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https://www.readbyqxmd.com/read/29019150/the-zollinger-ellison-syndrome-is-there-a-role-for-somatostatin-analogues-in-the-treatment-of-the-gastrinoma
#1
REVIEW
Valentina Guarnotta, Chiara Martini, Maria Vittoria Davì, Genoveffa Pizza, Annamaria Colao, Antongiulio Faggiano
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...
October 10, 2017: Endocrine
https://www.readbyqxmd.com/read/28949124/diagnosis-and-management-of-zollinger-ellison-syndrome-in-2017
#2
Claudio DE Angelis, Pablo Cortegoso Valdivia, Ludovica Venezia, Mauro Bruno, Rinaldo Pellicano
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...
September 25, 2017: Minerva Endocrinologica
https://www.readbyqxmd.com/read/28859856/gastrin-induces-nuclear-export-and-proteasome-degradation-of-menin-in-enteric-glial-cells
#3
Sinju Sundaresan, Cameron A Meininger, Anthony J Kang, Amanda L Photenhauer, Michael M Hayes, Nirakar Sahoo, Jolanta Grembecka, Tomasz Cierpicki, Lin Ding, Thomas J Giordano, Tobias Else, David J Madrigal, Malcolm J Low, Fiona Campbell, Ann-Marie Baker, Haoxing Xu, Nicholas A Wright, Juanita L Merchant
BACKGROUND & AIMS: The multiple endocrine neoplasia, type 1 (MEN1) locus encodes the nuclear protein and tumor suppressor menin. MEN1 mutations frequently cause neuroendocrine tumors (NETs) such as gastrinomas, characterized by their predominant duodenal location and local metastasis at time of diagnosis. Diffuse gastrin cell hyperplasia precedes the appearance of MEN1 gastrinomas, which develop within submucosal Brunner's glands. We investigated how menin regulates expression of the gastrin gene and induces generation of submucosal gastrin-expressing cell hyperplasia...
August 28, 2017: Gastroenterology
https://www.readbyqxmd.com/read/28768698/the-future-advances-in-therapeutic-approach-and-management-strategies-for-men1
#4
Rachel S van Leeuwaarde, Joanne M de Laat, Carolina R C Pieterman, Koen M A Dreijerink, Menno Vriens, Gerlof D Valk
Multiple endocrine neoplasia type 1 is a rare autosomal inherited disorder associated with a high risk for patients to simultaneously develop tumours of the parathyroid glands, duodeno-pancreatic neuroendocrine tumors and tumors of the anterior pituitary gland. Early identification of MEN1 in patients enables presymptomatic screening of manifestations which makes timely interventions possible with the intention to prevent morbidity and mortality. Causes of death nowadays have shifted towards local or metastatic progression of malignant neuro endocrine tumors...
August 2, 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28705492/primary-lymph-node-gastrinoma-a-single-institution-experience
#5
Yufei Chen, Vikram Deshpande, Cristina Ferrone, Lawrence S Blaszkowsky, Sareh Parangi, Andrew L Warshaw, Keith D Lillemoe, Carlos Fernandez-Del Castillo
BACKGROUND: Gastrinomas are rare neuroendocrine tumors that ectopically secrete gastrin and classically originate within the duodenum or pancreas. The presence of primary lymph node gastrinoma is controversial. We report on a single institution's experience with gastrinoma, with focus on primary lymph node tumors. METHODS: Patients who underwent operative resection of gastrinoma between 1992 and 2016 at a single institution were identified. A diagnosis of primary lymph node gastrinoma was defined as tumor confined to one or more resected peripancreatic lymph nodes, negative localization for any extra-nodal disease and normal gastrin postresection...
July 10, 2017: Surgery
https://www.readbyqxmd.com/read/28694576/zollinger-ellison-syndrome-in-a-12-year-old-child
#6
Abir Lal Nath, Nandita A Saxena, Bharati K Kulkarni, Shyam S Borwankar, Hemant N Lahoti, Sanjay N Oak
The syndrome described by Zollinger and Ellison in 1955 is a rare clinical entity which is even rarer in children. This report describes a 12-year-old boy who presented with refractory peptic ulcer disease which was finally diagnosed to be due to a gastrinoma and was successfully treated.
