O glucagonoma é um tipo raro e funcionante de tumor neuroendócrino pancreático (PNET) que hipersecreta glucagon, levando a uma síndrome composta por eritema migratório necrolítico, diabetes mellitus, anemia, perda de peso, anormalidades mucosas, tromboembolismo, sintomas gastrointestinais e neuropsiquiátricos.
Introdução
O que você precisa saber de cara
O glucagonoma é um tipo raro e funcionante de tumor neuroendócrino pancreático (PNET) que hipersecreta glucagon, levando a uma síndrome composta por eritema migratório necrolítico, diabetes mellitus, anemia, perda de peso, anormalidades mucosas, tromboembolismo, sintomas gastrointestinais e neuropsiquiátricos.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 14 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 42 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Glucagonoma
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Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
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Pesquisa e ensaios clínicos
34 ensaios clínicos encontrados, 2 ativos.
Publicações mais relevantes
Glucagonoma of the pancreas: diagnostic approach and therapeutic algorithm for a rare nosological entity.
Glucagonoma remains a very rare neuroendocrine tumor of the pancreas, accounting for 2% of all islet-cell carcinomas. The aim of this review is to highlight important aspects of pancreatic glucagonoma, including epidemiology, clinical presentation and diagnostic evaluation, and to elucidate the current therapeutic management of this nosologic entity. A combined automated and manual systematic search of the literature was performed using electronic search engines (Medline/PubMed, Scopus, Ovid and Cochrane Library), until April 2025. Glucagonoma originates from the a-cells of the pancreatic Langerhans islets. Its reported incidence is 1 in 20,000,000 per year. Typical clinical manifestations include necrolytic migratory erythema, diabetes mellitus (DM), weight loss and anemia, along with elevated serum glucagon levels. Other symptoms, such as venous thrombosis, neuropsychiatric findings known as 4D (dermatitis, DM, deep-vein thrombosis, depression), or diarrhea can also be present. Unfortunately, metastases are encountered in the majority of patients. Prompt diagnosis is usually accomplished by computed tomography and magnetic resonance imaging. The only treatment option is the surgical resection of the tumor. Conservative management, based on the administration of chemotherapy, somatostatin analogs, molecular targeted therapy and peptide receptor radionuclide therapy is also an alternative.
Glucagonoma Syndrome With Necrolytic Migratory Erythema.
Glucagonoma is a rare pancreatic alpha-cell tumor that leads to glucagonoma syndrome, typically characterized by necrolytic migratory erythema (NME), diabetes, weight loss, and anemia. We present the case of a 47-year-old man with a one-year history of diabetes and a relapsing pustular and scaly dermatosis. Examination revealed widespread annular erythematous plaques. Laboratory tests showed anemia, hypoalbuminemia, and hyperglycemia. Skin biopsy demonstrated epidermal necrolysis, and abdominal imaging identified a 3.1 cm tumor in the pancreatic tail. Surgical resection confirmed a well-differentiated neuroendocrine tumor (Ki67: 1%), and skin lesions resolved postoperatively. This case highlights an atypical presentation of NME and the importance of early recognition to reduce diagnostic delay in glucagonoma syndrome.
Shift in secretory profile of a pNET: from indolent glucagonoma to aggressive insulinoma - a case report.
Pancreatic neuroendocrine tumors (pNETs) can change their hormonal profile over time, leading to new clinical syndromes that significantly impact prognosis and management. We report the case of a patient with a metastatic glucagon-secreting pNET who, after 14 years of disease and multiple treatment lines, developed insulin hypersecretion and severe, treatment-refractory hypoglycemia. Despite several strategies, including diazoxide, somatostatin analogues, glucocorticoids, everolimus, peptide receptor radionuclide therapy, and continuous glucose and glucagon infusions, glycemic control was not achieved, and the patient ultimately died from treatment complications. This case highlights the clinical challenges of managing metachronous hormonal syndromes and the importance of long-term endocrine follow-up in pNET patients. It also emphasizes the limitations of current therapeutic strategies and the urgent need for new treatment options, including alpha-emitting radiopharmaceuticals, which may offer improved disease and symptom control in advanced, insulin-secreting pNETs.
