Raras
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Glucagonoma
ORPHA:97280CID-10 · D37.7CID-11 · 2C10.1DOENÇA RARA

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.

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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.

Pesquisas ativas
2 ensaios
34 total registrados no ClinicalTrials.gov
Publicações científicas
889 artigos
Último publicado: 2026

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
<1 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Europe
Início
Adult
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: D37.7
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🫃
Digestivo
13 sintomas
📏
Crescimento
8 sintomas
🧬
Pele e cabelo
3 sintomas
🩸
Sangue
2 sintomas
🫘
Rins
1 sintomas
🧠
Neurológico
1 sintomas

+ 14 sintomas em outras categorias

Características mais comuns

90%prev.
Neoplasia do pâncreas
Muito frequente (99-80%)
55%prev.
Anorexia
Frequente (79-30%)
55%prev.
Estomatite
Frequente (79-30%)
55%prev.
Constipação
Frequente (79-30%)
55%prev.
Fadiga crônica
Frequente (79-30%)
55%prev.
Apetite pobre
Frequente (79-30%)
42sintomas
Muito frequente (1)
Frequente (18)
Ocasional (12)
Muito raro (11)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 42 características clínicas mais associadas, ordenadas por frequência.

Neoplasia do pâncreasNeoplasm of the pancreas
Muito frequente (99-80%)90%
Anorexia
Frequente (79-30%)55%
EstomatiteStomatitis
Frequente (79-30%)55%
ConstipaçãoConstipation
Frequente (79-30%)55%
Fadiga crônicaChronic fatigue
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico889PubMed
Últimos 10 anos184publicações
Pico201624 papers
Linha do tempo
2026Hoje · 2026🧪 1996Primeiro ensaio clínico📈 2016Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

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

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
2Fase 211
1Fase 15
·Pré-clínico1
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 17 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Glucagonoma

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

34 ensaios clínicos encontrados, 2 ativos.

Distribuição por fase
NCT01824875 · Temozolomide With or Without Capecitabine in Treating Patien…Ativo
PHASE2
NCT03583528 · DOTATOC PET/CT for Imaging NET PatientsAtivo
NCT00655655 · Everolimus and Vatalanib in Treating Patients With Advanced …Concluído
PHASE1
NCT01525082 · Capecitabine, Temozolomide, and Bevacizumab for Metastatic o…Concluído
PHASE2
NCT02031536 · Everolimus in Patients With Pancreatic Neuroendocrine Tumors…Encerrado
PHASE2
NCT03147768 · Laser Tissue Welding - Distal Pancreatectomy Sealing StudyConcluído
PHASE1
NCT02259725 · Regorafenib in Treating Patients With Advanced or Metastatic…Concluído
PHASE2
NCT00454363 · Pazopanib Hydrochloride in Treating Patients With Advanced N…Concluído
PHASE2
NCT01010126 · Temsirolimus and Bevacizumab in Treating Patients With Advan…Concluído
PHASE2
NCT02831179 · Veliparib, Capecitabine, and Temozolomide in Patients With A…Cancelado
PHASE1
NCT02108782 · Dovitinib Lactate in Treating Patients With Pancreatic Neuro…Cancelado
PHASE2
NCT02273752 · Pharmacokinetically Guided Everolimus in Patients With Breas…Encerrado
PHASE2
NCT01204476 · Cixutumumab, Everolimus, and Octreotide Acetate in Treating …Concluído
PHASE1
NCT00131911 · Sorafenib Tosylate in Treating Patients With Progressive Met…Concluído
PHASE2
NCT00084461 · Romidepsin in Treating Patients With Locally Advanced or Met…Encerrado
PHASE2
NCT00075439 · Gefitinib in Treating Patients With Progressive Metastatic N…Concluído
PHASE2
NCT00004074 · Interleukin-12 and Trastuzumab in Treating Patients With Can…Concluído
PHASE1
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

🥉Melhor nível de evidência: Relato de caso
Timeline de publicações
174 papers (10 anos)
#1

Glucagonoma of the pancreas: diagnostic approach and therapeutic algorithm for a rare nosological entity.

Annals of gastroenterology2026

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.

#2

Glucagonoma Syndrome With Necrolytic Migratory Erythema.

Cureus2025 Dec

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.

#3

Shift in secretory profile of a pNET: from indolent glucagonoma to aggressive insulinoma - a case report.