July 2017: Journal of Indian Association of Pediatric Surgeons
https://www.readbyqxmd.com/read/28626400/long-term-efficacy-of-s-1-chemotherapy-plus-administration-of-octreotide-for-a-patient-with-metastatic-neuroendocrine-tumor-gastrinoma
#7
Sakura Hiraide, Sadahide Ono, Satoshi Kato
Metastatic neuroendocrine tumors (gastrinomas) have a poor prognosis. Octreotide can reduce gastrin levels and alleviate hormonal symptoms, and possibly slow tumor growth as well. No drugs were available except streptozocin for the treatment of metastatic pancreatic neuroendocrine tumor (PNET) in 2008. We report a case of PNET in a 53-year-old woman with multiple liver tumors treated with S-1 plus octreotide. After 6 months from the initiation of the treatment, the pancreatic tumor and liver metastases regressed, and the patient achieved partial response without the development of any serious adverse event...
May 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28597909/diagnostic-and-therapeutic-guidelines-for-gastro-entero-pancreatic-neuroendocrine-neoplasms-recommended-by-the-polish-network-of-neuroendocrine-tumours
#8
Beata Kos-Kudła, Jolanta Blicharz-Dorniak, Janusz Strzelczyk, Agata Bałdys-Waligórska, Tomasz Bednarczuk, Marek Bolanowski, Agnieszka Boratyn-Nowicka, Małgorzata Borowska, Andrzej Cichocki, Jarosław B Ćwikła, Massimo Falconi, Wanda Foltyn, Daria Handkiewicz-Junak, Alicja Hubalewska-Dydejczyk, Barbara Jarząb, Roman Junik, Dariusz Kajdaniuk, Grzegorz Kamiński, Agnieszka Kolasińska-Ćwikła, Aldona Kowalska, Robert Król, Leszek Królicki, Maciej Krzakowski, Jolanta Kunikowska, Katarzyna Kuśnierz, Paweł Lampe, Dariusz Lange, Anna Lewczuk-Myślicka, Andrzej Lewiński, Michał Lipiński, Magdalena Londzin-Olesik, Bogdan Marek, Anna Nasierowska-Guttmejer, Sergiusz Nawrocki, Ewa Nowakowska-Duława, Joanna Pilch-Kowalczyk, Violetta Rosiek, Marek Ruchała, Lucyna Siemińska, Anna Sowa-Staszczak, Teresa Starzyńska, Katarzyna Steinhof-Radwańska, Krzysztof Sworczak, Anhelli Syrenicz, Andrzej Szawłowski, Marek Szczepkowski, Ewa Wachuła, Wojciech Zajęcki, Anna Zemczak, Wojciech Zgliczyński, Krzysztof Zieniewicz
Progress in the diagnostics and therapy of gastro-entero-pancreatic (GEP) neuroendocrine neoplasms (NEN), the published results of new randomised clinical trials, and the new guidelines issued by the European Neuroendocrine Tumour Society (ENETS) have led the Polish Network of Neuroendocrine Tumours to update the 2013 guidelines regarding management of these neoplasms. We present the general recommendations for the management of NENs, developed by experts during the Third Round Table Conference - Diagnostics and therapy of gastro-entero-pancreatic neuroendocrine neoplasms: Polish recommendations in view of current European recommenda-tions, which took place in December 2016 in Żelechów near Warsaw...
