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Endocrinol Diabetes Metab Case Rep 2019 Mar 5;2019. Epub 2019 Mar 5.

Wolfson Diabetes & Endocrine Clinic, Cambridge University Hospitals NHS Foundation Trust.

A 67-year-old woman presented with a generalised rash associated with weight loss and resting tachycardia. She had a recent diagnosis of diabetes mellitus. Biochemical evaluation revealed elevated levels of circulating glucagon and chromogranin B. Read More

Acta Dermatovenerol Croat 2018 Dec;26(4):329-332

Professor Marija V'lckova-Laskoska, MD, PhD, Department of Dermatology , University Hospitals and Clinics, University of Sts. Cyril and Methodius Vodnjanska 17, 1000 Skopje, Macedonia;

Necrolytic migratory erythema is considered an obligatory cutaneous paraneoplastic sign associated with glucagonoma. Glucagonoma syndrome is defined by the presence of an alpha-cell secreting tumor of the pancreas, elevated levels of glucagon, and a characteristic rash called necrolytic migratory erythema. Although necrolytic migratory erythema is a specific finding in glucagonoma syndrome, it may occur in other settings, unassociated with an alpha-cell pancreatic tumor (pseudoglucagonoma syndrome). Read More

J Pancreat Cancer 2018 1;4(1):11-16. Epub 2018 May 1.

Pancreas Center, The First Affiliated Hospital of Nanjing Medical University, Nanjing, China.

Glucagonoma is an extremely rare neuroendocrine tumor arising from pancreatic islet cells. Although patients with glucagonoma manifest multiple typical symptoms, early diagnosis remains difficult due to the scarcity of this disease. In this study, we retrospectively screened the database of the pancreas center of Nanjing Medical University. Read More

J Clin Transl Endocrinol 2019 Mar 20;15:45-53. Epub 2018 Dec 20.

Internal Medicine Department, Hospital General Juan Cardona, c/ Pardo Bazán s/n, 15406 Ferrol, Spain.

Diabetes is a common metabolic disorder that involves glucose, amino acids, and fatty acids. Either insulin deficiency or insulin resistance may cause diabetes. Insulin deficiency causes type 1 diabetes and diabetes associated with total pancreatectomy. Read More

Br J Dermatol 2019 Jan;180(1):e1

Department of Dermatology, Hospital Universitario, 12 de Octubre, Avenida de Córdoba, Madrid, 28041, Spain.

J Clin Endocrinol Metab 2019 Apr;104(4):1336-1344

Department of Internal Medicine, Sector of Endocrinology, Erasmus Medical Center and Erasmus MC Cancer Institute, Rotterdam, Netherlands.

Purpose: Peptide receptor radionuclide therapy (PRRT) with the radiolabeled somatostatin analogue [Lutetium-177-DOTA0-Tyr3]octreotate (177Lu-DOTATATE) is widely applied for inoperable metastatic small intestinal and nonfunctioning pancreatic neuroendocrine tumors (pNETs). The aim of this study is to describe the safety and efficacy of the treatment of functioning pNETs.

Methods: Patients were treated with up to four cycles of 177Lu-DOTATATE with an intended dose of 7. Read More

J Surg Oncol 2019 Jan 27;119(1):5-11. Epub 2018 Nov 27.

Division of Surgical Oncology, Department of Surgery, Winship Cancer Institute, Emory University, Atlanta, Georgia.

Background: The goals of resection of functional neuroendocrine tumors (NETs) are two-fold: Oncological benefit and symptom control. The interaction between the two is not well understood.

Methods: All patients with functional NETs of the pancreas, duodenum, and ampulla who underwent curative-intent resection between 2000 and 2016 were identified. Read More

Am J Dermatopathol 2019 Mar;41(3):e29-e32

Department of Dermatology, Venerology and Allergology, University Medical Center, Ruprecht-Karls-University Heidelberg, Heidelberg, Germany.