Frontiers in endocrinology2025

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.

#4

Necrolytic Migratory Erythema as the First Manifestation of Functional Conversion in a Glucagonoma: A Case Report.

Clinical case reports2025 Dec

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.

#5

[The 514th case: urinary stone excretion, elevated blood glucose, pancreatic mass,and co-secretion of multiple hormones].

Zhonghua nei ke za zhi2025 Dec 01

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

Ver todas no PubMed

📚 EuropePMC504 artigos no totalmostrando 179

2026

Glucagonoma of the pancreas: diagnostic approach and therapeutic algorithm for a rare nosological entity.

Annals of gastroenterology
2025

Glucagonoma Syndrome With Necrolytic Migratory Erythema.

Cureus
2025

Shift in secretory profile of a pNET: from indolent glucagonoma to aggressive insulinoma - a case report.

Frontiers in endocrinology
2025

Necrolytic Migratory Erythema as the First Manifestation of Functional Conversion in a Glucagonoma: A Case Report.

Clinical case reports
2025

[The 514th case: urinary stone excretion, elevated blood glucose, pancreatic mass,and co-secretion of multiple hormones].

Zhonghua nei ke za zhi
2025

Study on Autophagy Death of Alpha TC1 Clone 6 (αTC1-6) Cells Induced by Trametenolic Acid Through PI3K/AKT Pathway.

Current issues in molecular biology
2025

Diagnosis and Management of Functional Pancreatic Neuroendocrine Tumors in Children-A Systematic Review.

Diagnostics (Basel, Switzerland)
2025

Diagnostic characteristics, treatment outcomes, and prognostic factors in glucagonomas.

Endocrine oncology (Bristol, England)
2025

A unique case of glucagonoma with atypical necrolytic migratory erythema.

JAAD case reports
2025

Premortem diagnosis of a pancreatic neuroendocrine tumour (glucagonoma) in a captive North American river otter (Lontra canadensis).

Journal of comparative pathology
2025

Nutritional Management of Functioning GEP-NENs.

Nutrients
2025

A Case Report of Necrolytic Migratory Erythema Associated with Glucagonoma.

Clinical, cosmetic and investigational dermatology
2025

Glucagonoma and Glucagonoma Syndrome: An Updated Review.

Clinical endocrinology
2025

Necrolytic migratory erythema with a pancreatic glucagonoma.

The British journal of dermatology
2025

Familial Mahvash disease with metastatic pancreatic NET and MEN1 mutations.

Endocrine-related cancer
2025

Functioning neuroendocrine tumors (NET): Minimum requirements for a NET specialist.

Cancer treatment reviews
2025

Clinical Relevance of ATRX/DAXX Gene Mutations and ALT in Functioning Pancreatic Neuroendocrine Tumors.

Endocrine pathology
2024

Case report: Anesthesia management for surgical treatment of glucagonoma with symptom of characterized necrolytic migratory erythema.

Frontiers in oncology
2024

Necrolytic migratory erythema: a fortuitous diagnosis.

BMJ case reports
2024

Glucagon-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 pathology
2024

Non-functional alpha-cell hyperplasia with glucagon-producing NET: a case report.

Frontiers in endocrinology
2024

Manifestations of Endocrine Disease in the Lower Extremities: Beyond the Diabetic Foot.

The international journal of lower extremity wounds
2024

Case report: Surgical treatment and literature review of a recurrent case of glucagonoma.

Frontiers in oncology
2024

A nuclear beacon of hope: an advanced, metastatic glucagonoma treated with [177Lu]Lu-DOTA-TATE.

Nuclear medicine review. Central &amp; Eastern Europe
2024

Management of functional neuroendocrine tumors.

Current problems in cancer
2024

Necrolytic migratory erythema caused by pancreatic hyperglycemia with emphasis on therapeutic and prognosis: A case report.

World journal of clinical cases
2024

Characteristics, therapy, and outcome of rare functioning pancreatic neuroendocrine neoplasms.

Scientific reports
2024

[Glucagonoma - a rare neuroendocrine tumor (NET) that typically originates in the pancreas].

Lakartidningen
2024

Advances in basic research on glucagon and alpha cells.

Diabetology international
2024

Well-differentiated G1 and G2 pancreatic neuroendocrine tumors: a meta-analysis of published expanded DNA sequencing data.

