.niddk.nih.gov. ·Endocrinol Metab Clin North Am · Pubmed #30098717.

ABSTRACT: This article reviews the role of surgical and medical management in patients with Zollinger-Ellison syndrome (ZES) due to a gastrin-secreting neuroendocrine tumor (gastrinoma). It concentrates on the status at present but also briefly reviews the changes over time in treatment approaches. Generally, surgical and medical therapy are complementary today; however, in some cases, such as patients with ZES and multiple endocrine neoplasia type 1, the treatment approach remains controversial.

9 Review Gastrinoma and Zollinger-Ellison syndrome in canids: a literature review and a case in a Mexican gray wolf. 2018

Struthers, Jason D / Robl, Nick / Wong, Valerie M / Kiupel, Matti. ·Animal Health Institute, Department of Pathology and Population Medicine, Midwestern University, Glendale, AZ (Struthers, Wong). · Veterinary Diagnostics and Infectious Diseases, Department of Animal, Dairy, and Veterinary Sciences, Utah State University, Logan, UT (Robl). · Veterinary Diagnostic Laboratory, Department of Pathobiology and Diagnostic Investigation, Michigan State University, Lansing, MI (Kiupel). ·J Vet Diagn Invest · Pubmed #29860931.

ABSTRACT: Gastrinoma, an infrequent diagnosis in middle-aged dogs, occurs with nonspecific gastrointestinal morbidity. Laboratory tests can yield a presumptive diagnosis, but definitive diagnosis depends on histopathology and immunohistochemistry. We describe a malignant pancreatic gastrinoma with lymph node metastases and corresponding Zollinger-Ellison syndrome in a Mexican gray wolf ( Canis lupus baileyi) and review this endocrine neoplasm in domestic dogs. A 12-y-old, captive, male Mexican gray wolf developed inappetence and weight loss. Abdominal ultrasonography revealed a thickened duodenum and peritoneal effusion. Two duodenal perforations were noted on exploratory celiotomy and were repaired. Persisting clinical signs led to a second celiotomy that revealed a mesenteric mass, which was diagnosed histologically as a neuroendocrine carcinoma. During the following 16 mo, the wolf received a combination of H

10 Review The Zollinger-Ellison syndrome: is there a role for somatostatin analogues in the treatment of the gastrinoma? 2018

Guarnotta, Valentina / Martini, Chiara / Davì, Maria Vittoria / Pizza, Genoveffa / Colao, Annamaria / Faggiano, Antongiulio / Anonymous80923. ·Biomedical Department of Internal and Specialist Medicine (DIBIMIS), Section of Endocrine-Metabolic Diseases, University of Palermo, Palermo, Italy. · Clinica Medica 3^, Department of Medicine, DIMED, University of Padova, Padova, Italy. chiara.martini@aopd.veneto.it. · Section of Endocrinology, Medicina Generale e Malattie Aterotrombotiche e Degenerative, Department of Medicine, University of Verona, Verona, Italy. · Department of Clinical Medicine and Surgery, "Federico II" University of Naples, Naples, Italy. · Thyroid and Parathyroid Surgery Unit, Istituto Nazionale per lo studio e la cura dei tumori "Fondazione G. Pascale" - IRCCS, Naples, Italy. ·Endocrine · Pubmed #29019150.

ABSTRACT: PURPOSE: Analyze the role of somatostatin analogues (SSAs) in the treatment of sporadic and MEN1-related gastrinomas, trying to define whether recent trials have changed the landscape of gastrinoma therapy. METHODS: We evaluate the rationale of SSA use in the treatment of gastrinomas, summarize the current literature concerning the effect of SSAs on the control of Zollinger-Ellison syndrome (ZES) and gastrinomas tumor progression and discuss their role in the most recent guidelines. RESULTS: The medical treatment of gastrinoma and related ZES is aimed at controlling acid hypersecretion and tumor progression, in inoperable patients. The use of proton pump inhibitors (PPIs) to control the syndrome is a cornerstone in the ZES therapy. SSAs are not usually indicated for antisecretory purpose, because PPIs are considered the treatment of choice, due to their long lasting high efficacy and oral availability. The antiproliferative effect of SSAs has been established by two placebo-controlled trials that have clearly demonstrated a significant increase in progression free survival in patients affected by non-functioning well-differentiated advanced neuroendocrine tumors (NETs). The recent ENETS guidelines recommend the use of SSAs in advanced well differentiated NETs as antiproliferative agents. CONCLUSIONS: The high sstr-expression in gastrinomas make them highly responsive to SSAs and support the use of such drugs to counteract the tumour growth in patients not amenable to surgical cure. Unfortunately, limited data, mainly case reports or small series, support the use of SSAs in advanced gastrinomas, therefore, it is difficult to quantify their ability to control tumour growth and disease progression.