Necrolytic migratory erythema (NEM) is associated with glucagonoma, an endocrine malignancy of the pancreas. It is a rare and a likely underrecognized paraneoplastic dermatitis. A 38-year-old woman presented to our clinic with a 3-year history of reocurring pruritic skin rashes with increasing intensity. Read More

Rapid Commun Mass Spectrom 2018 Aug;32(16):1414-1424

Institute of Metabolic Science, Metabolic Research Laboratories, Addenbrooke's Hospital, Hills Road, Cambridge, CB2 0QQ, UK.

Rationale: Diagnosis of pancreatic neuroendocrine tumours requires the study of patient plasma with multiple immunoassays, using multiple aliquots of plasma. The application of mass spectrometry based techniques could reduce the cost and amount of plasma required for diagnosis.

Methods: Plasma samples from two patients with pancreatic neuroendocrine tumours were extracted using an established acetonitrile-based plasma peptide enrichment strategy. Read More

J Clin Endocrinol Metab 2018 07;103(7):2417-2423

Division of Diabetes, Endocrinology, and Metabolism, Chiba University Hospital, Chiba, Japan.

Context: Necrolytic migratory erythema (NME) occurs in approximately 70% of patients with glucagonoma syndrome. Excessive stimulation of metabolic pathways by hyperglucagonemia, which leads to hypoaminoacidemia, contributes to NME pathogenesis. However, the molecular pathogenesis of glucagonoma and relationships between metabolic abnormalities and clinical symptoms remain unclear. Read More

Chirurg 2018 Jun;89(6):422-427

Sektionsleitung Endokrine Chirurgie, Klinik für Viszeral‑, Thorax- und Gefäßchirurgie, Universitätsklinikum Marburg, Baldingerstraße, 35043, Marburg, Deutschland.

Small (<2 cm) sporadic neuroendocrine pancreatic neoplasms (pNENs) are a heterogeneous group of neoplasms, which can be separated into functionally active or non-functional tumors. Functionally active pNENs release various hormones, such as insulin, gastrin, glucagon and vasoactive intestinal hormones and therefore lead to severe symptoms independent of their size. The main symptoms include hypoglycemia, coma (insulinoma), diarrhea (gastrinoma, VIPoma), intestinal ulcers (gastrinoma) and hyperglycemia (glucagonoma). Read More

Gland Surg 2018 Feb;7(1):20-27

Department of Medical Sciences, Endocrine Oncology, Uppsala University Hospital, Uppsala, Sweden.

Pancreatic neuroendocrine tumors (pNETs) constitute a heterogenous group of malignancies with varying clinical presentation, tumor biology and prognosis. The incidence of pNETs has steadily increased during the last decades with an estimated incidence 2012 of 4.8/100,000. Read More

Gastroenterology 2018 Sep 13;155(3):e7-e8. Epub 2018 Mar 13.

Hepato-Pancreato-Biliary Surgery and Liver transplantation, Pôle des Pathologies Digestives, Hépatiques et de la Transplantation, Hôpital de Hautepierre, Strasbourg, France.

Intern Med 2018 Aug 9;57(15):2283-2284. Epub 2018 Mar 9.

Department of General Internal Medicine 4, Kawasaki Medical School, Japan.

Int J Dermatol 2018 Jun 16;57(6):642-645. Epub 2018 Feb 16.

Division of Dermatology, Department of Internal Medicine, The Ohio State University Wexner Medical Center, Columbus, OH, USA.

Necrolytic migratory erythema (NME) is a rare skin disorder that is a cutaneous manifestation of the glucagonoma syndrome. It presents with annular eruptions of migrating erythematous papules and plaques with superficial epidermal necrosis, central flaccid bullae, and crusted erosions located primarily in the intertriginous areas. Treatment with the long-acting somatostatin analog Octreotide is a potential therapy to help ameliorate skin symptoms. Read More

Clin Imaging 2018 Sep - Oct;51:59-64. Epub 2018 Feb 8.