Frontiers in endocrinology
2024

Pseudoglucagonoma Syndrome Following Frey's Surgery: A Case Report of a Rare Presentation.

Cureus
2024

Genetic disorders and insulinoma/glucagonoma.

Endocrine-related cancer
2024

Diagnosis and comprehensive treatment of a glucagonoma in a patient with residual intrahepatic metastases postoperatively: A case report and literature review.

Oncology letters
2024

A novel likely pathogenetic variant p.(Cys235Arg) of the MEN1 gene in multiple endocrine neoplasia type 1 with multifocal glucagonomas.

Journal of endocrinological investigation
2023

Characteristics and treatment options of glucagonomas: a national study from the French Group of Endocrine Tumors and ENDOCAN-RENATEN network.

European journal of endocrinology
2023

Hepatic Glucagonoma in a Post-bariatric Female Patient: A Case Report.

Cureus
2023

Glucagonoma-induced Dilated Cardiomyopathy in a Young Woman.

JCEM case reports
2023

Synchronous Insulinoma and Glucagonoma: A Review of the Literature.

In vivo (Athens, Greece)
2023

Practical therapeutic approach in the management of diabetes mellitus secondary to Cushing's syndrome, acromegaly and neuroendocrine tumours.

Frontiers in endocrinology
2023

Diabetes Mellitus Secondary to Endocrine Diseases: An Update of Diagnostic and Treatment Particularities.

International journal of molecular sciences
2023

European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes.

Journal of neuroendocrinology
2023

Emerging therapies for advanced insulinomas and glucagonomas.

Endocrine-related cancer
2023

One hundred years after the discovery of insulin and glucagon: the history of tumors and hyperplasias that hypersecrete these hormones.

Endocrine-related cancer
2023

Glucagon cell hyperplasia and neoplasia: a recently recognized endocrine receptor disease.

Endocrine-related cancer
2022

Analysis of Clinical Characteristics and Survival in Patients With Functional Neuroendocrine Tumors of Gastrointestinal Origin.

Pancreas
2023

A case of multiple glucagonomas with no clinical manifestations of excess glucagon despite hyperglucagonemia.

DEN open
2022

Recurrent pruritic polymorphic lesions associated with weight loss.

JAAD case reports
2023

An Insight on Functioning Pancreatic Neuroendocrine Neoplasms.

Biomedicines
2022

Circulating Neuroendocrine Tumor Biomarkers: Past, Present and Future.

Journal of clinical medicine
2022

Glucagonoma Syndrome: A Rare Paraneoplastic Disorder due to Neuroendocrine Tumor of the Pancreas.

Journal of the College of Physicians and Surgeons--Pakistan : JCPSP
2022

Skin manifestations of neuroendocrine neoplasms: review of the literature.

Postepy dermatologii i alergologii
2022

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

Revista de la Facultad de Ciencias Medicas (Cordoba, Argentina)
2022

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

Scientific reports
2022

Case report of a recurrent resected glucagonoma.

Annals of medicine and surgery (2012)
2022

A Glucagonoma Presenting as Cerebral Vein Thrombosis and Diabetes.

Case reports in endocrinology
2022

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

Diagnostics (Basel, Switzerland)
2021

Case of delayed diagnosis of necrolytic migratory erythema.

Clinical case reports
2021

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

Nederlands tijdschrift voor geneeskunde
2021

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

The neurologist
2021

Glucagonoma syndrome with necrolytic migratory erythema as initial manifestation.

Hepatobiliary &amp; pancreatic diseases international : HBPD INT
2021

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

The Journal of dermatology
2021

Distinctive detection of insulinoma using [18F]FB(ePEG12)12-exendin-4 PET/CT.

Scientific reports
2021

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

Medicine
2021

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

Gastrointestinal endoscopy
2021

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

Clinical nuclear medicine
2021

[Neuroendorine paraneoplastic syndromes].

Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete
2021

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 journal of surgery
2021

Pancreatic neuroendocrine neoplasms: Clinicopathological features and pathological staging.

Histology and histopathology
2020

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

International journal of endocrinology
2021

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

Expert opinion on pharmacotherapy
2020

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

Case reports in oncology
2020

Concomitant Insulinoma and Glucagonoma in Solitary Cystic Pancreatic Lesion in a Young Male Patient.