11 Review Diagnosis and management of Zollinger-Ellison syndrome in 2018. 2018

De Angelis, Claudio / Cortegoso Valdivia, Pablo / Venezia, Ludovica / Bruno, Mauro / Pellicano, Rinaldo. ·Department of Gastroenterology and Digestive Endoscopy, AOU Città della Salute e della Scienza, University of Turin, Turin, Italy - eusdeang@hotmail.com. · Department of Gastroenterology and Digestive Endoscopy, AOU Città della Salute e della Scienza, University of Turin, Turin, Italy. ·Minerva Endocrinol · Pubmed #28949124.

ABSTRACT: Zollinger-Ellison syndrome (ZES) is a clinical syndrome characterized by gastric acid hypersecretion due to the ectopic secretion of gastrin by a gastrinoma, a neuroendocrine tumor (NET) which mostly develops in the duodenum and in the pancreas. This syndrome was first described by Zollinger and Ellison in 1964; if left untreated, ZES can lead to multiple complications mainly due to gastric hypersecretion and some patients can suffer from the complications of an advanced metastatic disease. Although its clinical features are considered typical, the diagnosis of ZES is often challenging for the clinician. A previous review was published in 2005 by our group, but in 12 years many things have changed: the diagnostic tools have been improved and many different therapeutical options are now available.

12 Review Catching the Zebra: Clinical Pearls and Pitfalls for the Successful Diagnosis of Zollinger-Ellison Syndrome. 2017

Mendelson, Aaron H / Donowitz, Mark. ·Division of Gastroenterology and Hepatology, Department of Internal Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA. amendel5@jhmi.edu. · Division of Gastroenterology and Hepatology, Department of Internal Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA. ·Dig Dis Sci · Pubmed #28776139.

ABSTRACT: Zollinger-Ellison syndrome (ZES) results from an ectopic gastrin-secreting tumor leading to peptic ulcer disease, reflux, and chronic diarrhea. While early recognition portends an excellent prognosis with >80% survival at 15 years, symptoms are often nonspecific making the diagnosis difficult to establish. Diagnosis involves a series of tests, including fasting gastrin, gastric pH, chromogranin A, and secretin stimulation. Performing these tests in the correct sequence and at the proper time is essential to avoid inaccurate results. Tumor localization is equally nuanced. Although providers have classically used

13 Review Neuroendocrine Tumors of the Stomach. 2017

Corey, Britney / Chen, Herbert. ·Department of Surgery, University of Alabama at Birmingham, KB 404, 1720 2nd Avenue South, Birmingham, AL 35294, USA. Electronic address: blprince@uabmc.edu. · Department of Surgery, UAB Hospital and Health System, UAB Comprehensive Cancer Center, University of Alabama at Birmingham, BDB 502, 1808 7th Avenue South, Birmingham, AL 35233, USA. ·Surg Clin North Am · Pubmed #28325190.

ABSTRACT: Gastric neuroendocrine tumors (NETs) are classified into three types. Type I gastric NETs are associated with chronic atrophic gastritis. They have a good prognosis and endoscopic resection is the mainstay of treatment. Type II gastric NETs are caused by hypergastrinemia. They have a poorer prognosis, and resection is required to control the disease. Endoscopic versus surgical resection is recommended for the gastric lesion. Type III gastric NETs are sporadic and not associated with any specific condition. They have the worst prognosis with the highest rate of metastatic disease, and oncologic resection is recommended. Medical therapies have some role.

14 Review The appropriate use of proton pump inhibitors (PPIs): Need for a reappraisal. 2017

Savarino, Vincenzo / Dulbecco, Pietro / de Bortoli, Nicola / Ottonello, Andrea / Savarino, Edoardo. ·Division of Gastroenterology, Department of Internal Medicine, University of Genoa, Genoa, Italy. Electronic address: vsavarin@unige.it. · Division of Gastroenterology, Department of Internal Medicine, University of Genoa, Genoa, Italy. · Department of Translational Research and New Technology in Medicine and Surgery, Division of Gastroenterology, University of Pisa, Cisanello Hospital, Pisa, Italy. · Department of Surgical and Diagnostic Integrated Sciences, University of Genoa, Genoa, Italy. · Division of Gastroenterology, Department of Surgery, Oncology and Gastroenterology, University of Padua, Padua, Italy. ·Eur J Intern Med · Pubmed #27784575.