Department of Radiology, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA. Electronic address:

Background: Gastroenteropancreatic neuroendocrine tumors (NETs) are rare entities arising from neuroendocrine cells in the gastroenteric tract and pancreas. The purpose of this article is to present four cases of gastroenteropancreatic NETs that featured a challenging diagnosis.

Case Presentation: We report a case series of four NETs, each with different features. Read More

Oncol Lett 2018 Mar 28;15(3):2749-2755. Epub 2017 Dec 28.

Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, P.R. China.

Glucagonoma is an extremely rare pancreatic α-islet cell tumor and is often accompanied by certain clinical symptoms including necrotizing migratory erythema (NME), diabetes, weight loss and anemia. The objectives of the current review were to discern the clinical features, diagnosis, treatment and prognosis of glucagonoma by evaluating 623 reported cases. A 1998 study reviewed 407 cases and 216 cases were reported in studies published after 1998. Read More

Clin Nucl Med 2018 Apr;43(4):252-255

Glucagonoma is an extremely rare, glucagon-secreting neuroendocrine tumor of the pancreas. Only sparse data are available about the characteristics of this tumor in somatostatin receptor imaging and only for the situation of initial diagnosis. We present a series of 3 glucagonoma patients who underwent at least 1 Ga-DOTATATE PET/CT scan. Read More

J Eur Acad Dermatol Venereol 2018 Feb 8. Epub 2018 Feb 8.

Department of Dermatology, Hietzing Municipal Hospital, Vienna, AT, Austria.

Medicine (Baltimore) 2017 Dec;96(50):e9252

Department of Radiology, Memorial Sloan Kettering Cancer Center, NY.

Rationale: Glucagonoma is an uncommon disease but it has been associated with a pattern of symptoms defined as glucagonoma syndrome. These symptoms, if promptly recognized, could help to speed up the diagnosing process.

Patient Concerns: We report a case of a 68-year-old woman with a pancreatic glucagonoma. Read More

Gastroenterol Hepatol 2018 Oct 23;41(8):500-502. Epub 2017 Oct 23.

Servicio de Cirugía General y del Aparato Digestivo, Hospital General Universitario Santa Lucía, Cartagena, Murcia, España.

Medicine (Baltimore) 2017 Oct;96(43):e8448

aDiabetic Foot Center, Department of Endocrinology and Metabolism bDepartment of Pathology cDepartment of Pancreatic Surgery dDepartment of Radiology, West China Hospital, Sichuan University, Chengdu, Sichuan, People's Republic of China.

Rationale: Glucagonoma and pancreatic serous oligocystic adenoma (SOA) are rare neuroendocrine and exocrine tumors of the pancreas, respectively. The coexistence of glucagonoma syndrome (GS) and SOA is a rare clinical condition and has not yet been reported. Additionally, necrolytic migratory erythema (NME), a hallmark clinical sign of GS, is often misdiagnosed as other skin lesions by clinicians due to their lack of related knowledge, which delays diagnosis of GS and thus exacerbates the prognosis. Read More

ANZ J Surg 2017 Oct 5. Epub 2017 Oct 5.

Department of Surgery, Prince of Wales Hospital, Sydney, New South Wales, Australia.

J Cutan Med Surg 2017 Nov/Dec;21(6):559-561. Epub 2017 Jun 29.

1 Department of Dermatology, Saint-Luc Hospital, Montreal University, Montréal, QC, Canada.

Background: Necrolytic migratory erythema (NME) is most commonly a paraneoplastic condition. It is the dermatologic manifestation classically associated with glucagonoma pancreatic neuroendocrine tumour. Glucagonoma syndrome has been defined by the constellation of secreting tumour associated with overproduction by the α-cells in the pancreatic islets of Langerhans, abnormally elevated blood level of glucagon, and skin findings of NME. Read More

Am J Med Sci 2017 Jun 9;353(6):610-612. Epub 2016 Dec 9.