Pancreas
2020

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

Experimental and therapeutic medicine
2020

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

Biochemical and biophysical research communications
2020

Necrolytic Migratory Erythema Associated with a Glucagonoma.

The New England journal of medicine
2021

Necrolytic migratory erythema: an important sign of glucagonoma.

Postgraduate medical journal
2020

MANIFESTATIONS OF GLUCAGONOMA SYNDROME.

AACE clinical case reports
2020

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

The Journal of biological chemistry
2020

Pseudoglucagonoma syndrome: Description of an 'Idiopatic' case.

The Australasian journal of dermatology
2020

Necrolytic Acral Erythema: Current Insights.

Clinical, cosmetic and investigational dermatology
2020

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

Lancet (London, England)
2020

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

Journal of radiosurgery and SBRT
2020

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

Surgery today
2020

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

La Revue de medecine interne
2019

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

OncoTargets and therapy
2020

Occult 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.]
2020

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

The Journal of dermatology
2019

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

Autopsy &amp; case reports
2020

Molecular 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 reports
2019

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

Journal of community hospital internal medicine perspectives
2019

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

Medicine
2019

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

Medicine
2020

Glucagonoma-related necrolytic migratory erythema.

Medicina clinica
2019

Systemic and Autoimmune Diseases.

Clinics in colon and rectal surgery
2019

Resolution of necrolytic migratory erythema with somatostatin analogue in a patient diagnosed with pancreatic glucagonoma.

BMJ case reports
2021

Glucagonoma syndrome with atypical necrolytic migratory erythema.

Indian journal of dermatology, venereology and leprology
2020

Glucagonoma with Paraneoplasic Dermatitis: Diagnosis and Management.

Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract
2019

PARP-14 Promotes Survival of Mammalian α but Not β Pancreatic Cells Following Cytokine Treatment.

Frontiers in endocrinology
2019

Clinicopathological characteristics and risk factors for recurrence of well-differentiated pancreatic neuroendocrine tumors after radical surgery: a case-control study.

World journal of surgical oncology
2019

Pancreatic Ductal Adenocarcinoma Derived From an Intraductal Papillary Neoplasm With Synchronous Incidental Glucagonoma: A Case Report and Literature Review.

Pancreas
2019

[Necrolytic migratory erythema revealing glucagonoma syndrome].

Annales de dermatologie et de venereologie
2019

Necrolytic migratory erythema.

CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienne
2019

Glucagonoma-associated dilated cardiomyopathy refractory to somatostatin analogue therapy.

Endocrinology, diabetes &amp; metabolism case reports
2019

Imaging of Pancreatic-Neuroendocrine Tumours: An Outline of Conventional Radiological Techniques.

Current radiopharmaceuticals
2018

Necrolytic Migratory Erythema: Complete Healing after Surgical Removal of Pancreatic Carcinoma.

Acta dermatovenerologica Croatica : ADC
2018

Glucagonoma and Glucagonoma Syndrome: One Center's Experience of Six Cases.

Journal of pancreatic cancer
2019

Metabolic effects of glucagon in humans.

Journal of clinical &amp; translational endocrinology
2019

Image Gallery: Necrolytic migratory erythema associated with glucagonoma.

The British journal of dermatology
2019

Symptomatic and Radiological Response to 177Lu-DOTATATE for the Treatment of Functioning Pancreatic Neuroendocrine Tumors.

The Journal of clinical endocrinology and metabolism
2019

The 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 oncology
2019

Glucagonoma-Associated Necrolytic Migratory Erythema: The Broad Spectrum of the Clinical and Histopathological Findings and Clues to the Diagnosis.

The American Journal of dermatopathology
2018

Peptidomic analysis of endogenous plasma peptides from patients with pancreatic neuroendocrine tumours.

Rapid communications in mass spectrometry : RCM
2018

Glucagonoma With Necrolytic Migratory Erythema: Metabolic Profile and Detection of Biallelic Inactivation of DAXX Gene.

The Journal of clinical endocrinology and metabolism
2018

[Surgical strategies for small sporadic neuroendocrine pancreatic tumors].

Der Chirurg; Zeitschrift fur alle Gebiete der operativen Medizen
2018

Management of functional neuroendocrine tumors of the pancreas.

Gland surgery
2018

A Persistent Migrating Rash.

Gastroenterology
2018

Erythema as a Visual Surrogate Marker of Glucagonoma.

