1,123 results match your criteria Glucagonoma Syndrome

[Migratory necrolytic erythema as a manifestation of pancreatic neuroendocrine tumor. Clinical-radiological evaluation].

Rev Fac Cien Med Univ Nac Cordoba 2022 06 6;79(2):188-192. Epub 2022 Jun 6.

Hospital Italiano de Buenos Aires.

Glucagonoma is a neuroendocrine tumour of very low incidence, estimated at 1 case per 20 million people per year. It typically manifests with a clinical syndrome that includes a characteristic dermatosis called necrolytic migratory erythema (NME). We present the case of a 60-year-old male with NME as the initial presentation of a pancreatic tumour and its imaging findings. Read More

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Necrolytic migratory erythema is an important visual cutaneous clue of glucagonoma.

Sci Rep 2022 May 31;12(1):9053. Epub 2022 May 31.

Department of Hepatobiliary Surgery, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, 710061, People's Republic of China.

Glucagonoma is an extremely rare neuroendocrine tumor that arises from pancreatic islet alpha cells. Although glucagonoma is usually accompanied by a variety of characteristic clinical symptoms, early diagnosis is still difficult due to the scarcity of the disease. In this study, we present the cumulative experiences, clinical characteristics and treatments of seven patients diagnosed with glucagonoma during the past 10 years at the First Affiliated Hospital of Xi'an Jiaotong University. Read More

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Case report of a recurrent resected glucagonoma.

Ann Med Surg (Lond) 2022 May 9;77:103604. Epub 2022 Apr 9.

Department of Surgery, A La Rabta, Tunis, Tunisia.

Introduction And Importance: Glucagonoma is a rare neuroendocrine tumor (NET). Most glucagonomas are in the tail or body of the pancreas and are diagnosed at a metastatic stage. We report a case of an early recurrence after surgical resection of a glucagonoma and its management. Read More

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A Glucagonoma Presenting as Cerebral Vein Thrombosis and Diabetes.

Case Rep Endocrinol 2022 22;2022:7659341. Epub 2022 Apr 22.

Division of Endocrinology and Metabolism, Department of Medicine, McGill University, Montreal, Quebec, Canada.

Glucagonomas are rare pancreatic neuroendocrine tumors (pNETs), malignant in 80% of cases, thus highlighting the importance of early diagnosis and treatment. Primary manifestations are diabetes, dermatosis, depression, weight loss, and deep vein thrombosis. Unlike other pNETs, glucagonomas are associated with a higher incidence of thromboembolic events, often resulting in death. Read More

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How Many Times Can One Go Back to the Drawing Board before the Accurate Diagnosis and Surgical Treatment of Glucagonoma?

Diagnostics (Basel) 2022 Jan 16;12(1). Epub 2022 Jan 16.

Department of Endocrinology, Diabetes Mellitus, Nutrition and Metabolic Disorders, Carol Davila University of Medicine and Pharmacy, 020021 Bucharest, Romania.

Glucagonomas are neuroendocrine tumors (NETs) that arise from the alpha cells of the pancreatic islets. They are typically slow-growing tumors associated with abnormal glucagon secretion, resulting in one or more non-specific clinical features, such as necrolytic migratory erythema (NME), diabetes, diarrhea, deep vein thrombosis, weight loss, and depression. Here, we report the case of a 44-year-old male with a history of diabetes mellitus, presenting with a pruritic and painful disseminated cutaneous eruption of erythematous plaques, with scales and peripheral pustules, misdiagnosed as disseminated pustular psoriasis and treated for 2 years with oral retinoid and glucocorticoids. Read More

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January 2022

Case of delayed diagnosis of necrolytic migratory erythema.

Clin Case Rep 2021 Dec 11;9(12):e05179. Epub 2021 Dec 11.

Dermatology Department - Rabta Hospital Tunis Tunisia.

