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https://www.readbyqxmd.com/read/28329615/necrolytic-migratory-erythema-associated-with-fatty-liver-disease-and-the-psuedoglucagonoma-syndrome
#1
Collin Fuller, Jaimie Glick, Shelly Rivas, Katy Burris
We report a 48-year-old woman with a past medical history of psoriasis, nonalcoholic steatohepatitis (NASH), and type II diabetes mellitus, who presented to the emergency department with a 1 week history of erosive annular plaques with associated atrophy and telangiectasias on her legs bilaterally, thighs and buttock, histopathologically consistent with necrolytic migratory erythema. Although classically associated with a pancreatic glucagonoma, this patient experienced this figurate erythema in the setting of fatty liver disease with no glucagonoma...
September 15, 2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/28273025/glucagonoma-associated-rash
#2
Luke Mountjoy, Daniel Kollmorgen
A 65-year-old man with type 2 diabetes and deterioration of glycemic control presented with a 2-to-3-month history of weight loss (7 to 8 kg), early satiety, and mild flank pain. Before the occurrence of these symptoms, a rash had developed over the course of 3 to 4 weeks. The rash involved the..
March 9, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28218201/b-mode-and-contrast-enhancement-characteristics-of-small-nonincidental-neuroendocrine-pancreatic-tumors
#3
Barbara Braden, Christian Jenssen, Mirko D'Onofrio, Michael Hocke, Uwe Will, Kathleen Möller, Andre Ignee, Yi Dong, Xin-Wu Cui, Adrian Sãftoiu, Christoph F Dietrich
BACKGROUND AND OBJECTIVES: Imaging of the pancreas for detection of neuroendocrine tumors is indicated as surveillance in multiple endocrine neoplasia type 1 (MEN1) or if typical clinical symptoms combined with hormone production raise the suspicion of a neuroendocrine tumor. Endoscopic ultrasound (EUS) is considered the best imaging modality to detect small pancreatic tumors. However, little is known about how small pancreatic neuroendocrine tumors (pNETs) present on EUS. PATIENTS AND METHODS: In this multicenter study, we retrospectively analyzed the endosonographic characteristics of small pNETs which had been detected due to typical biochemistry and clinical symptoms or during surveillance of MEN 1...
January 2017: Endoscopic Ultrasound
https://www.readbyqxmd.com/read/28188614/foxa2-a-novel-protein-partner-of-the-tumour-suppressor-menin-is-deregulated-in-mouse-and-human-men1-glucagonomas
#4
Rémy Bonnavion, Romain Teinturier, Samuele Gherardi, Emmanuelle Leteurtre, Run Yu, Martine Cordier-Bussat, Rui Du, François Pattou, Marie-Christine Vantyghem, Philippe Bertolino, Jieli Lu, Chang Xian Zhang
Foxa2, known as one of the pioneer factors, plays a crucial role in islet development and endocrine functions. Its expression and biological functions are regulated by various factors, including, in particular, insulin and glucagon. However, its expression and biological role in adult pancreatic α-cells remain elusive. In the current study, we showed that Foxa2 was overexpressed in islets from α-cell-specific Men1 mutant mice, at both the transcriptional level and the protein level. More importantly, immunostaining analyses showed its prominent nuclear accumulation, specifically in α-cells, at a very early stage after Men1 disruption...
February 11, 2017: Journal of Pathology
https://www.readbyqxmd.com/read/28108603/glucagon-and-amino-acids-are-linked-in-a-mutual-feedback-cycle-the-liver-%C3%AE-cell-axis
#5
Jens J Holst, Nicolai J Wewer Albrechtsen, Jens Pedersen, Filip K Knop
Glucagon is usually viewed as an important counterregulatory hormone in glucose metabolism, with actions opposing those of insulin. Evidence exists that shows glucagon is important for minute-to-minute regulation of postprandial hepatic glucose production, although conditions of glucagon excess or deficiency do not cause changes compatible with this view. In patients with glucagon-producing tumors (glucagonomas), the most conspicuous signs are skin lesions (necrolytic migratory erythema), while in subjects with inactivating mutations of the glucagon receptor, pancreatic swelling may be the first sign; neither condition is necessarily associated with disturbed glucose metabolism...