2017: Endokrynologia Polska
https://www.readbyqxmd.com/read/28566601/challenging-differential-diagnosis-of-hypergastremia-and-hyperglucagonemia-with-chronic-renal-failure-report-of-a-case-with-multiple-endocrine-neoplasia-type-1
#9
Takaaki Murakami, Takeshi Usui, Yuji Nakamoto, Akio Nakajima, Yuki Mochida, Sumio Saito, Takahiro Shibayama, Nobuhisa Yamazaki, Tomonobu Hatoko, Tomoko Kato, Shin Yonemitsu, Seiji Muro, Shogo Oki
A 53-year-old woman developed end-stage renal failure during a 15-year clinical course of primary hyperparathyroidism and was referred to our hospital for evaluation of suspected multiple endocrine neoplasia type 1 (MEN1). Genetic testing revealed a novel deletion mutation at codon 467 in exon 10 of the MEN1 gene. Systemic and selective arterial calcium injection (SACI) testing revealed hyperglucagonemia and hypergastrinemia with positive gastrin responses. A pathological examination revealed glucagonoma and a lymph node gastrinoma...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28540972/gastroduodenal-neuroendocrine-neoplasms-including-gastrinoma-management-guidelines-recommended-by-the-polish-network-of-neuroendocrine-tumours
#10
Michał Lipiński, Grażyna Rydzewska, Wanda Foltyn, Elżbieta Andrysiak-Mamos, Agata Bałdys-Waligórska, Tomasz Bednarczuk, Jolanta Blicharz-Dorniak, Marek Bolanowski, Agnieszka Boratyn-Nowicka, Małgorzata Borowska, Andrzej Cichocki, Jarosław B Ćwikła, Massimo Falconi, Daria Handkiewicz-Junak, Alicja Hubalewska-Dydejczyk, Barbara Jarząb, Roman Junik, Dariusz Kajdaniuk, Grzegorz Kamiński, Agnieszka Kolasińska-Ćwikła, Aldona Kowalska, Robert Król, Leszek Królicki, Jolanta Kunikowska, Katarzyna Kuśnierz, Paweł Lampe, Dariusz Lange, Anna Lewczuk-Myślicka, Andrzej Lewiński, Magdalena Londzin-Olesik, Bogdan Marek, Anna Nasierowska-Guttmejer, Ewa Nowakowska-Duława, Joanna Pilch-Kowalczyk, Karolina Poczkaj, Violetta Rosiek, Marek Ruchała, Lucyna Siemińska, Anna Sowa-Staszczak, Teresa Starzyńska, Katarzyna Steinhof-Radwańska, Janusz Strzelczyk, Krzysztof Sworczak, Anhelli Syrenicz, Andrzej Szawłowski, Marek Szczepkowski, Ewa Wachuła, Wojciech Zajęcki, Anna Zemczak, Wojciech Zgliczyński, Beata Kos-Kudła
This paper presents the updated Polish Neuroendocrine Tumour Network expert panel recommendations on the management of neuroendocrine neoplasms (NENs) of the stomach and duodenum, including gastrinoma. The recommendations discuss the epidemiology, pathogenesis, and clinical presentation of these tumours as well as their diagnosis, including biochemical, histopathological, and localisation diagnoses. The principles of treatment are discussed, including endoscopic, surgical, pharmacological, and radionuclide treatments...
2017: Endokrynologia Polska
https://www.readbyqxmd.com/read/28507564/management-of-the-hormonal-syndrome-of-neuroendocrine-tumors
#11
Paweł Gut, Joanna Waligórska-Stachura, Agata Czarnywojtek, Nadia Sawicka-Gutaj, Maciej Bączyk, Katarzyna Ziemnicka, Jakub Fischbach, Kosma Woliński, Jarosław Kaznowski, Elżbieta Wrotkowska, Marek Ruchała
Gastroenteropancreatic neuroendocrine tumors (GEP/NET) are unusual and rare neoplasms that present many clinical challenges. They characteristically synthesize store and secrete a variety of peptides and neuroamines which can lead to the development of distinct clinical syndrome, however many are clinically silent until late presentation with mass effects. Management strategies include surgery cure and cytoreduction with the use of somatostatin analogues. Somatostatin have a broad range of biological actions that include inhibition of exocrine and endocrine secretions, gut motility, cell proliferation, cell survival and angiogenesis...
