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Postgrad Med J 2020 Jun 11. Epub 2020 Jun 11.

Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

AACE Clin Case Rep 2020 Jan-Feb;6(1):e46-e49. Epub 2020 Jan 3.

Objective: Glucagonoma is a rare neuroendocrine tumor of the pancreas. We present the case of a young female patient who presented with the major clinical manifestations of glucagonoma syndrome.

Methods: The major clinical manifestations of glucagonoma syndrome are described in a 44-year-old, female patient. Read More

J Diabetes Investig 2020 Jun 7. Epub 2020 Jun 7.

Department of Endocrinology, Division of Stress Adaptation and Protection, Research Institute of Environmental Medicine, Nagoya University, Nagoya, Japan.

Glucagon increases hepatic glucose production through stimulation of glycogenolysis and gluconeogenesis. Recently, accumulating data show that glucagon plays pivotally important roles in regulation of amino acid catabolism, the product of which serves as substrates for gluconeogenesis . Glucagon also promotes lipolysis and produce glycerol, which serves as a gluconeogenic substrate. Read More

J Biol Chem 2020 May 21. Epub 2020 May 21.

University of Toronto, Canada.

Type 2 diabetes (T2D) is a chronic metabolic disease characterized by pancreatic β-cell dysfunction and peripheral insulin resistance. Among individuals with T2D, approximately 30% exhibit hypomagnesemia. Hypomagnesemia has been linked to insulin resistance through reduced tyrosine kinase activity of the insulin receptor; however, its impact on pancreatic β-cell function is unknown. Read More

Australas J Dermatol 2020 May 8. Epub 2020 May 8.

Outpatients' Department of Dermatology, Local Health Centre Taranto, Taranto, Italy.

A case of pseudoglucagonoma syndrome, that is necrolytic migratory erythema, in a patient with no coexistent glucagonoma, is described. The patient was a 59-year-old man with waxing and waning dermatitis of the buttocks, characterised by arciform erythematous papulo-squamous lesions with micro-pustulation. Histopathology was characteristic for necrolytic migratory erythema, but no other underlying disease was detected. Read More

Clin Cosmet Investig Dermatol 2020 5;13:275-281. Epub 2020 Apr 5.

Department of Dermatology, Venereology and Leprosy, SN Medical College, Bagalkote 587102, Karnataka, India.

Necrolytic acral erythema (NAE) is now considered as a distinct clinical entity. It clinically presents as well demarcated hyperpigmented papules and plaques with thick adherent scales distributed symmetrically over dorsum of feet. It usually develops in patients with Hepatitis C virus (HCV) infection. Read More

Lancet 2020 03;395(10228):985

Section of Clinical and Molecular Dermatology, Medical Faculty Mannheim and European Center for Angioscience, Heidelberg University, Mannheim, Germany.

J Radiosurg SBRT 2020 ;6(4):321-324

Division of Medical Oncology, Odette Cancer Centre, Sunnybrook Health Sciences Centre, University of Toronto. 2075 Bayview Avenue, Toronto, ON, M4N 3M5, Canada.

Dysregulated hormonal production remains a challenge in the management of neuroendocrine neoplasms (NEN). We report 4 cases of patients with functional NEN treated with stereotactic body radiation therapy (SBRT) to either the primary/dominant metastatic site of disease or the end organ of hormonal release. No significant toxicities were observed during or after treatment. Read More

Surg Today 2020 Feb 3. Epub 2020 Feb 3.

Department of Visceral-, Thoracic- and Vascular Surgery, Philipps-University Marburg, Baldingerstrasse, 35043, Marburg, Germany.

Purpose: The present study aimed to compare robotic-assisted versus laparoscopic distal pancreatic resection and enucleation for potentially benign pancreatic neoplasms.

Methods: Patients were retrieved from a prospectively maintained database. Demographic data, tumor types, and the perioperative outcomes were retrospectively analyzed. Read More

Rev Med Interne 2020 Mar 24;41(3):210-213. Epub 2020 Jan 24.

Service de médecine interne, boulevard Robert Ballanger, 93600 Aulnay-sous-Bois, France.

Onco Targets Ther 2019 20;12:11339-11344. Epub 2019 Dec 20.

Department of Abdominal Oncology, Cancer Center of West China Hospital, Sichuan University, Chengdu 610041, Sichuan, People's Republic of China.

Glucagonoma, a rare neuroendocrine tumor of the pancreas, which is often misdiagnosed because of non-characteristic clinical manifestations. In addition, the treatment has not been well established for this disease so far. We here report a case of glucagonoma previous misdiagnosed as recurrent erythema. Read More

Pancreatology 2020 03 24;20(2):293-296. Epub 2019 Dec 24.