Necrolytic Migratory Erythema as the First Manifestation of Functional Conversion in a Glucagonoma: A Case Report.
Necrolytic migratory erythema is a characteristic but often underrecognized manifestation of glucagonoma. Its timely identification can lead to early diagnosis and curative surgery of pancreatic neuroendocrine tumors that initially present as clinically silent lesions.
[The 514th case: urinary stone excretion, elevated blood glucose, pancreatic mass,and co-secretion of multiple hormones].
A 44-year-old male presented with a 19-year history of urinary calculi and a 1-year history of polydipsia and weight loss. Laboratory tests revealed hyperparathyroidism and evidence of glucagonoma-associated diabetes. Imaging studies identified masses in the pancreatic head and body/tail, suggestive of glucagonoma and a parathyroid adenoma. Furthermore, the patient exhibited hypercalcitoninemia and elevated cortisol and adrenocorticotropic hormone levels. Genetic testing revealed a heterozygous MEN1 mutation [c.65T>G (p.Leu22Arg)], confirming the diagnosis of multiple endocrine neoplasia type 1 (MEN-1). The patient subsequently underwent near-total parathyroidectomy and total pancreatectomy. Postoperative immunohistochemical staining of the pancreatic tail tumor was positive for glucagon and calcitonin. The patient's postoperative hormone levels (calcitonin, glucagon, adrenocorticotropic hormone, cortisol) normalized, suggesting a rare pancreatic neuroendocrine tumor (pNET) that was co-secreting multiple hormones. Postoperative management included pancreatic enzyme supplementation, calcium supplementation, vitamin D supplementation, and insulin for glycemic control. Follow-up evaluations at 10 months demonstrated a stable clinical condition, well-controlled blood glucose and biochemical parameters, and an acceptable quality of life. This case study highlights that the presence of pNETs should be considered in patients with MEN-1 and multiple abnormal hormone levels. Timely surgical management of the involved glands and postoperative complications can effectively improve prognosis. 患者男,44岁,因尿中排石19年,多饮、消瘦1年就诊。实验室检查提示甲状旁腺功能亢进、胰高血糖素分泌过多引起的特殊类型糖尿病,影像学提示胰腺头部及体尾部占位,考虑为胰高血糖素瘤、甲状旁腺腺瘤。患者同时合并高降钙素血症,皮质醇及促肾上腺皮质激素升高,基因检测见MEN1:c.65T>G(p.Leu22Arg)杂合变异,确诊为多发性内分泌腺瘤病1型(MEN-1),先后行甲状旁腺近全切除术、全胰切除术。术后病理免疫组织化学染色提示胰尾肿瘤组织中胰高血糖素、降钙素均为阳性,且术后降钙素、胰高血糖素、促肾上腺皮质激素及皮质醇等激素均降至正常,考虑为罕见的共分泌多种激素的胰腺神经内分泌肿瘤(pNET)。术后予补充胰酶、钙剂、活性维生素D及胰岛素降糖治疗。随访10个月时患者病情平稳,血糖及生化指标控制达标,生活质量可。本例的诊治提示对于出现多种异常激素水平的MEN-1患者,应考虑pNET的可能,受累腺体的及时手术及术后并发症的管理可有效改善患者的预后。.
Publicações recentes
[Necrolytic migratory erythema as the key to the diagnosis of pancreatic neoplasms].
🥉 Relato de casoSomatostatin Receptor PET/CT in a Glucagonoma Presenting With Necrolytic Migratory Erythema and Normoglucagonemia: A Diagnostic Dilemma.