February 2017: Diabetes
https://www.readbyqxmd.com/read/27933176/pancreatic-%C3%AE-cell-hyperplasia-and-hyperglucagonemia-due-to-a-glucagon-receptor-splice-mutation
#6
Etienne Larger, Nicolai J Wewer Albrechtsen, Lars H Hansen, Richard W Gelling, Jacqueline Capeau, Carolyn F Deacon, Ole D Madsen, Fumiatsu Yakushiji, Pierre De Meyts, Jens J Holst, Erica Nishimura
Glucagon stimulates hepatic glucose production by activating specific glucagon receptors in the liver, which in turn increase hepatic glycogenolysis as well as gluconeogenesis and ureagenesis from amino acids. Conversely, glucagon secretion is regulated by concentrations of glucose and amino acids. Disruption of glucagon signaling in rodents results in grossly elevated circulating glucagon levels but no hypoglycemia. Here, we describe a patient carrying a homozygous G to A substitution in the invariant AG dinucleotide found in a 3' mRNA splice junction of the glucagon receptor gene...
2016: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/27863775/operation-for-insulinomas-in-multiple-endocrine-neoplasia-type-1-when-pancreatoduodenectomy-is-appropriate
#7
Francesco Tonelli, Francesco Giudici, Gabriella Nesi, Giacomo Batignani, Maria Luisa Brandi
BACKGROUND: Distal pancreatectomy is the most frequent operation for insulinomas complicating multiple endocrine neoplasia type 1 insulinoma, although there are conditions for which a different operative approach might be preferable. In this article, we report the operative experience of a referral center for multiple endocrine neoplasia type 1 insulinoma. METHODS: Twelve patients underwent operations between 1992 and 2015: 8 underwent a distal pancreatic resection, and 4 underwent a pancreatoduodenectomy...
March 2017: Surgery
https://www.readbyqxmd.com/read/27622478/necrolytic-migratory-erythema-and-pancreatic-glucagonoma
#8
Gerzaín Rodríguez, Elga Vargas, Claudia Abaúnza, Sergio Cáceres
Necrolytic migratory erythema is a rare paraneoplastic dermatosis that may be the first clinical manifestation of the glucagonoma syndrome, a disorder characterized by mucocutaneous rash, glucose intolerance, hypoaminoacidemia, hyperglucagonaemia and pancreatic glucagonoma. The clinical case of a 45-year-old woman is presented. She had been experiencing weight loss, polydipsia, polyphagia, postprandial emesis, excessive hair loss and abdominal pain for two months. Erythematous, scaly and migratory plaques with 20 days of evolution were found on her trunk, perineum, elbows, hands, feet, inframammary and antecubital folds...
June 3, 2016: Biomédica: Revista del Instituto Nacional de Salud
https://www.readbyqxmd.com/read/27567335/-necrolytic-migratory-erythema-a-diagnostic-clue-in-glucagonoma-syndrome
#9
Fátima Moreno-Suárez, Águeda Pulpillo-Ruiz, María Fontillón Alberdi
No abstract text is available yet for this article.
August 24, 2016: Medicina Clínica
https://www.readbyqxmd.com/read/27450270/prognostic-factors-in-resected-pancreatic-neuroendocrine-tumours-experience-in-95-patients
#10
Francisco Sánchez-Bueno, José Manuel Rodríguez González, Gloria Torres Salmerón, Antonio Bernabé Peñalver, María Balsalobre Salmeron, Jesús de la Peña Moral, Matilde Fuster Quiñonero, Pascual Parrilla Paricio
INTRODUCTION: The aim of this study was to analyze prognostic factors for survival and recurrence in patients with resected pancreatic neuroendocrine tumors (PNT). METHODS: Medical records of 95 patients with resected PNT were retrospectively reviewed. The variables studied were: age, sex, form of presentation (sporadic/familial tumors), functionality, type of tumor, localization, type of surgery, tumor size, multifocal tumors and recurrent rate. The new WHO classification (2010) was used...