Department of Medicine, Division of Medical Oncology, Orlando Veterans Affairs Medical Center, Orlando, Florida. Electronic address:

Rev Med Suisse 2017 May;13(565):1158-1162

Service d'endocrinologie, diabétologie, hypertension et nutrition, HUG, 1211 Genève 14.

An endocrine disease can be associated with glucose intolerance or diabetes mellitus, and the latter can falsely be considered as type 2 diabetes. Glycemic imbalance can be a direct or indirect consequence of excessive hormone production. Endocrine diseases such as acromegaly, Cushing's syndrome and pheochromocytoma can increase glucose production and cause insulin resistance. Read More

Case Rep Dermatol 2017 Jan-Apr;9(1):69-73. Epub 2017 Mar 17.

Division of Dermatology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.

Necrolytic acral erythema (NAE) is a distinctive skin disorder. The exact cause and pathogenesis is still unclear. Most studies report an association of NAE with hepatitis C virus (HCV) infection. Read More

Intern Med 2017 1;56(11):1375-1381. Epub 2017 Jun 1.

Department of Diabetes and Endocrinology, Osaka Red Cross Hospital, Japan.

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. Read More

Dermatol Ther 2017 Jul 16;30(4). Epub 2017 May 16.

Unidad de Gestión Clínica de Dermatología, Hospital Universitario Puerta del Mar, Cádiz, Spain.

Arch Med Sci 2017 Apr 1;13(3):515-524. Epub 2016 Jun 1.

Department of Endocrinology, Poznan University of Medical Sciences, Poznan, Poland.

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. Read More

N Engl J Med 2017 Mar;376(10):e18

Iowa Methodist Medical Center, Des Moines, IA

Endosc Ultrasound 2017 Jan-Feb;6(1):49-54

Medical Department, Caritas-Krankenhaus, Uhlandstr. 7 D-97980 Bad Mergentheim, Germany; Sino-German Research Center of Ultrasound in Medicine, The first Affiliated Hospital of Zhengzhou University, Zhengzhou, China.

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. Read More

J Pathol 2017 05 27;242(1):90-101. Epub 2017 Mar 27.

INSERM U1052, Lyon, France.

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. Read More

Transl Gastroenterol Hepatol 2016 19;1:70. Epub 2016 Sep 19.

Department of Pancreatic Surgery, Zhongshan Hospital, Fudan University, Shanghai 200032, China.

A 60-year-old male patient was admitted due to protracted systemic pruritus and erythema for 3 years without an obvious dermatologic cause, which exacerbated in the past 3 months, along with weight loss and diarrhoea. He had significant fasting hyperglycemia and dramatically elevated serum glucagon level in biochemical examination. Elevated chromogranin A (CgA) and neuron-specific enolase (NSE) levels were also manifested, whereas carbohydrate antigen 19-9 (CA-199) and carcinoembryonic antigen (CEA) were unremarkable. Read More

Diabetes 2017 02;66(2):235-240

Novo Nordisk Foundation Center for Basic Metabolic Research, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark.

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. Read More

Endocrinol Diabetes Metab Case Rep 2016 21;2016. Epub 2016 Nov 21.

Metabolic Disease Research , Novo Nordisk A/S, Måløv , Denmark.

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. Read More

Abdom Radiol (NY) 2017 04;42(4):1113-1131

Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, 1400 Pressler St., Houston, TX, 77030, USA.

Gastrointestinal secretory tumors, or gastroenteropancreatic neuroendocrine tumors, encompass a wide array of endocrine cell tumors. The significance of these tumors lies in their ability to alter physiology through hormone production as we well as in their malignant potential. Functioning tumors may present earlier due to symptomatology; conversely, non-functioning tumors are often diagnosed late as they reach large sizes, causing symptoms secondary to local mass effect. Read More

Surgery 2017 03 15;161(3):727-734. Epub 2016 Nov 15.