🥉 Relato de casoGlucagonoma of the pancreas: diagnostic approach and therapeutic algorithm for a rare nosological entity.
Glucagonoma Syndrome With Necrolytic Migratory Erythema.
🥉 Relato de casoShift in secretory profile of a pNET: from indolent glucagonoma to aggressive insulinoma - a case report.
📚 EuropePMC504 artigos no totalmostrando 179
Glucagonoma of the pancreas: diagnostic approach and therapeutic algorithm for a rare nosological entity.
Annals of gastroenterologyGlucagonoma Syndrome With Necrolytic Migratory Erythema.
CureusShift in secretory profile of a pNET: from indolent glucagonoma to aggressive insulinoma - a case report.
Frontiers in endocrinologyNecrolytic Migratory Erythema as the First Manifestation of Functional Conversion in a Glucagonoma: A Case Report.
Clinical case reports[The 514th case: urinary stone excretion, elevated blood glucose, pancreatic mass,and co-secretion of multiple hormones].
Zhonghua nei ke za zhiStudy on Autophagy Death of Alpha TC1 Clone 6 (αTC1-6) Cells Induced by Trametenolic Acid Through PI3K/AKT Pathway.
Current issues in molecular biologyDiagnosis and Management of Functional Pancreatic Neuroendocrine Tumors in Children-A Systematic Review.
Diagnostics (Basel, Switzerland)Diagnostic characteristics, treatment outcomes, and prognostic factors in glucagonomas.
Endocrine oncology (Bristol, England)A unique case of glucagonoma with atypical necrolytic migratory erythema.
JAAD case reportsPremortem diagnosis of a pancreatic neuroendocrine tumour (glucagonoma) in a captive North American river otter (Lontra canadensis).
Journal of comparative pathologyNutritional Management of Functioning GEP-NENs.
NutrientsA Case Report of Necrolytic Migratory Erythema Associated with Glucagonoma.
Clinical, cosmetic and investigational dermatologyGlucagonoma and Glucagonoma Syndrome: An Updated Review.
Clinical endocrinologyNecrolytic migratory erythema with a pancreatic glucagonoma.
The British journal of dermatologyFamilial Mahvash disease with metastatic pancreatic NET and MEN1 mutations.
Endocrine-related cancerFunctioning neuroendocrine tumors (NET): Minimum requirements for a NET specialist.
Cancer treatment reviewsClinical Relevance of ATRX/DAXX Gene Mutations and ALT in Functioning Pancreatic Neuroendocrine Tumors.
Endocrine pathologyCase report: Anesthesia management for surgical treatment of glucagonoma with symptom of characterized necrolytic migratory erythema.
Frontiers in oncologyNecrolytic migratory erythema: a fortuitous diagnosis.
BMJ case reportsGlucagon-Producing Pancreatic Neuroendocrine Tumors (Glucagonomas) are Enriched in Aggressive Neoplasms with ARX and PDX1 Co-expression, DAXX/ATRX Mutations, and ALT (Alternative Lengthening of Telomeres).
Endocrine pathologyNon-functional alpha-cell hyperplasia with glucagon-producing NET: a case report.
Frontiers in endocrinologyManifestations of Endocrine Disease in the Lower Extremities: Beyond the Diabetic Foot.
The international journal of lower extremity woundsCase report: Surgical treatment and literature review of a recurrent case of glucagonoma.
Frontiers in oncologyA nuclear beacon of hope: an advanced, metastatic glucagonoma treated with [177Lu]Lu-DOTA-TATE.
Nuclear medicine review. Central & Eastern EuropeManagement of functional neuroendocrine tumors.
Current problems in cancerNecrolytic migratory erythema caused by pancreatic hyperglycemia with emphasis on therapeutic and prognosis: A case report.
World journal of clinical casesCharacteristics, therapy, and outcome of rare functioning pancreatic neuroendocrine neoplasms.