October 2016: Cirugía Española
https://www.readbyqxmd.com/read/27422767/glucagonoma-syndrome-a-review-and-update-on-treatment
#11
REVIEW
A M John, R A Schwartz
Glucagonoma syndrome is defined by the presence of an alpha-cell secreting tumour of the pancreas, elevated levels of glucagon, and a characteristic rash called necrolytic migratory erythema (NME). NME is usually a specific and often initial finding of glucagonoma syndrome, but it may occur in other settings unassociated with an alpha-cell pancreatic tumour (pseudoglucagonoma syndrome). Glucagonoma syndrome must be distinguished from pseudoglucagonoma syndrome. Prompt recognition of NME and subsequent workup for a glucagonoma can allow for an earlier diagnosis and enhance the chances of a favourable outcome...
December 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/27390586/over-expression-of-slc30a8-znt8-selectively-in-the-mouse-%C3%AE-cell-impairs-glucagon-release-and-responses-to-hypoglycemia
#12
Antonia Solomou, Erwann Philippe, Pauline Chabosseau, Stephanie Migrenne-Li, Julien Gaitan, Jochen Lang, Christophe Magnan, Guy A Rutter
BACKGROUND: The human SLC30A8 gene encodes the secretory granule-localised zinc transporter ZnT8 whose expression is chiefly restricted to the endocrine pancreas. Single nucleotide polymorphisms (SNPs) in the human SLC30A8 gene have been associated, through genome-wide studies, with altered type 2 diabetes risk. In addition to a role in the control of insulin release, recent studies involving targeted gene ablation from the pancreatic α cell (Solomou et al., J Biol Chem 290(35):21432-42) have also implicated ZnT8 in the control of glucagon release...
2016: Nutrition & Metabolism
https://www.readbyqxmd.com/read/27191542/the-uncovering-and-characterization-of-a-cckoma-syndrome-in-enteropancreatic-neuroendocrine-tumor-patients
#13
Jens F Rehfeld, Birgitte Federspiel, Mikkel Agersnap, Ulrich Knigge, Linda Bardram
OBJECTIVE: Neuroendocrine tumors in the pancreas and the gastrointestinal tract may secrete hormones which cause specific syndromes. Well-known examples are gastrinomas, glucagonomas, and insulinomas. Cholecystokinin-producing tumors (CCKomas) have been induced experimentally in rats, but a CCKoma syndrome in man has remained unknown until now. MATERIAL AND METHODS: Using a panel of immunoassays for CCK peptides and proCCK as well as for chromogranin A, we have examined plasma samples from 284 fasting patients with gastroenteropancreatic neuroendocrine tumors...
October 2016: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/26981306/glucagonoma-and-glucagonoma-syndrome-a-case-report-with-review-of-recent-advances-in-management
#14
Ashraf Al-Faouri, Khaled Ajarma, Samer Alghazawi, Sura Al-Rawabdeh, Adnan Zayadeen
The rarity of glucagonoma imposes a challenge with most patients being diagnosed after a long period of treatment for their skin rash (months-years). Awareness of physicians and dermatologists of the characteristic necrolytic migratory erythema often leads to early diagnosis. Early diagnosis of glucagonoma even in the presence of resectable liver metastases may allow curative resection. Herein, we present a typical case of glucagonoma treated at our center and review the literature pertinent to its management...
2016: Case Reports in Surgery
https://www.readbyqxmd.com/read/26971840/a-short-history-of-neuroendocrine-tumours-and-their-peptide-hormones
#15
REVIEW
Wouter W de Herder, Jens F Rehfeld, Mark Kidd, Irvin M Modlin
The discovery of neuroendocrine tumours of the gastrointestinal tract and pancreas started in 1870, when Rudolf Heidenhain discovered the neuroendocrine cells, which can lead to the development of these tumours. Siegfried Oberndorfer was the first to introduce the term carcinoid in 1907. The pancreatic islet cells were first described in 1869 by Paul Langerhans. In 1924, Seale Harris was the first to describe endogenous hyperinsulinism/insulinoma. In 1942 William Becker and colleagues were the first to describe the glucagonoma syndrome...