ABSTRACT: The advent of powerful acid-suppressive drugs, such as proton pump inhibitors (PPIs), has revolutionized the management of acid-related diseases and has minimized the role of surgery. The major and universally recognized indications for their use are represented by treatment of gastro-esophageal reflux disease, eradication of Helicobacter pylori infection in combination with antibiotics, therapy of H. pylori-negative peptic ulcers, healing and prophylaxis of non-steroidal anti-inflammatory drug-associated gastric ulcers and control of several acid hypersecretory conditions. However, in the last decade, we have witnessed an almost continuous growth of their use and this phenomenon cannot be only explained by the simple substitution of the previous H2-receptor antagonists, but also by an inappropriate prescription of these drugs. This endless increase of PPI utilization has created an important problem for many regulatory authorities in terms of increased costs and greater potential risk of adverse events. The main reasons for this overuse of PPIs are the prevention of gastro-duodenal ulcers in low-risk patients or the stress ulcer prophylaxis in non-intensive care units, steroid therapy alone, anticoagulant treatment without risk factors for gastro-duodenal injury, the overtreatment of functional dyspepsia and a wrong diagnosis of acid-related disorder. The cost for this inappropriate use of PPIs has become alarming and requires to be controlled. We believe that gastroenterologists together with the scientific societies and the regulatory authorities should plan educational initiatives to guide both primary care physicians and specialists to the correct use of PPIs in their daily clinical practice, according to the worldwide published guidelines.

15 Review Treatment of symptomatic neuroendocrine tumor syndromes: recent advances and controversies. 2016

Ito, Tetsuhide / Lee, Lingaku / Jensen, Robert T. ·a Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences , Kyushu University , Fukuoka , Japan. · b Digestive Diseases Branch , NIDDK, NIH , Bethesda , MD , USA. ·Expert Opin Pharmacother · Pubmed #27635672.

ABSTRACT: INTRODUCTION: Neuroendocrine tumors(NETs), once thought rare, are increasing in frequency in most countries and receiving increasing-attention. NETs present two-treatment problems. A proportion is aggressive and a proportion has a functional, hormone-excess-state(F-NET), each of which must be treated. Recently, there have been many advances, well-covered in reviews/consensus papers on imaging-NETs; new, novel anti-tumor treatments and understanding their pathogenesis. However, little attention has been paid to advances in the treatment of the hormone-excess-state. These advances are usually reported in case-series, and case-reports with few large studies. In this paper these advances are reviewed. Areas covered: Advances in the last 5-years are concentrated on, but a review of literature from the last 10-years was performed. PubMed and other databases (Cochrane, etc.) were searched for F-NET-syndromes including carcinoid-syndrome, as well as meeting-abstracts on NETs. All advances that controlled hormone-excess-states or facilitated-control were covered. These include new medical-therapies [serotonin-synthesis inhibitors(telotristat), Pasireotide, new agents for treating ACTHomas], increased dosing with conventional therapies (octreotide-LAR, Lanreotide-Autogel), mTor inhibitors(everolimus), Tyrosine-kinase inhibitors(sunitinib),cytoreductive surgery, liver-directed therapies (embolization, chemoembolization, radioembolization, RFA), peptide radio-receptor-therapy(PRRT) and

16 Review Zollinger-Ellison Syndrome Associated with von Recklinghausen Disease: Case Report and Literature Review. 2016

Alshikho, Mohamad J / Noureldine, Salem I / Talas, Joud M / Nasimian, Antoine / Zazou, Safi / Mobaed, Bashir / Nasser, Mahmoud. ·Department of Neurology, Massachusetts General Hospital, Harvard University, Boston, MA, USA. · Department of Otolaryngology - Head and Neck Surgery, Johns Hopkins University, Baltimore, MD, USA. · Department of Dermatology, Aleppo University, Aleppo, Syrian Arab Republic. · Department of Radiology, Aleppo University, Aleppo, Syrian Arab Republic. · Department of General Internal Medicine, Aleppo University, Aleppo, Syrian Arab Republic. · Department of Gastroenterology, Aleppo University, Aleppo, Syrian Arab Republic. ·Am J Case Rep · Pubmed #27292293.

