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https://www.readbyqxmd.com/read/27863775/operation-for-insulinomas-in-multiple-endocrine-neoplasia-type-1-when-pancreatoduodenectomy-is-appropriate
#1
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...
November 15, 2016: Surgery
https://www.readbyqxmd.com/read/27622478/necrolytic-migratory-erythema-and-pancreatic-glucagonoma
#2
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...
2016: Biomédica: Revista del Instituto Nacional de Salud
https://www.readbyqxmd.com/read/27567335/-necrolytic-migratory-erythema-a-diagnostic-clue-in-glucagonoma-syndrome
#3
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
#4
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
#5
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...
July 16, 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
#6
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
#7
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
#8
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
#9
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
#10
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...
2016: Bosnian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/26693280/heterogeneity-of-glucagonomas-due-to-differential-processing-of-proglucagon-derived-peptides
#11
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
#12
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
#13
Jia Huo, Ping Liu, Xing Chen, Jiawen Wu, Jingang An, Jianwen Ren
No abstract text is available yet for this article.
December 17, 2015: International Journal of Dermatology
https://www.readbyqxmd.com/read/26657531/metastatic-vipoma-presenting-as-an-ovarian-mass
#14
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
https://www.readbyqxmd.com/read/26648573/iatrogenic-necrolytic-migratory-erythema-in-an-infant-with-congenital-hyperinsulinism
#15
Carrie C Coughlin, Sani M Roy, Lisa M Arkin, N Scott Adzick, Albert C Yan, Diva D De León, Adam I Rubin
Necrolytic migratory erythema (NME) is a rare cutaneous finding characterized by painful, pruritic, scaly red patches and plaques, bullae, and superficial erosions. Typically NME is a paraneoplastic phenomenon associated with glucagonoma. We report the exceptional case of an infant who developed iatrogenic NME arising secondary to glucagon therapy for congenital hyperinsulinism.
March 2016: Pediatric Dermatology
https://www.readbyqxmd.com/read/26622635/glucagonoma-syndrome-a-case-report
#16
Jishu Wei, Shibo Lin, Cong Wang, Junli Wu, Zhuyin Qian, Cuncai Dai, Kuirong Jiang, Y I Miao
Necrolytic migratory erythema (NME), diabetes mellitus and glucagon-secreting tumors form the hallmarks of glucagonoma syndrome, and represent the major clinical manifestations of glucagonoma. NME is usually presented as the initial complaint of patients. Due to the rare incidence of glucagonoma, its diagnosis is often delayed, which leads to its progression. Here, we report a case of NME with a typical skin rash, which was misdiagnosed and treated with corticosteroids for two years. Removal of the tumor in the pancreatic body led to the rapid relief of the symptoms...
August 2015: Oncology Letters
https://www.readbyqxmd.com/read/26585841/pediatric-necrolytic-migratory-erythema-as-a-presenting-sign-of-glucagonoma-syndrome
#17
A J Luber, L S Ackerman, K S Culpepper, C M Buschmann, L J Koep
Glucagonoma syndrome is an extremely rare pancreatic neuroendocrine tumor often associated with necrolytic migratory erythema. While glucagonomas are neoplasms of adulthood, we report the first case in a pediatric patient. We present the case of a 15-year-old female with a 4-year history of a rash, consistent with necrolytic migratory erythema, found to have a localized glucagonoma. Immediately following resection of the tumor, there was complete resolution of her rash and systemic symptoms. Detection of the cutaneous rash of necrolytic migratory erythema can aid in the early diagnosis of a glucagonoma as well as the prevention of metastatic disease...
November 20, 2015: British Journal of Dermatology
https://www.readbyqxmd.com/read/26493062/proposed-radiation-induced-hormone-crisis-in-a-patient-with-glucagonoma
#18
Cory Allen Gaiser, Natasha Dhawan
No abstract text is available yet for this article.
October 22, 2015: Journal of Gastrointestinal Cancer
https://www.readbyqxmd.com/read/26303702/biochemical-testing-in-patients-with-neuroendocrine-tumors
#19
REVIEW
Dan Granberg
Neuroendocrine tumors are usually slow-growing tumors. Many of these are capable of secreting peptide hormones or biogenic amines that may lead to endocrine syndromes. Nonfunctioning tumors can either secrete no hormones at all, or secrete hormones not giving rise to endocrine symptoms, such as chromogranin A, chromogranin B or pancreatic polypeptide. Chromogranin A is produced by the majority of endocrine tumors, both functioning and nonfunctioning, and is the best available marker for diagnosis, follow-up and treatment monitoring of patients with differentiated neuroendocrine tumors...
2015: Frontiers of Hormone Research
https://www.readbyqxmd.com/read/26248239/glucagonoma-syndrome-associated-with-necrolytic-migratory-erythema
#20
Florentino de Araújo Cardoso Filho, Roney Gonçalves Fechine Feitosa, Carolina Oliveira Costa Fechine, Carlos Márcio Melo de Matos, Amanda Linhares Cardoso, Daniel Linhares Cardoso
INTRODUCTION: glucagonoma is a pancreatic neuroendocrine tumor derived from alpha-cells of the islets of Langerhans. It is marked by tumoral autonomous production of glucagon and characterized, among other symptoms, by necrolytic migratory erythema, an erythematous circinate lesion with areas of necrosis and sloughing. This is a rare disease with worldwide incidence estimated at 1 case per 20 million people. CASE REPORT: we report a case of glucagonoma associated necrolytic migratory erythema in a male patient, 56 years, with signs of skin lesions mainly on his legs and groin, hyperglycemia and weight loss...
May 2015: Revista da Associação Médica Brasileira
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