January 2016: Best Practice & Research. Clinical Endocrinology & Metabolism
https://www.readbyqxmd.com/read/26773171/do-glucagonomas-always-produce-glucagon
#16
REVIEW
Nicolai Jacob Wewer Albrechtsen, Benjamin G Challis, Ivan Damjanov, Jens Juul Holst
Pancreatic islet α-cell tumours that overexpress proglucagon are typically associated with the glucagonoma syndrome, a rare disease entity characterised by necrolytic migratory erythema, impaired glucose tolerance, thromboembolic complications and psychiatric disturbances. Paraneoplastic phenomena associated with enteric overexpression of proglucagon-derived peptides are less well recognized and include gastrointestinal dysfunction and hyperinsulinaemic hypoglycaemia. The diverse clinical manifestations associated with glucagon-expressing tumours can be explained, in part, by the repertoire of tumorally secreted peptides liberated through differential post-translational processing of tumour-derived proglucagon...
February 1, 2016: Bosnian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/26693280/heterogeneity-of-glucagonomas-due-to-differential-processing-of-proglucagon-derived-peptides
#17
Benjamin G Challis, Nicolai J Wewer Albrechtsen, Vishakha Bansiya, Keith Burling, Peter Barker, Bolette Hartmann, Fiona Gribble, Stephen O'Rahilly, Jens J Holst, Helen L Simpson
UNLABELLED: Pancreatic neuroendocrine tumours (pNETs) secreting proglucagon are associated with phenotypic heterogeneity. Here, we describe two patients with pNETs and varied clinical phenotypes due to differential processing and secretion of proglucagon-derived peptides (PGDPs). Case 1, a 57-year-old woman presented with necrolytic migratory erythema, anorexia, constipation and hyperinsulinaemic hypoglycaemia. She was found to have a grade 1 pNET, small bowel mucosal thickening and hyperglucagonaemia...
2015: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/26684862/different-hormonal-expression-patterns-between-primary-pancreatic-neuroendocrine-tumors-and-metastatic-sites
#18
Hideyo Kimura, Takao Ohtsuka, Takaaki Fujimoto, Kenjiro Date, Taketo Matsunaga, Ana Ines Cases, Atsushi Abe, Yusuke Mizuuchi, Yoshihiro Miyasaka, Tetsuhide Ito, Yoshinao Oda, Masafumi Nakamura, Masao Tanaka
OBJECTIVES: Pancreatic neuroendocrine tumors (PNETs) are known to have heterogeneity in terms of their ability to produce multiple hormones. The aim of this study was to evaluate the heterogeneity of PNETs from the viewpoint of hormonal expression. METHODS: The expressions of 4 representative hormones, gastrin, insulin, glucagon, and somatostatin, in both primary and metastatic lesions, were analyzed by immunohistochemical staining in 20 patients with metastatic PNETs (6 gastrinomas, 1 insulinoma, 1 glucagonoma, and 12 nonfunctioning PNETs [NF-PNETs])...
August 2016: Pancreas
https://www.readbyqxmd.com/read/26679842/delayed-diagnosis-of-glucagonoma-syndrome-a-case-report
#19
Jia Huo, Ping Liu, Xing Chen, Jiawen Wu, Jingang An, Jianwen Ren
No abstract text is available yet for this article.
November 2016: International Journal of Dermatology
https://www.readbyqxmd.com/read/26657531/metastatic-vipoma-presenting-as-an-ovarian-mass
#20
Jaron Mark, Stephen Bush, Evan Glazer, Jonathan Strosberg, Ozlen Saglam, Sachin M Apte
INTRODUCTION: Pancreatic VIPomas are exceedingly rare, with an annual incidence of less than 1 per million. Most VIPomas are metastatic at diagnosis, with the liver being the most common site of spread. PRESENTATION OF CASE: We describe a highly unusual case of a metastatic pancreatic VIPoma to an ovary in a 54 year-old patient. She was ten years out from her initial diagnosis when routine CT scan showed an enlarging left adnexal mass. After having both ovaries removed laparoscopically the final pathology was consistent with her pancreatic primary...
2015: International Journal of Surgery Case Reports
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