Internal medicine (Tokyo, Japan)
2018

Supportive therapy in gastroenteropancreatic neuroendocrine tumors: Often forgotten but important.

Reviews in endocrine &amp; metabolic disorders
2018

A review of cutaneous manifestations within glucagonoma syndrome: necrolytic migratory erythema.

International journal of dermatology
2018

Imaging features of malignant abdominal neuroendocrine tumors with rare presentation.

Clinical imaging
2018

Glucagonoma and the glucagonoma syndrome.

Oncology letters
2018

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.

Clinical nuclear medicine
2018

Normoglycemic glucagonoma syndrome associated with necrolytic migratory erythema.

Journal of the European Academy of Dermatology and Venereology : JEADV
2017

Malignant transformation of glucagonoma with SPECT/CT In-111 OctreoScan features: A case report.

Medicine
2018

Psoriasiform lesions: Uncommon presentation of glucagonoma.

Gastroenterologia y hepatologia
2017

Glucagonoma syndrome with serous oligocystic adenoma: A rare case report.

Medicine
2019

Emergency pancreatic resection for glucagonoma associated with severe necrolytic migratory erythema.

ANZ journal of surgery
2017

Necrolytic Migratory Erythema: A Forgotten Paraneoplastic Condition.

Journal of cutaneous medicine and surgery
2017

Metastatic Hepatoid Carcinoma of the Pancreas: First Description of Treatment With Capecitabine and Temozolomide.

The American journal of the medical sciences
2017

[Diabetes mellitus secondary to an endocrine pathology : when to think about it ?].

Revue medicale suisse
2017

Necrolytic Acral Erythema in Seronegative Hepatitis C.

Case reports in dermatology
2017

Challenging Differential Diagnosis of Hypergastremia and Hyperglucagonemia with Chronic Renal Failure: Report of a Case with Multiple Endocrine Neoplasia Type 1.

Internal medicine (Tokyo, Japan)
2017

Necrolytic migratory erythema associated with glucagonoma treated successfully with cyclosporine.

Dermatologic therapy
2017

Management of the hormonal syndrome of neuroendocrine tumors.

Archives of medical science : AMS
2017

Glucagonoma-Associated Rash.

The New England journal of medicine
2017

ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors: Pre- and Perioperative Therapy in Patients with Neuroendocrine Tumors.

Neuroendocrinology
2017

B-mode and contrast-enhancement characteristics of small nonincidental neuroendocrine pancreatic tumors.

Endoscopic ultrasound
2017

Foxa2, a novel protein partner of the tumour suppressor menin, is deregulated in mouse and human MEN1 glucagonomas.

The Journal of pathology
2016

Glucagonoma syndrome: report of one case.

Translational gastroenterology and hepatology
2017

Glucagon and Amino Acids Are Linked in a Mutual Feedback Cycle: The Liver-α-Cell Axis.

Diabetes
2016

Pancreatic α-cell hyperplasia and hyperglucagonemia due to a glucagon receptor splice mutation.

Endocrinology, diabetes &amp; metabolism case reports
2017

Imaging of secretory tumors of the gastrointestinal tract.

Abdominal radiology (New York)
2017

Operation for insulinomas in multiple endocrine neoplasia type 1: When pancreatoduodenectomy is appropriate.

Surgery
2016

Characterization of pancreatic glucagon-producing tumors and pituitary gland tumors in transgenic mice overexpressing MYCN in hGFAP-positive cells.

Oncotarget
2016

The biology of glucagon and the consequences of hyperglucagonemia.

Biomarkers in medicine
2016

Necrolytic migratory erythema and pancreatic glucagonoma.

Biomedica : revista del Instituto Nacional de Salud
2017

Necrolytic migratory erythema: A diagnostic clue in glucagonoma syndrome.

Medicina clinica
2016

Medical management of secretory syndromes related to gastroenteropancreatic neuroendocrine tumours.

Endocrine-related cancer
2016

Prognostic factors in resected pancreatic neuroendocrine tumours: Experience in 95 patients.

Cirugia espanola
2017

Bone turnover response is linked to both acute and established metabolic changes in ultra-marathon runners.

Endocrine
2016

Glucagonoma syndrome: a review and update on treatment.

