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https://www.readbyqxmd.com/read/29450880/a-review-of-cutaneous-manifestations-within-glucagonoma-syndrome-necrolytic-migratory-erythema
#1
REVIEW
Starling Tolliver, Jaqueline Graham, Benjamin H Kaffenberger
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. We present a case of a patient with a 1-year history of a pancreatic glucagonoma that developed an ulcerated, plaque-like, weeping rash over multiple areas of their body despite current treatment with Octreotide and stable pancreatic tumor staging...
February 16, 2018: International Journal of Dermatology
https://www.readbyqxmd.com/read/29435000/glucagonoma-and-the-glucagonoma-syndrome
#2
Xujun Song, Suli Zheng, Gang Yang, Guangbing Xiong, Zhe Cao, Mengyu Feng, Taiping Zhang, Yupei Zhao
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. The current review consisted of 268 males and 339 females, with an average age of 52...
March 2018: Oncology Letters
https://www.readbyqxmd.com/read/29432346/a-case-series-of-molecular-imaging-of-glucagonoma-after-initial-therapy-68ga-dotatate-pet-ct-reveals-similar-results-as-in-neuroendocrine-tumors-of-other-origin-in-follow-up-and-re-evaluation
#3
Christof Rottenburger, Dimitrios Papantoniou, Dalvinder Mandair, Martyn Caplin, Shaunak Navalkissoor
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. All patients were diagnosed by either histology and/or elevated serum levels of glucagon. The presented cases suggest that somatostatin receptor-based imaging can probably be used for re-evaluation of disease status in patients with glucagonoma in a similar way as it is already established for neuroendocrine tumors of other origin...
February 9, 2018: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29419911/normoglycemic-glucagonoma-syndrome-associated-with-necrolytic-migratory-erythema
#4
R Feldmann, S Wahl, A Steiner
Glucagonoma is an extremely rare tumor of the pancreatic alpha-cells. Its estimated annual incidence is 1 case per 20 million individuals. Necrolytic migratory erythema (NME) is the hallmark clinical sign. We report a patient with normoglycemic glucagonoma and NME. A 44-year-old male patient presented with a 4-year history of unexplained exanthema and unintentional weight loss. Skin examination revealed a mildly pruritic rash on the trunc, the extremities and the face (fig 1). One found erythematous polycyclic migratory lesions with scaling and crusting margins and central resolution...
February 8, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29390362/malignant-transformation-of-glucagonoma-with-spect-ct-in-111-octreoscan-features-a-case-report
#5
Giuseppe Corrias, Natally Horvat, Serena Monti, Olca Basturk, Oscar Lin, Luca Saba, Lisa Bodei, Diane L Reidy, Lorenzo Mannelli
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. Her symptoms at the onset were typical of the glucagonoma syndrome. DIAGNOSES: After a significant weight loss, she underwent a computer tomography scan of the abdomen, which showed a hypervascular lesion of the tail of the pancreas and hypervascular lesions of the liver...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29074313/psoriasiform-lesions-uncommon-presentation-of-glucagonoma
#6
Álvaro Martínez Manzano, María Dolores Balsalobre Salmerón, María Aránzazu García López, Sara Soto García, José Luis Vázquez Rojas
No abstract text is available yet for this article.
October 23, 2017: Gastroenterología y Hepatología
https://www.readbyqxmd.com/read/29069049/glucagonoma-syndrome-with-serous-oligocystic-adenoma-a-rare-case-report
#7
Yun Gao, Chun Wang, Yunyi Gao, Huijiao Chen, Bing Peng, Weixia Chen, Xingwu Ran
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...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28982222/emergency-pancreatic-resection-for-glucagonoma-associated-with-severe-necrolytic-migratory-erythema
#8
Daniel M Daly, Benjamin Thompson, Julia Low, Jacqui Slater, Glenda Wood, Vasanth Kamath, Koroush S Haghighi
No abstract text is available yet for this article.
October 5, 2017: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/28662584/necrolytic-migratory-erythema-a-forgotten-paraneoplastic-condition
#9
Catherine Tremblay, Isabelle Marcil
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. OBJECTIVE: Although rare, all dermatologists must know and recognise NME promptly to request useful investigations for the diagnosis of this characteristic neuroendocrine tumour...
June 1, 2017: Journal of Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/28639759/-diabetes-mellitus-secondary-to-an-endocrine-pathology-when-to-think-about-it
#10
Nathalie Rouiller, François R Jornayvaz
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. Hyperthyroidism, hyperaldosteronism, glucagonoma and somatostatinoma lead to hyperglycemia by other physiopathological mechanisms detailed in this article...