ABSTRACT: BACKGROUND: Pancreatic endocrine tumors (PETs) are rare and can occur as part of neurofibromatosis type 1 (NF1). Gastrinomas are functional PETs that are rarely associated with NF1. Only two cases of their occurrence have been reported in the literature. CASE REPORT: A 28-year-old woman was admitted for further evaluation of epigastric soreness, heartburn, nausea, vomiting, diarrhea, and a significant weight loss. Physical examination was remarkable for cutaneous findings (axillary freckling and multiple café-au-lait spots) as well as neurofibromas (dermal, plexiform). A diagnosis of NF1 was confirmed. Esophagogastroduodenoscopy (EGD) revealed multiple ulcers in the duodenum and the upper jejunum. A fasting gastrin level exceeded ten times the normal limit. An abdominal multi-slice 128 computed tomography (CT) scan revealed an oval mass of 26 mm in diameter adjacent to the second section of the duodenum. The patient was examined carefully to rule out multiple endocrine neoplasia type 1 (MEN1). Surgical resection was performed and a gastrinoma, causing Zollinger-Ellison syndrome (ZES), was diagnosed by histological examinations of the extirpated mass. The serum gastrin level decreased to normal limits shortly after surgery. Continuous follow-up revealed that the symptoms and the EGD findings completely resolved without recurrences. CONCLUSIONS: Although NF1 has common skeletal, visual, neurological, and cardiovascular complications, it also has a rare association with duodenal or pancreatic gastrinomas. Vigilance for this possible association is important to promote timely and careful management to help eliminate serious and potentially life-threatening complications.

17 Review [Management of gastrinoma]. 2016

Hain, Elisabeth / Coriat, Romain / Dousset, Bertrand / Gaujoux, Sébastien. ·AP-HP, hôpital Cochin, service de chirurgie digestive hépato-biliaire et endocrienne, Paris, France. · AP-HP, hôpital Cochin, service de gastroentérologie, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France; Université Paris Descartes, 12, rue de l'École-de-Médecine, 75006 Paris, France. · AP-HP, hôpital Cochin, service de chirurgie digestive hépato-biliaire et endocrienne, Paris, France; Université Paris Descartes, 12, rue de l'École-de-Médecine, 75006 Paris, France. · AP-HP, hôpital Cochin, service de chirurgie digestive hépato-biliaire et endocrienne, Paris, France; Université Paris Descartes, 12, rue de l'École-de-Médecine, 75006 Paris, France. Electronic address: sebastien.gaujoux@aphp.fr. ·Presse Med · Pubmed #27262229.

ABSTRACT: Gastrinoma is a very rare tumor leading to gastrin hypersecretion and characterised by Zollinger-Ellisson syndrome (ZES) i.e. severe gastric and duodenal ulceration and profuse diarrhea. This disease can be sporadic or familial within a multiple endocrine neoplasia type 1 (MEN-1) syndrome. Diagnosis is based on hypergastrinemia/hypercholrhydria. Tumors are usually located in the duodeno-pancreas. Preoperative tumor location by CT, echoendoscopy and fibroscopy is not always possible because of the small size of the lesion that are frequently multiple. The aim of gastrinoma treatment is 1/to control the hormonal hypersecretion 2/to remove the neoplasm when it is possible. Surgery is the only chance to cure. Gastrinoma is a slow-growing tumor, and overall survival is good with a median survival above 10years and a 5-year survival above 80 % in surgically resected patients. Recurrence is frequent, a biochemical recurrence is observed in 65 % of cases and morphological recurrence in 40 % of patients at 2years. Metastases are associated with a dismal prognosis.

