1,111 results match your criteria Glucagonoma Syndrome


Glucagonoma with Diffuse Enlargement of Pancreas Mimicking Autoimmune Pancreatitis Diagnosed by EUS-guided Fine-needle Aspiration.

Gastrointest Endosc 2021 May 28. Epub 2021 May 28.

Department of Gastroenterology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School. Electronic address:

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Treatment of Glucagonoma-Related Necrolytic Migratory Erythema With Peptide Receptor Radionuclide Therapy.

Clin Nucl Med 2021 May 26. Epub 2021 May 26.

From the Department of Nuclear Medicine, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

Abstract: Glucagonomas are rare types of pancreatic neuroendocrine tumors. They may present with a clinical entity called glucagonoma syndrome, which includes necrolytic migratory erythema as a skin component. Here we present a 26-year-old woman experiencing ongoing skin lesions, excessive weight loss, and nausea. Read More

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[Neuroendorine paraneoplastic syndromes].

Authors:
M Böhm R Gellner

Hautarzt 2021 Apr 4;72(4):299-306. Epub 2021 Mar 4.

Medizinische Klinik B, Universitätsklinikum Münster, Münster, Deutschland.

Skin is commonly affected by neuroendorine paraneoplastic syndromes (PNS). This is due to the expression of receptors in the skin by which abnormally secreted neuroendocrine hormones and mediators elicit directly, and indirectly, cutaneous key signs and thus facilitate early diagnosis of these diseases. In acromegaly, induction of the growth hormone-insulin-like growth factor‑1 axis results in trophic changes of the acral portions of the skin and mucosal membranes including cutis verticis gyrata. Read More

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Pancreatoduodenectomy for Neuroendocrine Tumors in Patients with Multiple Endocrine Neoplasia Type 1: An AFCE (Association Francophone de Chirurgie Endocrinienne) and GTE (Groupe d'étude des Tumeurs Endocrines) Study.

World J Surg 2021 Jun 1;45(6):1794-1802. Epub 2021 Mar 1.

Department of Digestive and Endocrine Surgery, Dijon University Hospital, University of Burgundy, Dijon, France.

Aim: To assess postoperative complications and control of hormone secretions following pancreatoduodenectomy (PD) performed on multiple endocrine neoplasia type 1 (MEN1) patients with duodenopancreatic neuroendocrine tumors (DP-NETs).

Background: The use of PD to treat MEN1 remains controversial, and evaluating the right place of PD in MEN1 disease makes sense.

Methods: Thirty-one MEN1 patients from the Groupe d'étude des Tumeurs Endocrines MEN1 cohort who underwent PD for DP-NETs between 1971 and 2013 were included. Read More

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Pancreatic neuroendocrine neoplasms: Clinicopathological features and pathological staging.

Histol Histopathol 2021 Apr 11;36(4):367-382. Epub 2020 Dec 11.

School of Medicine, Griffith University, Gold Coast, QLD, Australia.

The nomenclature and classification of pancreatic neuroendocrine neoplasms has evolved in the last 15 years based on the advances in knowledge of the genomics, clinical behaviour and response to therapies. The current 2019 World Health Organization classification of pancreatic neuroendocrine neoplasms categorises them into three groups; pancreatic neuroendocrine tumours (PanNETs)(grade 1 grade 2, grade 3), pancreatic neuroendocrine carcinomas and mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs) based on the mitotic rate, Ki-67 index, morphological differentiation and/or co-existing tissue subtype. PanNETs are also classified into non-functional NET, insulinoma, gastrinoma, VIPoma, glucagonoma, somatostatinoma, ACTH-producing NET and serotonin producing NET based on hormone production and clinical manifestations. Read More

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Clinical Characteristics and Management of Functional Pancreatic Neuroendocrine Neoplasms: A Single Institution 20-Year Experience with 286 Patients.

Int J Endocrinol 2020 6;2020:1030518. Epub 2020 Nov 6.

Department of Endocrinology, Chinese PLA General Hospital, Beijing 100853, China.

Background: Functional pancreatic neuroendocrine neoplasms (PanNENs) are very rare disorders but have complex spectrum, including insulinoma, gastrinoma, glucagonoma, somatostatinoma, and VIPoma. Patients with PanNENs usually present with characteristic symptoms caused by corresponding hormone hypersecretion. It has always been challenging in dealing with such rare but complicated disorders. Read More

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November 2020

Perspectives on the current pharmacotherapeutic strategies for management of functional neuroendocrine tumor syndromes.

Expert Opin Pharmacother 2021 Apr 11;22(6):685-693. Epub 2020 Nov 11.

Digestive Diseases Branch, NIDDK,NIH, Bethesda, MD, USA.