Scientific reports[Glucagonoma - a rare neuroendocrine tumor (NET) that typically originates in the pancreas].
LakartidningenAdvances in basic research on glucagon and alpha cells.
Diabetology internationalWell-differentiated G1 and G2 pancreatic neuroendocrine tumors: a meta-analysis of published expanded DNA sequencing data.
Frontiers in endocrinologyPseudoglucagonoma Syndrome Following Frey's Surgery: A Case Report of a Rare Presentation.
CureusGenetic disorders and insulinoma/glucagonoma.
Endocrine-related cancerDiagnosis and comprehensive treatment of a glucagonoma in a patient with residual intrahepatic metastases postoperatively: A case report and literature review.
Oncology lettersA novel likely pathogenetic variant p.(Cys235Arg) of the MEN1 gene in multiple endocrine neoplasia type 1 with multifocal glucagonomas.
Journal of endocrinological investigationCharacteristics and treatment options of glucagonomas: a national study from the French Group of Endocrine Tumors and ENDOCAN-RENATEN network.
European journal of endocrinologyHepatic Glucagonoma in a Post-bariatric Female Patient: A Case Report.
CureusGlucagonoma-induced Dilated Cardiomyopathy in a Young Woman.
JCEM case reportsSynchronous Insulinoma and Glucagonoma: A Review of the Literature.
In vivo (Athens, Greece)Practical therapeutic approach in the management of diabetes mellitus secondary to Cushing's syndrome, acromegaly and neuroendocrine tumours.
Frontiers in endocrinologyDiabetes Mellitus Secondary to Endocrine Diseases: An Update of Diagnostic and Treatment Particularities.
International journal of molecular sciencesEuropean Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes.
Journal of neuroendocrinologyEmerging therapies for advanced insulinomas and glucagonomas.
Endocrine-related cancerOne hundred years after the discovery of insulin and glucagon: the history of tumors and hyperplasias that hypersecrete these hormones.
Endocrine-related cancerGlucagon cell hyperplasia and neoplasia: a recently recognized endocrine receptor disease.
Endocrine-related cancerAnalysis of Clinical Characteristics and Survival in Patients With Functional Neuroendocrine Tumors of Gastrointestinal Origin.
PancreasA case of multiple glucagonomas with no clinical manifestations of excess glucagon despite hyperglucagonemia.
DEN openRecurrent pruritic polymorphic lesions associated with weight loss.
JAAD case reportsAn Insight on Functioning Pancreatic Neuroendocrine Neoplasms.
BiomedicinesCirculating Neuroendocrine Tumor Biomarkers: Past, Present and Future.
Journal of clinical medicineGlucagonoma Syndrome: A Rare Paraneoplastic Disorder due to Neuroendocrine Tumor of the Pancreas.
Journal of the College of Physicians and Surgeons--Pakistan : JCPSPSkin manifestations of neuroendocrine neoplasms: review of the literature.
Postepy dermatologii i alergologii[Migratory necrolytic erythema as a manifestation of pancreatic neuroendocrine tumor. Clinical-radiological evaluation].
Revista de la Facultad de Ciencias Medicas (Cordoba, Argentina)Necrolytic migratory erythema is an important visual cutaneous clue of glucagonoma.
Scientific reportsCase report of a recurrent resected glucagonoma.
Annals of medicine and surgery (2012)A Glucagonoma Presenting as Cerebral Vein Thrombosis and Diabetes.
Case reports in endocrinologyHow Many Times Can One Go Back to the Drawing Board before the Accurate Diagnosis and Surgical Treatment of Glucagonoma?
Diagnostics (Basel, Switzerland)Case of delayed diagnosis of necrolytic migratory erythema.
Clinical case reports[Necrolytic migratory erythema in a patient with a glucagonoma].
Nederlands tijdschrift voor geneeskundeBilateral Vestibular Failure May Improve With Treatment of the Underlying Condition: A Report of 2 Cases.