Department of Surgery and Translational Medicine, University of Florence, Florence, Italy.

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. Read More

Oncotarget 2016 11;7(46):74415-74426

Center for Neuropathology, Ludwig-Maximilians University, Munich, Germany.

Amplification or overexpression of MYCN is involved in development and maintenance of multiple malignancies. A subset of these tumors originates from neural precursors, including the most aggressive forms of the childhood tumors, neuroblastoma and medulloblastoma. In order to model the spectrum of MYCN-driven neoplasms in mice, we transgenically overexpressed MYCN under the control of the human GFAP-promoter that, among other targets, drives expression in neural progenitor cells. Read More

Biomark Med 2016 Nov 9;10(11):1141-1151. Epub 2016 Sep 9.

Department of Biomedical Sciences, Faculty of Health & Medical Sciences, University of Copenhagen, Denmark.

The proglucagon-derived peptide hormone, glucagon, comprises 29 amino acids. Its secretion from the pancreatic α cells is regulated by several factors. Glucagon increases blood glucose levels through gluconeogenesis and glycogenolysis. Read More

Endocr Pract 2017 Jan 15;23(1):116. Epub 2016 Sep 15.

From the 1Department of Endocrinology, Diabetes & Metabolism, Christian Medical College, Vellore, India.

Biomedica 2016 Jun 3;36(2):176-81. Epub 2016 Jun 3.

Grupo de Microbiología Molecular, Facultad de Medicina, Universidad de La Sabana, Chía, Colombia.

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. Read More

Med Clin (Barc) 2017 Apr 24;148(8):e45. Epub 2016 Aug 24.

Servicio de Anatomía Patológica, Hospitales Universitarios Virgen del Rocío, Sevilla, España.

Endocr Relat Cancer 2016 09 26;23(9):R423-36. Epub 2016 Jul 26.

Oxford Center for DiabetesEndocrinology and Metabolism, University of Oxford, Oxford, UK.

Although recent epidemiological evidence indicates that the prevalence of non-functioning gastroenteropancreatic (GEP) neuroendocrine tumours (NETs) is rising, a significant number of GEP-NETs still present with symptoms related to the secretion of biologically active substances leading to the development of distinct clinical syndromes. In the past, these syndromes were associated with substantial morbidity and mortality due to the lack of specific therapies; however, since the introduction of long-acting somatostatin analogues and medications such as proton pump inhibitors, their control has been greatly improved. As a result, nowadays, the main cause of morbidity and mortality in GEP-NETs is mostly directly related to tumour growth and the extent of metastatic disease. Read More

Cir Esp 2016 Oct 19;94(8):473-80. Epub 2016 Jul 19.

Servicio de Cirugía, Hospital Clínico Universitario Virgen de la Arrixaca, Murcia, España.

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. Read More

Endocrine 2017 Apr 15;56(1):196-204. Epub 2016 Jul 15.

Laboratory of Experimental Biochemistry & Molecular Biology, IRCCS Istituto Ortopedico Galeazzi, Milano, Italy.

Bone and energy metabolisms regulation depends on a two-way street aimed at regulating energy utilization. Mountain ultra-marathons are highly demanding aerobic performances that deeply affect the whole body homeostasis. In this study we aimed to investigate and characterize the metabolic profile (in terms of hormones involved in energy metabolism), the inflammatory adipokines, and the bone turnover; in particular the osteocalcin-mediated response has been compared in experienced mountain ultra-marathons runners versus control subjects. Read More

J Eur Acad Dermatol Venereol 2016 Dec 16;30(12):2016-2022. Epub 2016 Jul 16.

Dermatology and Pathology, Rutgers-New Jersey Medical School, Newark, NJ, USA.

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. Read More