18 Review [Endoscopic therapy for gastroduodenal neuroendocrine neoplasms]. 2016

Maasberg, S / Jürgensen, C / Scheerer, F / Pschowski, R / Felder, S / Begum, N / Wiedenmann, B / Pascher, A / Pape, U-F. ·Medizinische Klinik mit Schwerpunkt Hepatologie und Gastroenterologie (einschließlich Arbeitsbereich Stoffwechselerkrankungen), Charité - Universitätsmedizin Berlin, Campus Charité Mitte, Charitéplatz 1, 10117, Berlin, Deutschland. · Klinik für Gastroenterologie und Hepatologie, Klinikum Neuperlach, Städtisches Klinikum München, München, Deutschland. · Klinik für Allgemein-, Viszeral- und minimalinvasive Chirurgie, Kreiskrankenhaus Stadthagen, Klinik Schaumburg, Stadthagen, Deutschland. · Klinik für Allgemein-, Viszeral-, Gefäß- und Thoraxchirurgie, Charité - Universitätsmedizin Berlin, Campus Charité Mitte, Berlin, Deutschland. · Medizinische Klinik mit Schwerpunkt Hepatologie und Gastroenterologie (einschließlich Arbeitsbereich Stoffwechselerkrankungen), Charité - Universitätsmedizin Berlin, Campus Charité Mitte, Charitéplatz 1, 10117, Berlin, Deutschland. ulrich-frank.pape@charite.de. ·Chirurg · Pubmed #26960437.

ABSTRACT: Neuroendocrine neoplasms (NEN) represent a group of potentially malignant tumors, which can be located in every section of the gastrointestinal tract, the pancreas and the bronchopulmonary system. Gastroduodenal NENs have a relatively good prognosis in comparison to other subentities, e.g. pancreatic or ileojejunal NENs. In the stomach there are four different types of NENs, while in the duodenum there are five types and all vary in the malignant potential and the therapeutic approach. Due to the simple access endoscopic methods not only have diagnostic but also important therapeutic relevance in this subgroup. Lesions smaller than 1 cm can easily be resected with forceps or snare resection and for larger lesions up to 2 cm more invasive strategies, such as endoscopic mucosal resection (EMR) or endoscopic submucosal dissection (ESD) are available. Important criteria in gastric NEN for the risk evaluation of endoscopic treatment alone are the size of the lesion, depth of invasion and the tumor biology, e.g. neuroendocrine tumor (NET) G1/G2 versus neuroendocrine carcinoma (NEC) G3. In duodenal NEN the aforementioned risk factors also apply and in addition only lesions outside the ampulla of Vater should be endoscopically resected whereas periampullary lesions need to be addressed surgically. As an individualized therapeutic approach the possibility of a combined endoscopic and laparoscopic resection technique exists. Follow-up endoscopic investigations are necessary, especially in gastric type 1 NENs, which have a tendency to relapse.

19 Review [Evolution of clinical ideas about Zollinger-Ellison syndrome]. 2014

Maev, I V / Andreev, D N / Kucheryavyi, Yu A / Barkalova, E V. · ·Ter Arkh · Pubmed #24772514.

ABSTRACT: The paper gives the current views of the diagnosis and treatment of Zollinger-Ellison syndrome (ZES). It underlines the importance of including ZES in differential diagnosis in patient with frequently recurrent and standard-dose proton pump inhibitor therapy-resistant erosive and ulcerative lesions of the upper gastrointestinal tract. It provides the current stepwise algorithm for the diagnosis of the pathology in question. Relevant and promising treatments in patients with ZES are considered.

20 Review Zollinger-Ellison syndrome: classical considerations and current controversies. 2014

Epelboym, Irene / Mazeh, Haggi. ·Department of Surgery, Columbia University Medical Center, New York, New York, USA; Department of Surgery, Hadassah-Hebrew University Medical Center, Mount Scopus, Jerusalem, Israel. ·Oncologist · Pubmed #24319020.

ABSTRACT: Zollinger-Ellison syndrome (ZES) is an endocrinopathy characterized by gastrin-secreting tumors, responsible for causing the formation of multiple, refractory, and recurrent peptic ulcers in the distal duodenum and proximal jejunum. Two main variants have been described, sporadic and those found in association with parathyroid and pituitary tumors, a genetic disorder known as multiple endocrine neoplasia-1 (MEN-1). Biochemical serum evaluation for elevated gastrin, followed by radiological or nuclear localization of the primary lesion, is mandated for establishing diagnosis. The mainstays of treatment include management of hypersecretory state with medical suppression of gastric acid production and surgical resection of primary tumor for the prevention of malignant transformation and metastatic complications. Medical therapy with proton pump inhibitors has virtually eliminated the need for acid-reducing surgical procedures. Surgical approach to sporadic and MEN-1-associated ZES varies based on our understanding of the natural history of the condition and the probability of cure; however, resection to a negative microscopic margin is indicated in both cases. Postoperative surveillance involves measurement of gastrin level, followed by imaging if elevation is detected. Re-excision of recurrent or resection of metastatic disease is a subject of controversy; however, at the present time aggressive cytoreductive approach is favored.