The neurologistGlucagonoma syndrome with necrolytic migratory erythema as initial manifestation.
Hepatobiliary & pancreatic diseases international : HBPD INTNecrolytic migratory erythema-like eruption and paradoxical psoriasis associated with adalimumab treatment.
The Journal of dermatologyDistinctive detection of insulinoma using [18F]FB(ePEG12)12-exendin-4 PET/CT.
Scientific reportsA germline c.1546dupC MEN1 mutation in an MEN1 family: A case report.
MedicineGlucagonoma with diffuse enlargement of pancreas mimicking autoimmune pancreatitis diagnosed by EUS-guided FNA.
Gastrointestinal endoscopyTreatment of Glucagonoma-Related Necrolytic Migratory Erythema With Peptide Receptor Radionuclide Therapy.
Clinical nuclear medicine[Neuroendorine paraneoplastic syndromes].
Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte GebietePancreatoduodenectomy 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 journal of surgeryPancreatic neuroendocrine neoplasms: Clinicopathological features and pathological staging.
Histology and histopathologyClinical Characteristics and Management of Functional Pancreatic Neuroendocrine Neoplasms: A Single Institution 20-Year Experience with 286 Patients.
International journal of endocrinologyPerspectives on the current pharmacotherapeutic strategies for management of functional neuroendocrine tumor syndromes.
Expert opinion on pharmacotherapyBenign-Appearing Glucagonoma Undergoing Malignant Transformation after a Prolonged Period.
Case reports in oncologyConcomitant Insulinoma and Glucagonoma in Solitary Cystic Pancreatic Lesion in a Young Male Patient.
PancreasGlucagonoma: From skin lesions to the neuroendocrine component (Review).
Experimental and therapeutic medicineThe RNA-binding protein, HuD regulates proglucagon biosynthesis in pancreatic α cells.
Biochemical and biophysical research communicationsNecrolytic Migratory Erythema Associated with a Glucagonoma.
The New England journal of medicineNecrolytic migratory erythema: an important sign of glucagonoma.
Postgraduate medical journalMANIFESTATIONS OF GLUCAGONOMA SYNDROME.
AACE clinical case reportsThe magnesium transporter NIPAL1 is a pancreatic islet-expressed protein that conditionally impacts insulin secretion.
The Journal of biological chemistryPseudoglucagonoma syndrome: Description of an 'Idiopatic' case.
The Australasian journal of dermatologyNecrolytic Acral Erythema: Current Insights.
Clinical, cosmetic and investigational dermatologyNeuropsychiatric symptoms, skin disease, and weight loss: necrolytic migratory erythema and a glucagonoma.
Lancet (London, England)Proof of concept for stereotactic body radiation therapy in the treatment of functional neuroendocrine neoplasms.
Journal of radiosurgery and SBRTA retrospective comparison of robotic versus laparoscopic distal resection and enucleation for potentially benign pancreatic neoplasms.
Surgery today[Facial edema and erythroderma in a 54 year-old woman].
La Revue de medecine interneApplication of Somatostatin, Chemotherapy Combined with TAE in Heterogeneous Glucagonoma Presented with Necrolytic Migratory Erythema.
OncoTargets and therapyOccult insulinoma, glucagonoma and pancreatic endocrine pseudotumour in a patient with multiple endocrine neoplasia type 1.
Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.]Case of autoimmune progesterone dermatitis presenting as necrotic migratory erythema successfully controlled by danazol.
The Journal of dermatologyDiarrhea: a missed D in the 4D glucagonoma syndrome.
Autopsy & case reportsMolecular imaging of a glucagonoma with 18F-FDG PET/CT and 68Ga-DOTATATE PET/CT imaging: A case report and review of the literature.
Radiology case reportsUnremitting chronic skin lesions: a case of delayed diagnosis of glucagonoma.