April 1, 2017: Archives of Medical Science: AMS
https://www.readbyqxmd.com/read/28503312/germline-and-somatic-genetic-changes-in-multicentric-tumors-obtained-from-a-patient-with-multiple-endocrine-neoplasia-type-1
#12
Akane Naruoka, Sumiko Ohnami, Takeshi Nagashima, Masakuni Serizawa, Keiichi Ohshima, Shumpei Ohnami, Kenichi Urakami, Yasue Horiuchi, Yoshimi Kiyozumi, Masato Abe, Takashi Nakajima, Teiichi Sugiura, Katsuhiko Uesaka, Masatoshi Kusuhara, Ken Yamaguchi
Multiple endocrine neoplasia type 1 (MEN1) is a hereditary cancer syndrome caused by germline mutations of the MEN1 gene located in chromosome 11q13. In patients with MEN1, multicentric tumors develop in the involved organs; however, precise evaluation of genetic changes in these multicentric tumors has not been performed. In the present study, using whole-exome sequencing, we analyzed germline and somatic genetic changes in blood cells, two pancreatic endocrine tumors and one duodenal tumor obtained from a patient with MEN1 gastrinoma...
2017: Human Genome Variation
https://www.readbyqxmd.com/read/28483790/effect-of-hormone-secretory-syndromes-on-neuroendocrine-tumor-prognosis
#13
REVIEW
Wouter T Zandee, Kimberly Kamp, Roxanne C van Adrichem, Richard A Feelders, Wouter W de Herder
The treatment of hormone hypersecretory syndromes caused by neuroendocrine tumors (NETs) can be a major challenge. NETs originating from the small intestine often secrete serotonin causing flushing, diarrhea and valve fibrosis, leading to dehydration or heart failure in severe cases. NETs from the pancreas can secrete a wider variety of hormones, like insulin, glucagon and gastrin leading to distinct clinical syndromes. Historically mortality in patients with functioning NETs was high due to the complications caused by the hypersecretion of hormones...
July 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28470558/phase-ii-study-of-lanreotide-autogel-in-japanese-patients-with-unresectable-or-metastatic-well-differentiated-neuroendocrine-tumors
#14
Tetsuhide Ito, Yoshitaka Honma, Susumu Hijioka, Atsushi Kudo, Akira Fukutomi, Akira Nozaki, Yasutoshi Kimura, Fuyuhiko Motoi, Hiroyuki Isayama, Izumi Komoto, Seiichi Hisamatsu, Akihiro Nakajima, Akira Shimatsu
Background Lanreotide is a long-acting somatostatin analog with demonstrated efficacy against enteropancreatic neuroendocrine tumor (NET) in the phase III (CLARINET) study. Materials and Methods In this single-arm study, Japanese patients with grade (G) 1/G2 NET received lanreotide (120 mg/4 weeks) for 48 weeks. Those who completed the study were enrolled in a long-term extension study. The primary endpoint was the clinical benefit rate (CBR) defined as a complete response, partial response (PR), or stable disease (SD) over 24-weeks...