Hans Christian Andersen Children's Hospital, Odense University Hospital, Odense, Denmark; Clinical Research, Faculty of Health Sciences, University of Southern Denmark, Denmark; OPAC, Odense Pancreas Centre, Odense University Hospital, Odense, Denmark. Electronic address:

J Dermatol 2020 Feb 12;47(2):178-180. Epub 2019 Dec 12.

Department of Dermatology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.

Autoimmune progesterone dermatitis (APD) is a rare cutaneous disorder with cyclic skin eruptions during the luteal phase of the menstrual cycle. Patients can present with various clinical manifestations, including urticaria and angioedema, erythema multiforme, eczema, fixed drug eruption and centrifugal erythema annulare. In our case, however, the patient's skin lesions mimic necrotic migratory erythema (NME) which is most commonly associated with glucagonoma and rarely with liver disease, inflammatory bowel disease, malnutrition and other tumors. Read More

Autops Case Rep 2019 Oct-Dec;9(4):e2019129. Epub 2019 Nov 27.

University of Campinas, Department of Gastroenterology (Gastrocentro). Campinas, SP, Brazil.

Glucagonoma is a rare and slow-growing pancreatic tumor that usually manifests as glucagonoma syndrome. It is mainly characterized by a typical Dermatosis named necrolytic migratory erythema (NME), Diabetes and glucagon oversecretion. Deep vein thrombosis and Depression complete this set. Read More

Radiol Case Rep 2020 Jan 8;15(1):19-22. Epub 2019 Nov 8.

Department of Radiology, Medical Imaging Service, King Faisal Specialist Hospital & Research Center, MBC#28, P. O Box 3354, Riyadh 11211, Saudi Arabia.

A 35-year-old man presented with significant weight loss of 30 kg over the previous 6 months, with newly diagnosed diabetes. Routine laboratory tests were normal, except for markedly elevated blood glucose. Computed tomography (CT) of the abdomen revealed a large severely enhanced mass replacing most of the pancreas and liver metastatic nodules and multiple paraaortic lymph node metastases, F-fluorodeoxygluocse positron emission tomography/computed tomography (F-FDG PET/CT) was performed and revealed mild FDG uptake in the pancreatic mass, as well as mild uptake in the liver and lymph node metastases. Read More

J Community Hosp Intern Med Perspect 2019 1;9(5):425-429. Epub 2019 Nov 1.

Division of Medical Oncology, Department of Internal Medicine, University of Kansas Medical Center, Kansas City, KS, USA.

A 54-year-old Caucasian male with history of hypertension, hyperlipidemia, insulin-dependent diabetes mellitus, and chronic skin rash of 4 years presented to the emergency department with worsening rash and weight loss. Physical examination revealed diffuse erythematous rash, skin ulceration, bullae with associated paresthesia in the lower extremities, trunk, bilateral upper extremities, and palms and soles. A computed tomography (CT) scan with contrast showed a large, heterogenously enhancing pancreatic mass measuring 9. Read More

Medicine (Baltimore) 2019 Sep;98(38):e17037

Department of Hepatobiliary Surgery.

Rationale: Glucagonoma is a rare type of functional pancreatic neuroendocrine tumor that is characterized by distinctive clinical manifestations; among these, necrolytic migratory erythema represents the hallmark clinical sign of glucagonoma syndrome and is usually presented as the initial complaint of patients.

Patient Concerns: A 30-year-old male patient was admitted to our hospital with a complaint of diffuse erythematous ulcerating skin rash for more than 10 months. He also complained of hyperglycemia and a weight loss of 15 kg in those months. Read More

Medicine (Baltimore) 2019 Sep;98(37):e17158

Department of General Surgery, the Affiliated Hospital of Inner Mongolia Medical University, Hohhot, China.

Rationale: Glucagonoma is a rare neuroendocrine tumor of the pancreas. Glucagonoma syndrome is often misdiagnosed as other skin lesions by clinicians due to a typical clinical sign of necrolytic migratory erythema (NME) with severe erythematous rash.

Patient Concerns: A 48-year-old female patient was admitted to our department because she presented with unclear recurrent severe erythematous rash. Read More

Med Clin (Barc) 2019 Sep 9. Epub 2019 Sep 9.

Servicio de Dermatología, Hospital Clínic de Barcelona, Barcelona, España.

Clin Colon Rectal Surg 2019 Sep 6;32(5):372-376. Epub 2019 Sep 6.

Hospital de Clínicas José de San Martín, Buenos Aires, Argentina.

This article reviews the clinical features of systemic and autoimmune diseases affecting the perianal region and its surrounding integumentary structures. Read More

BMJ Case Rep 2019 Aug 10;12(8). Epub 2019 Aug 10.

Medical Oncology, The Christie NHS Foundation Trust, Manchester, UK.