Journal of community hospital internal medicine perspectivesSpleen-preserving distal pancreatectomy and lymphadenectomy for glucagonoma syndrome: A case report.
MedicineGlucagonoma syndrome with severe erythematous rash: A rare case report.
MedicineGlucagonoma-related necrolytic migratory erythema.
Medicina clinicaSystemic and Autoimmune Diseases.
Clinics in colon and rectal surgeryResolution of necrolytic migratory erythema with somatostatin analogue in a patient diagnosed with pancreatic glucagonoma.
BMJ case reportsGlucagonoma syndrome with atypical necrolytic migratory erythema.
Indian journal of dermatology, venereology and leprologyGlucagonoma with Paraneoplasic Dermatitis: Diagnosis and Management.
Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary TractPARP-14 Promotes Survival of Mammalian α but Not β Pancreatic Cells Following Cytokine Treatment.
Frontiers in endocrinologyClinicopathological characteristics and risk factors for recurrence of well-differentiated pancreatic neuroendocrine tumors after radical surgery: a case-control study.
World journal of surgical oncologyPancreatic Ductal Adenocarcinoma Derived From an Intraductal Papillary Neoplasm With Synchronous Incidental Glucagonoma: A Case Report and Literature Review.
Pancreas[Necrolytic migratory erythema revealing glucagonoma syndrome].
Annales de dermatologie et de venereologieNecrolytic migratory erythema.
CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienneGlucagonoma-associated dilated cardiomyopathy refractory to somatostatin analogue therapy.
Endocrinology, diabetes & metabolism case reportsImaging of Pancreatic-Neuroendocrine Tumours: An Outline of Conventional Radiological Techniques.
Current radiopharmaceuticalsNecrolytic Migratory Erythema: Complete Healing after Surgical Removal of Pancreatic Carcinoma.
Acta dermatovenerologica Croatica : ADCGlucagonoma and Glucagonoma Syndrome: One Center's Experience of Six Cases.
Journal of pancreatic cancerMetabolic effects of glucagon in humans.
Journal of clinical & translational endocrinologyImage Gallery: Necrolytic migratory erythema associated with glucagonoma.
The British journal of dermatologySymptomatic and Radiological Response to 177Lu-DOTATATE for the Treatment of Functioning Pancreatic Neuroendocrine Tumors.
The Journal of clinical endocrinology and metabolismThe impact of failure to achieve symptom control after resection of functional neuroendocrine tumors: An 8-institution study from the US Neuroendocrine Tumor Study Group.
Journal of surgical oncologyGlucagonoma-Associated Necrolytic Migratory Erythema: The Broad Spectrum of the Clinical and Histopathological Findings and Clues to the Diagnosis.
The American Journal of dermatopathologyPeptidomic analysis of endogenous plasma peptides from patients with pancreatic neuroendocrine tumours.
Rapid communications in mass spectrometry : RCMGlucagonoma With Necrolytic Migratory Erythema: Metabolic Profile and Detection of Biallelic Inactivation of DAXX Gene.
The Journal of clinical endocrinology and metabolism[Surgical strategies for small sporadic neuroendocrine pancreatic tumors].
Der Chirurg; Zeitschrift fur alle Gebiete der operativen MedizenManagement of functional neuroendocrine tumors of the pancreas.
Gland surgeryA Persistent Migrating Rash.
GastroenterologyErythema as a Visual Surrogate Marker of Glucagonoma.
Internal medicine (Tokyo, Japan)Supportive therapy in gastroenteropancreatic neuroendocrine tumors: Often forgotten but important.
Reviews in endocrine & metabolic disordersA review of cutaneous manifestations within glucagonoma syndrome: necrolytic migratory erythema.
International journal of dermatologyImaging features of malignant abdominal neuroendocrine tumors with rare presentation.
Clinical imagingGlucagonoma and the glucagonoma syndrome.
Oncology lettersA 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.