August 2017: Investigational New Drugs
https://www.readbyqxmd.com/read/28458907/hypersecretion-of-acth-and-prl-from-pituitary-adenoma-in-men1-adequately-managed-by-medical-therapy
#15
Shinsuke Uraki, Hiroyuki Ariyasu, Asako Doi, Hiroto Furuta, Masahiro Nishi, Takeshi Usui, Hiroki Yamaue, Takashi Akamizu
SUMMARY: A 54-year-old man had gastrinoma, parathyroid hyperplasia and pituitary tumor. His family history indicated that he might have multiple endocrine neoplasia type 1 (MEN1). MEN1 gene analysis revealed a heterozygous germline mutation (Gly156Arg). Therefore, we diagnosed him with MEN1. Endocrinological tests revealed that his serum prolactin (PRL) and plasma adrenocorticotropic hormone (ACTH) levels were elevated to 1699 ng/mL and 125 pg/mL respectively. Immunohistochemical analysis of the resected pancreatic tumors revealed that the tumors did not express ACTH...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28446479/severe-chronic-diarrhoea-secondary-to-primary-lymph-node-gastrinoma
#16
Mouhanna Abu Ghanimeh, Khalil Abuamr, Esmat Sadeddin, Osama Yousef
The existence of primary lymph node (LN) gastrinoma is questionable and controversial. In fact, the presence of gastrinoma in such uncommon site raises the possibility of metastasis from another occult primary site. An extensive evaluation and careful follow-up is always warranted. A female aged 48 years presented with chronic abdominal pain and watery diarrhoea. Her serum gastrin and chromogranin were elevated, and an underlying gastrinoma was suspected. Further evaluation with an octreotide scan, an endoscopic ultrasound and a secretin stimulation test confirmed the diagnosis...
April 26, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28429092/natural-history-of-men1-gep-net-single-center-experience-after-a-long-follow-up
#17
Francesco Giudici, Tiziana Cavalli, Francesca Giusti, Giorgio Gronchi, Giacomo Batignani, Francesco Tonelli, Maria Luisa Brandi
BACKGROUND: The multiple endocrine neoplasia type 1 syndrome (MEN1) natural history is poorly evaluated, and few single-institution experiences about hereditary gastroenteropancreatic neuroendocrine tumors (GEP-NET) are reported. Our purpose is to analyze the role of GEP-NET in MEN1-related death, as well as the behavior of these lesions during follow-up. METHODS: The study population consists of 77 patients diagnosed with MEN1 GEP-NET, regularly followed up since 1990...
September 2017: World Journal of Surgery
https://www.readbyqxmd.com/read/28422824/pancreatic-carcinoids-serotonin-producing-pancreatic-neuroendocrine-neoplasms-report-of-5-cases-and-review-of-the-literature
#18
REVIEW
Nikolaos Tsoukalas, Eleftherios Chatzellis, Dimitra Rontogianni, Krystallenia I Alexandraki, Georgios Boutzios, Anna Angelousi, Gregory Kaltsas
INTRODUCTION: Pancreatic neuroendocrine neoplasms (pNENs) are relatively rare tumors representing 1% to 2% of all pancreatic neoplasms. These tumors can secrete a variety of biologically active substances giving rise to distinct clinical symptoms or can be clinically nonfunctioning. Apart from insulinomas and gastrinomas, which constitute the majority of functioning pNENs, some tumors may secrete serotonin presenting with the features of the carcinoid syndrome. These so-called pancreatic carcinoids are considered relatively rare tumors and are associated with increased urinary levels of 5-hydroxyindoleacetic acid (5-HIAA)...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28321346/a-perplexing-case-of-abdominal-pain-that-led-to-the-diagnosis-of-zollinger-ellison-syndrome
#19
Adrienne Lenhart, Mona Hassan, Alireza Meighani, Omar Sadiq, Yousuf Siddiqui
Zollinger-Ellison syndrome (ZES) is a rare clinical disorder, characterized by hypersecretion of gastric acid and multiple ulcers distal to the duodenal bulb. This occurs via the release of gastrin by neuroendocrine tumors known as gastrinomas. Patients with ZES present with nonspecific GI symptoms, which often leads to a delay in diagnosis. Our patient is a 55-year-old female with chronic abdominal pain, nausea, and diarrhea. She underwent EGD, EUS, MRCP, CT scans, and cholecystectomy, which did not reveal the cause of her symptoms...
2017: Case Reports in Gastrointestinal Medicine
https://www.readbyqxmd.com/read/28300688/duodenal-gastrinoma-discovered-on-evaluation-for-incidental-gastric-carcinoid
#20
Preeth Jayaram, Alex Shindel, James Buxbaum
No abstract text is available yet for this article.
March 11, 2017: Clinical Gastroenterology and Hepatology
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