May 31, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/28566601/challenging-differential-diagnosis-of-hypergastremia-and-hyperglucagonemia-with-chronic-renal-failure-report-of-a-case-with-multiple-endocrine-neoplasia-type-1
#11
Takaaki Murakami, Takeshi Usui, Yuji Nakamoto, Akio Nakajima, Yuki Mochida, Sumio Saito, Takahiro Shibayama, Nobuhisa Yamazaki, Tomonobu Hatoko, Tomoko Kato, Shin Yonemitsu, Seiji Muro, Shogo Oki
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. A pathological examination revealed glucagonoma and a lymph node gastrinoma...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28507564/management-of-the-hormonal-syndrome-of-neuroendocrine-tumors
#12
Paweł Gut, Joanna Waligórska-Stachura, Agata Czarnywojtek, Nadia Sawicka-Gutaj, Maciej Bączyk, Katarzyna Ziemnicka, Jakub Fischbach, Kosma Woliński, Jarosław Kaznowski, Elżbieta Wrotkowska, Marek Ruchała
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. Somatostatin have a broad range of biological actions that include inhibition of exocrine and endocrine secretions, gut motility, cell proliferation, cell survival and angiogenesis...
April 1, 2017: Archives of Medical Science: AMS
https://www.readbyqxmd.com/read/28329615/necrolytic-migratory-erythema-associated-with-fatty-liver-disease-and-the-psuedoglucagonoma-syndrome
#13
Collin Fuller, Jaimie Glick, Shelly Rivas, Katy Burris
We report a 48-year-old woman with a past medical history of psoriasis, nonalcoholic steatohepatitis (NASH), and type II diabetes mellitus, who presented to the emergency department with a 1 week history of erosive annular plaques with associated atrophy and telangiectasias on her legs bilaterally, thighs and buttock, histopathologically consistent with necrolytic migratory erythema. Although classically associated with a pancreatic glucagonoma, this patient experienced this figurate erythema in the setting of fatty liver disease with no glucagonoma...
September 15, 2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/28273025/glucagonoma-associated-rash
#14
Luke Mountjoy, Daniel Kollmorgen
A 65-year-old man with type 2 diabetes and deterioration of glycemic control presented with a 2-to-3-month history of weight loss (7 to 8 kg), early satiety, and mild flank pain. Before the occurrence of these symptoms, a rash had developed over the course of 3 to 4 weeks. The rash involved the..
March 9, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28218201/b-mode-and-contrast-enhancement-characteristics-of-small-nonincidental-neuroendocrine-pancreatic-tumors
#15
Barbara Braden, Christian Jenssen, Mirko D'Onofrio, Michael Hocke, Uwe Will, Kathleen Möller, Andre Ignee, Yi Dong, Xin-Wu Cui, Adrian Sãftoiu, Christoph F Dietrich
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. PATIENTS AND METHODS: In this multicenter study, we retrospectively analyzed the endosonographic characteristics of small pNETs which had been detected due to typical biochemistry and clinical symptoms or during surveillance of MEN 1...
January 2017: Endoscopic Ultrasound
https://www.readbyqxmd.com/read/28188614/foxa2-a-novel-protein-partner-of-the-tumour-suppressor-menin-is-deregulated-in-mouse-and-human-men1-glucagonomas
#16
Rémy Bonnavion, Romain Teinturier, Samuele Gherardi, Emmanuelle Leteurtre, Run Yu, Martine Cordier-Bussat, Rui Du, François Pattou, Marie-Christine Vantyghem, Philippe Bertolino, Jieli Lu, Chang Xian Zhang
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. In the current study, we showed that Foxa2 was overexpressed in islets from α-cell-specific Men1 mutant mice, at both the transcriptional level and the protein level. More importantly, immunostaining analyses showed its prominent nuclear accumulation, specifically in α-cells, at a very early stage after Men1 disruption...
May 2017: Journal of Pathology
https://www.readbyqxmd.com/read/28108603/glucagon-and-amino-acids-are-linked-in-a-mutual-feedback-cycle-the-liver-%C3%AE-cell-axis
#17
Jens J Holst, Nicolai J Wewer Albrechtsen, Jens Pedersen, Filip K Knop
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...
February 2017: Diabetes
https://www.readbyqxmd.com/read/27933176/pancreatic-%C3%AE-cell-hyperplasia-and-hyperglucagonemia-due-to-a-glucagon-receptor-splice-mutation
#18
Etienne Larger, Nicolai J Wewer Albrechtsen, Lars H Hansen, Richard W Gelling, Jacqueline Capeau, Carolyn F Deacon, Ole D Madsen, Fumiatsu Yakushiji, Pierre De Meyts, Jens J Holst, Erica Nishimura
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. Here, we describe a patient carrying a homozygous G to A substitution in the invariant AG dinucleotide found in a 3' mRNA splice junction of the glucagon receptor gene...
2016: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/27863775/operation-for-insulinomas-in-multiple-endocrine-neoplasia-type-1-when-pancreatoduodenectomy-is-appropriate
#19
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...
March 2017: Surgery
https://www.readbyqxmd.com/read/27622478/necrolytic-migratory-erythema-and-pancreatic-glucagonoma
#20
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...
June 3, 2016: Biomédica: Revista del Instituto Nacional de Salud
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