Clinical nuclear medicineNormoglycemic glucagonoma syndrome associated with necrolytic migratory erythema.
Journal of the European Academy of Dermatology and Venereology : JEADVMalignant transformation of glucagonoma with SPECT/CT In-111 OctreoScan features: A case report.
MedicinePsoriasiform lesions: Uncommon presentation of glucagonoma.
Gastroenterologia y hepatologiaGlucagonoma syndrome with serous oligocystic adenoma: A rare case report.
MedicineEmergency pancreatic resection for glucagonoma associated with severe necrolytic migratory erythema.
ANZ journal of surgeryNecrolytic Migratory Erythema: A Forgotten Paraneoplastic Condition.
Journal of cutaneous medicine and surgeryMetastatic Hepatoid Carcinoma of the Pancreas: First Description of Treatment With Capecitabine and Temozolomide.
The American journal of the medical sciences[Diabetes mellitus secondary to an endocrine pathology : when to think about it ?].
Revue medicale suisseNecrolytic Acral Erythema in Seronegative Hepatitis C.
Case reports in dermatologyChallenging Differential Diagnosis of Hypergastremia and Hyperglucagonemia with Chronic Renal Failure: Report of a Case with Multiple Endocrine Neoplasia Type 1.
Internal medicine (Tokyo, Japan)Necrolytic migratory erythema associated with glucagonoma treated successfully with cyclosporine.
Dermatologic therapyManagement of the hormonal syndrome of neuroendocrine tumors.
Archives of medical science : AMSGlucagonoma-Associated Rash.
The New England journal of medicineENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors: Pre- and Perioperative Therapy in Patients with Neuroendocrine Tumors.
NeuroendocrinologyB-mode and contrast-enhancement characteristics of small nonincidental neuroendocrine pancreatic tumors.
Endoscopic ultrasoundFoxa2, a novel protein partner of the tumour suppressor menin, is deregulated in mouse and human MEN1 glucagonomas.
The Journal of pathologyGlucagonoma syndrome: report of one case.
Translational gastroenterology and hepatologyGlucagon and Amino Acids Are Linked in a Mutual Feedback Cycle: The Liver-α-Cell Axis.
DiabetesPancreatic α-cell hyperplasia and hyperglucagonemia due to a glucagon receptor splice mutation.
Endocrinology, diabetes & metabolism case reportsImaging of secretory tumors of the gastrointestinal tract.
Abdominal radiology (New York)Operation for insulinomas in multiple endocrine neoplasia type 1: When pancreatoduodenectomy is appropriate.
SurgeryCharacterization of pancreatic glucagon-producing tumors and pituitary gland tumors in transgenic mice overexpressing MYCN in hGFAP-positive cells.
OncotargetThe biology of glucagon and the consequences of hyperglucagonemia.
Biomarkers in medicineNecrolytic migratory erythema and pancreatic glucagonoma.
Biomedica : revista del Instituto Nacional de SaludNecrolytic migratory erythema: A diagnostic clue in glucagonoma syndrome.
Medicina clinicaMedical management of secretory syndromes related to gastroenteropancreatic neuroendocrine tumours.
Endocrine-related cancerPrognostic factors in resected pancreatic neuroendocrine tumours: Experience in 95 patients.
Cirugia espanolaBone turnover response is linked to both acute and established metabolic changes in ultra-marathon runners.
EndocrineGlucagonoma syndrome: a review and update on treatment.
Journal of the European Academy of Dermatology and Venereology : JEADVNecrolytic migratory erythema with recalcitrant dermatitis as the only presenting symptom.
CutisPancreatic neuroendocrine tumors: contemporary diagnosis and management.
Hospital practice (1995)Over-expression of Slc30a8/ZnT8 selectively in the mouse α cell impairs glucagon release and responses to hypoglycemia.
Nutrition & metabolismClinical Presentation and Diagnosis of Pancreatic Neuroendocrine Tumors.