A 70-year-old man reported progressive weight loss, fatigue and a generalised rash. The rash was consistent with necrolytic migratory erythema, further investigations were performed and the patient was diagnosed with a mass in the tail of the pancreas, in keeping with a localised glucagonoma. Somatostatin analogue therapy was started for symptom control, leading to complete resolution of the skin rash and an improvement in constitutional symptoms. Read More

Indian J Dermatol Venereol Leprol 2019 Aug 1. Epub 2019 Aug 1.

Department of Dermatology, The Second Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou, China.

Necrolytic migratory erythema is most commonly associated with glucagonoma syndrome. We report a rare case of glucagonoma syndrome with necrolytic migratory erythema presenting as pruritic papules and follicular pustules in a 57-year-old woman; showing eosinophilic infiltration on histology. However, the final diagnosis was confirmed by demonstrating neuroendocrine tumour on histopathological examination of the liver metastases. Read More

Endocr Pract 2020 02 6;26(2):253. Epub 2019 Jun 6.

From the Section of Dermatology and Venereology, Department of Medicine, University of Verona, Verona, Italy.

J Gastrointest Surg 2020 Mar 31;24(3):701-703. Epub 2019 May 31.

Department of Digestive surgery, Hôpital Nord, Université Aix-Marseille, France, Hôpital Nord, Chemin des Bourrely 13915, cedex 20, Marseille, France.

Front Endocrinol (Lausanne) 2019 3;10:271. Epub 2019 May 3.

Department of Biomedical and Biotechnological Sciences, Section of Medical Biochemistry, University of Catania, Catania, Italy.

PARP-14 (poly-ADP Ribose Polymerase-14), a member of the PARP family, belongs to the group of Bal proteins (B Aggressive Lymphoma). PARP-14 has recently appeared to be involved in the transduction pathway mediated by JNKs (c Jun N terminal Kinases), among which JNK2 promotes cancer cell survival. Several pharmacological PARP inhibitors are currently used as antitumor agents, even though they have also proved to be effective in many inflammatory diseases. Read More

World J Surg Oncol 2019 Apr 11;17(1):66. Epub 2019 Apr 11.

Department of Integrative Oncology, China-Japan Friendship Hospital, 2 Yinghuadong Road, Chaoyang District, Beijing, 100029, China.

Background: Well-differentiated pancreatic neuroendocrine tumors (PanNETs) usually have a good prognosis; however, there are patients that experience recurrence after curative resection.

Aim: To explore recurrence-related risk factors by analyzing clinicopathological data of PanNETs after radical surgery.

Methods: Clinical and pathological data from 47 patients with well-differentiated PanNETs at China-Japan Friendship Hospital from January 2012 to March 2016 were analyzed retrospectively. Read More

Pancreas 2019 04;48(4):e24-e26

Department of Surgery Galliera Hospital Genova, Italy Department of Pathology Galliera Hospital Genova, Italy Department of Surgery Monsignor Galliano Hospital Acqui Terme (AL), Italy Department of Surgery Galliera Hospital Genova, Italy.

Ann Dermatol Venereol 2019 May 23;146(5):419-421. Epub 2019 Mar 23.

Service de dermatologie et de vénérologie, centre hospitalier universitaire Ibn Sina, université Mohammed V, 10170 Rabat, Maroc.

CMAJ 2019 03;191(10):E286

Department of Dermatology, School of Medicine, Kyungpook National University, Kyungpook National University Hospital, Daegu, Republic of Korea

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

Curr Radiopharm 2019 ;12(2):135-155

Hammersmith Hospital, Imperial College Healthcare NHS Trust, London W12 0HS, United Kingdom.

Introduction: Pancreatic Neuroendocrine Tumours (p-NETs) are an important disease entity and comprise of peptide-secreting tumours often with a functional syndrome. Accounting for a small percentage of all pancreatic tumours, they have a good overall survival rate when diagnosed early, with surgery being curative. The role of nuclear medicine in the diagnosis and treatment of these tumours is evident. 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 01;180(1):e1

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

J Clin Endocrinol Metab 2019 04;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.

Rev Endocr Metab Disord 2018 06;19(2):145-158

Department of Internal Medicine IV, University-Hospital Campus Grosshadern, Ludwig-Maximilian University of Munich, Munich, Germany.

Neuroendocrine tumors (NETs) are a group of rare and heterogeneous malignancies that can develop in various organs. A significant number of gastroenteropancreatic neuroendocrine tumours (GEP-NETs) is functionally active and presents with symptoms related to the secretion of biologically active substances, leading to the development of distinct clinical syndromes. There are various therapeutic approaches for GEP-NETs, including curative surgery, palliative surgery, local-ablative and loco-regional therapies as well as systemic therapeutic options including peptide receptor radionuclide therapy, cytotoxic therapy, and molecularly targeted therapies. Read More

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