Necrolytic migratory erythema (NME) is a rare cutaneous paraneoplastic manifestation of glucagonoma. We report a case of a woman with a 6-year history of delayed diagnosis of glucagonoma. This case highlights the atypical clinical features of NME which makes the diagnosis difficult. Read More

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December 2021

[Necrolytic migratory erythema in a patient with a glucagonoma].

Ned Tijdschr Geneeskd 2021 10 11;165. Epub 2021 Oct 11.

Amphia ziekenhuis, afd. Dermatologie, Breda.

This case concerns an 81-year-old man with weight loss and erythematosquamous plaques, with central clearing and atrophy. Due to a CT scan and blood test the diagnosis necrolytic migratory erythema as a paraneoplastic manifestation of glucagonoma was made. The pathogenesis is not completely elucidated. Read More

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October 2021

Bilateral Vestibular Failure May Improve With Treatment of the Underlying Condition: A Report of 2 Cases.

Neurologist 2021 Nov 26;27(2):79-81. Epub 2021 Nov 26.

Department of Neurology, Wellington Hospital, Wellington, New Zealand.

Introduction: Bilateral vestibular failure (BVF) is an uncommon condition with numerous etiologies. It causes chronic oscillopsia and imbalance and is usually irreversible. We report 2 cases of BVF due to unusual causes, both of which improved with treatment of the underlying condition. Read More

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November 2021

Glucagonoma syndrome with necrolytic migratory erythema as initial manifestation.

Hepatobiliary Pancreat Dis Int 2021 Dec 8;20(6):598-600. Epub 2021 Sep 8.

Department of Hepatobiliary and Pancreatic Surgery, the First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China. Electronic address:

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December 2021

Necrolytic migratory erythema-like eruption and paradoxical psoriasis associated with adalimumab treatment.

J Dermatol 2021 Nov 6;48(11):e572-e573. Epub 2021 Sep 6.

Department of Dermatology, Zhongshan Hospital Xiamen University, Xiamen, China.

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November 2021

Distinctive detection of insulinoma using [F]FB(ePEG12)12-exendin-4 PET/CT.

Sci Rep 2021 07 22;11(1):15014. Epub 2021 Jul 22.

Department of Diabetes, Endocrinology and Nutrition, Graduate School of Medicine, Kyoto University, 54 Kawahara-cho, Shogoin, Sakyo-ku, Kyoto, 606-8507, Japan.

Specifying the exact localization of insulinoma remains challenging due to the lack of insulinoma-specific imaging methods. Recently, glucagon-like peptide-1 receptor (GLP-1R)-targeted imaging, especially positron emission tomography (PET), has emerged. Although various radiolabeled GLP-1R agonist exendin-4-based probes with chemical modifications for PET imaging have been investigated, an optimal candidate probe and its scanning protocol remain a necessity. Read More

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A germline c.1546dupC MEN1 mutation in an MEN1 family: A case report.

Medicine (Baltimore) 2021 Jun;100(25):e26382

Division of Endocrinology and Metabolism, Department of Internal Medicine, Chung-Ang University, College of Medicine, Seoul, Korea.

Rationale: Multiple endocrine neoplasia type 1 (MEN1) is a rare tumor syndrome with an autosomal dominant inheritance, and genetic testing for MEN1 gene is important for both affected individuals and their relatives. We present a 2-person family affected by a germline c.1546dupC MEN1 mutation, and one of them had a full-spectrum of MEN-related endocrine tumors. Read More

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Glucagonoma with diffuse enlargement of pancreas mimicking autoimmune pancreatitis diagnosed by EUS-guided FNA.

Gastrointest Endosc 2021 10 28;94(4):862-863.e1. Epub 2021 May 28.

Department of Gastroenterology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, Jiangsu, China.

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October 2021

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

Clin Nucl Med 2021 Dec;46(12):1002-1003

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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December 2021

[Neuroendorine paraneoplastic syndromes].

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 06 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

A Rare Cause of Rash.

Gastroenterology 2021 May 21;160(6):1943-1946. Epub 2020 Aug 21.

Department of Hepatobiliary Surgery, Xijing Hospital, Fourth Military Medical University, Xi'an, China. Electronic address:

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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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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