Surgical oncology clinics of North AmericaGlucagonoma and Glucagonoma Syndrome: A Case Report with Review of Recent Advances in Management.
Case reports in surgeryA short history of neuroendocrine tumours and their peptide hormones.
Best practice & research. Clinical endocrinology & metabolismDo glucagonomas always produce glucagon?
Bosnian journal of basic medical sciencesNecrolytic migratory erythema associated with fatty liver disease and the psuedoglucagonoma syndrome.
Dermatology online journalDifferent Hormonal Expression Patterns Between Primary Pancreatic Neuroendocrine Tumors and Metastatic Sites.
PancreasDelayed diagnosis of glucagonoma syndrome: a case report.
International journal of dermatologyMetastatic VIPoma presenting as an ovarian mass.
International journal of surgery case reportsIatrogenic Necrolytic Migratory Erythema in an Infant with Congenital Hyperinsulinism.
Pediatric dermatologyGlucagonoma syndrome: A case report.
Oncology lettersPaediatric necrolytic migratory erythema as a presenting sign of glucagonoma syndrome.
The British journal of dermatologyRapid Clearance of Necrolytic Migratory Erythema Following Intravenous Administration of Amino Acids.
JAMA dermatology[A case of MEN type I which complicated with glucagonoma after surgical treatment for gastrinoma in the 36th year].
Nihon Naika Gakkai zasshi. The Journal of the Japanese Society of Internal MedicineProgranulin Stimulates Proliferation of Mouse Pancreatic Islet Cells and Is Overexpressed in the Endocrine Pancreatic Tissue of an MEN1 Mouse Model.
PancreasProposed Radiation Induced Hormone Crisis in a Patient with Glucagonoma.
Journal of gastrointestinal cancerA Novel Missense Mutation of the MEN1 Gene in a Patient with Multiple Endocrine Neoplasia Type 1 with Glucagonoma and Obesity.
Internal medicine (Tokyo, Japan)Biochemical Testing in Patients with Neuroendocrine Tumors.
Frontiers of hormone researchGlucagonoma syndrome associated with necrolytic migratory erythema.
Revista da Associacao Medica Brasileira (1992)Glucagonoma Pancreatic Neuroendocrine Tumor Treated With 177Lu DOTATATE Induction and Maintenance Peptide Receptor Radionuclide Therapy.
Clinical nuclear medicineRapid improvement of glucagonoma-related necrolytic migratory erythema with octreotide.
Clinical journal of gastroenterology[Neuroendocrine well-differentiated pancreatic tumors].
La Revue du praticienGlucagon receptor gene mutations with hyperglucagonemia but without the glucagonoma syndrome.
World journal of gastrointestinal surgeryImaging features of glucagonoma syndrome: A case report and review of the literature.
Oncology lettersDiagnosis of functioning pancreaticoduodenal neuroendocrine tumors.
Journal of hepato-biliary-pancreatic sciencesReversal of dilated cardiomyopathy after glucagonoma excision.
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Glucagonoma of the pancreas: diagnostic approach and therapeutic algorithm for a rare nosological entity.
- Glucagonoma Syndrome With Necrolytic Migratory Erythema.
- Shift in secretory profile of a pNET: from indolent glucagonoma to aggressive insulinoma - a case report.
- Necrolytic Migratory Erythema as the First Manifestation of Functional Conversion in a Glucagonoma: A Case Report.
- [The 514th case: urinary stone excretion, elevated blood glucose, pancreatic mass,and co-secretion of multiple hormones].
- [Necrolytic migratory erythema as the key to the diagnosis of pancreatic neoplasms].
- Somatostatin Receptor PET/CT in a Glucagonoma Presenting With Necrolytic Migratory Erythema and Normoglucagonemia: A Diagnostic Dilemma.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:97280(Orphanet)
- MONDO:0019959(MONDO)
- GARD:2496(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q770843(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
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