: In the past, controlling the hormone-excess-state was the main determinant of survival in Functional-Neuroendocrine-Neoplasm-syndromes (F-NENs). This was difficult because the pharmacological-armamentarium available was limited. Recently, new therapeutic strategies have increased but it also generated controversies/uncertainties. Read More

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Benign-Appearing Glucagonoma Undergoing Malignant Transformation after a Prolonged Period.

Case Rep Oncol 2020 Sep-Dec;13(3):1109-1115. Epub 2020 Sep 21.

Department of Endocrinology, Walter Reed National Military Medical Center, Bethesda, Maryland, USA.

Glucagonoma are rare neuroendocrine tumors arising in the Langerhans islets of the pancreas. We report a patient with glucagonoma differentiation into a malignant form after 13 years. A 63-year-old asymptomatic man was evaluated for an enhancing lesion at the distal pancreas. Read More

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September 2020

Glucagonoma: From skin lesions to the neuroendocrine component (Review).

Exp Ther Med 2020 Oct 3;20(4):3389-3393. Epub 2020 Jul 3.

Department of Gynecology, Emergency University Hospital, 050098 Bucharest, Romania.

Glucagonoma is a hormonally active rare pancreatic neuroendocrine tumour causing an excess of glucagon. This is a narrative review based on a multidisciplinary approach of the tumour. Typically associated dermatosis is necrolytic migratory erythema (NME) which is most frequently seen at disease onset. Read More

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October 2020

The RNA-binding protein, HuD regulates proglucagon biosynthesis in pancreatic α cells.

Biochem Biophys Res Commun 2020 09 6;530(1):266-272. Epub 2020 Aug 6.

Department of Biochemistry, College of Medicine, The Catholic University of Korea, Seoul, 06591, South Korea; Institute for Aging and Metabolic Diseases, College of Medicine, The Catholic University of Korea, Seoul, 06591, South Korea. Electronic address:

Glucagon is a peptide hormone generated by pancreatic α cells. It is the counterpart of insulin and plays an essential role in the regulation of blood glucose level. Therefore, a tight regulation of glucagon levels is pivotal to maintain homeostasis of blood glucose. Read More

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September 2020

Necrolytic Migratory Erythema Associated with a Glucagonoma.

N Engl J Med 2020 Aug;383(6):e39

P.D. Hinduja National Hospital and Medical Research Center, Mumbai, India

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Necrolytic migratory erythema: an important sign of glucagonoma.

Postgrad Med J 2021 Mar 11;97(1145):199. Epub 2020 Jun 11.

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

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MANIFESTATIONS OF GLUCAGONOMA SYNDROME.

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

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January 2020

The magnesium transporter NIPAL1 is a pancreatic islet-expressed protein that conditionally impacts insulin secretion.

J Biol Chem 2020 07 21;295(29):9879-9892. Epub 2020 May 21.

Department of Physiology, University of Toronto, Toronto, Ontario, Canada

Type 2 diabetes is a chronic metabolic disease characterized by pancreatic β-cell dysfunction and peripheral insulin resistance. Among individuals with type 2 diabetes, ∼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

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Pseudoglucagonoma syndrome: Description of an 'Idiopatic' case.

Australas J Dermatol 2020 Nov 8;61(4):e403-e405. 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

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November 2020

Necrolytic Acral Erythema: Current Insights.

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

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Neuropsychiatric symptoms, skin disease, and weight loss: necrolytic migratory erythema and a glucagonoma.

Lancet 2020 03;395(10228):985

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

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Proof of concept for stereotactic body radiation therapy in the treatment of functional neuroendocrine neoplasms.

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

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January 2020

A retrospective comparison of robotic versus laparoscopic distal resection and enucleation for potentially benign pancreatic neoplasms.

Surg Today 2020 Aug 3;50(8):872-880. 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

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[Facial edema and erythroderma in a 54 year-old woman].

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.

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Application of Somatostatin, Chemotherapy Combined with TAE in Heterogeneous Glucagonoma Presented with Necrolytic Migratory Erythema.

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

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December 2019

Occult insulinoma, glucagonoma and pancreatic endocrine pseudotumour in a patient with multiple endocrine neoplasia type 1.

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:

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Case of autoimmune progesterone dermatitis presenting as necrotic migratory erythema successfully controlled by danazol.

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

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February 2020

Diarrhea: a missed D in the 4D glucagonoma syndrome.

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

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November 2019

Molecular imaging of a glucagonoma with F-FDG PET/CT and Ga-DOTATATE PET/CT imaging: A case report and review of the literature.

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

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January 2020

Unremitting chronic skin lesions: a case of delayed diagnosis of glucagonoma.

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

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November 2019

Spleen-preserving distal pancreatectomy and lymphadenectomy for glucagonoma syndrome: A case report.

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

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September 2019

Glucagonoma syndrome with severe erythematous rash: A rare case report.

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

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September 2019

Glucagonoma-related necrolytic migratory erythema.

Med Clin (Barc) 2020 11 9;155(9):418-419. Epub 2019 Sep 9.

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

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November 2020