21 Review Biliary tree gastrinomas in multiple endocrine neoplasia type 1 syndrome. 2013

Tonelli, Francesco / Giudici, Francesco / Nesi, Gabriella / Batignani, Giacomo / Brandi, Maria Luisa. ·Francesco Tonelli, Francesco Giudici, Gabriella Nesi, Giacomo Batignani, Maria Luisa Brandi, Surgical Unit, Department of Surgery and Translational Medicine, University of Florence, 50014 Florence, Italy. ·World J Gastroenterol · Pubmed #24363522.

ABSTRACT: 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.

22 Review Current management of the Zollinger-Ellison syndrome. 2013

Krampitz, Geoffrey W / Norton, Jeffrey A. ·Stanford University School of Medicine, Department of Surgery, 300 Pasteur Drive, H3591, Stanford, CA 94305-5655, USA. ·Adv Surg · Pubmed #24298844.

ABSTRACT: In summary, ZES is a syndrome caused by gastrinoma, usually located within the gastrinoma triangle and associated with symptoms of peptic ulcer disease, GERD, and diarrhea. The diagnosis of ZES is made by measuring fasting levels of serum gastrin, BAO, and the secretin stimulation test. Because of the high association of ZES and MEN1, HPT must be excluded by obtaining a serum calcium and parathyroid hormone level. Treatment of ZES consists of medical control of symptoms with PPIs and evaluation for potentially curative surgical intervention. Noninvasive imaging studies including SRS, CT, and MRI should be performed initially to evaluate for metastases and identify resectable disease. Invasive imaging modalities such as EUS may be performed to further evaluate primary tumors. IOUS, palpation, and duodenotomy are used for intraoperative localization of gastrinomas. In patients with MEN1, surgical resection should be pursued only if there is an identifiable tumor larger than 2 cm and after surgery for the primary hyperparathyroidism (3 1/2-gland parathyroidectomy). All patients with resectable localized sporadic gastrinoma should undergo surgical exploration, even those with biochemical evidence but negative imaging studies. Tumor is most commonly found in the duodenum, and the cure rate is high. In patients with liver metastases, surgery should be considered if all identifiable tumor can be safely removed. A multidisciplinary approach including surgical and nonsurgical therapies should be taken in patients with advanced disease.

23 Review Pancreatic neuroendocrine tumors. 2013

Krampitz, Geoffrey W / Norton, Jeffrey A. · ·Curr Probl Surg · Pubmed #24206780.

ABSTRACT: -- No abstract --

24 Review Zollinger-Ellison syndrome: recent advances and controversies. 2013

Ito, Tetsuhide / Igarashi, Hisato / Jensen, Robert T. ·aDepartment of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Higashi-ku, Fukuoka, Japan bDigestive Diseases Branch, NIDDK, NIH, Bethesda, Maryland, USA. ·Curr Opin Gastroenterol · Pubmed #24100728.

ABSTRACT: PURPOSE OF REVIEW: To review the recent advances and current controversies in patients with Zollinger-Ellison syndrome (ZES). RECENT FINDINGS: Recent advances in the management of ZES include: improved understanding of the pathogenesis of gastrinoma and pancreatic neuroendocrine tumors, new prognostic classification systems, new diagnostic algorithms, more sensitive localization studies, new treatment strategies including improved control of gastric acid secretion and role for surgery, and new approaches to patients with advanced disease. Controversies include: the best approach to a patient with hypergastrinemia suspected of possibly having ZES, the appropriate gastrin assay to use, the role of surgery in patients with ZES, especially those with multiple endocrine neoplasia type 1, and the precise order of therapeutic modalities in the treatment of patients with advanced disease. SUMMARY: This review updates clinicians regarding important advances and controversies required to optimally diagnose and manage patients with ZES.

25 Review Multiple endocrine neoplasia type 1 with upper gastrointestinal hemorrhage and perforation: a case report and review. 2013

Lu, Ying-Ying / Zhu, Feng / Jing, Da-Dao / Wu, Xie-Ning / Lu, Lun-Gen / Zhou, Gen-Quan / Wang, Xing-Peng. ·Department of Gastroenterology, Shanghai First People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200080, China. ·World J Gastroenterol · Pubmed #23482249.

ABSTRACT: 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.