Journal of the European Academy of Dermatology and Venereology : JEADV
2016

Necrolytic migratory erythema with recalcitrant dermatitis as the only presenting symptom.

Cutis
2016

Pancreatic neuroendocrine tumors: contemporary diagnosis and management.

Hospital practice (1995)
2016

Over-expression of Slc30a8/ZnT8 selectively in the mouse α cell impairs glucagon release and responses to hypoglycemia.

Nutrition &amp; metabolism
2016

Clinical Presentation and Diagnosis of Pancreatic Neuroendocrine Tumors.

Surgical oncology clinics of North America
2016

Glucagonoma and Glucagonoma Syndrome: A Case Report with Review of Recent Advances in Management.

Case reports in surgery
2016

A short history of neuroendocrine tumours and their peptide hormones.

Best practice &amp; research. Clinical endocrinology &amp; metabolism
2016

Do glucagonomas always produce glucagon?

Bosnian journal of basic medical sciences
2016

Necrolytic migratory erythema associated with fatty liver disease and the psuedoglucagonoma syndrome.

Dermatology online journal
2016

Different Hormonal Expression Patterns Between Primary Pancreatic Neuroendocrine Tumors and Metastatic Sites.

Pancreas
2016

Delayed diagnosis of glucagonoma syndrome: a case report.

International journal of dermatology
2015

Metastatic VIPoma presenting as an ovarian mass.

International journal of surgery case reports
2016

Iatrogenic Necrolytic Migratory Erythema in an Infant with Congenital Hyperinsulinism.

Pediatric dermatology
2015

Glucagonoma syndrome: A case report.

Oncology letters
2016

Paediatric necrolytic migratory erythema as a presenting sign of glucagonoma syndrome.

The British journal of dermatology
2016

Rapid Clearance of Necrolytic Migratory Erythema Following Intravenous Administration of Amino Acids.

JAMA dermatology
2015

[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 Medicine
2016

Progranulin Stimulates Proliferation of Mouse Pancreatic Islet Cells and Is Overexpressed in the Endocrine Pancreatic Tissue of an MEN1 Mouse Model.

Pancreas
2016

Proposed Radiation Induced Hormone Crisis in a Patient with Glucagonoma.

Journal of gastrointestinal cancer
2015

A Novel Missense Mutation of the MEN1 Gene in a Patient with Multiple Endocrine Neoplasia Type 1 with Glucagonoma and Obesity.

Internal medicine (Tokyo, Japan)
2015

Biochemical Testing in Patients with Neuroendocrine Tumors.

Frontiers of hormone research
2015

Glucagonoma syndrome associated with necrolytic migratory erythema.

Revista da Associacao Medica Brasileira (1992)
2015

Glucagonoma Pancreatic Neuroendocrine Tumor Treated With 177Lu DOTATATE Induction and Maintenance Peptide Receptor Radionuclide Therapy.

Clinical nuclear medicine
2014

Rapid improvement of glucagonoma-related necrolytic migratory erythema with octreotide.

Clinical journal of gastroenterology
2015

[Neuroendocrine well-differentiated pancreatic tumors].

La Revue du praticien
2015

Glucagon receptor gene mutations with hyperglucagonemia but without the glucagonoma syndrome.

World journal of gastrointestinal surgery
2015

Imaging features of glucagonoma syndrome: A case report and review of the literature.

Oncology letters
2015

Diagnosis of functioning pancreaticoduodenal neuroendocrine tumors.

Journal of hepato-biliary-pancreatic sciences
2015

Reversal of dilated cardiomyopathy after glucagonoma excision.

Hormones (Athens, Greece)
Ver todos os 504 no EuropePMC

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Doenças relacionadas

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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.

  1. Glucagonoma of the pancreas: diagnostic approach and therapeutic algorithm for a rare nosological entity.
    Annals of gastroenterology· 2026· PMID 41868867mais citado
  2. Glucagonoma Syndrome With Necrolytic Migratory Erythema.
    Cureus· 2025· PMID 41567922mais citado
  3. Shift in secretory profile of a pNET: from indolent glucagonoma to aggressive insulinoma - a case report.
    Frontiers in endocrinology· 2025· PMID 41561059mais citado
  4. Necrolytic Migratory Erythema as the First Manifestation of Functional Conversion in a Glucagonoma: A Case Report.
    Clinical case reports· 2025· PMID 41403981mais citado
  5. [The 514th case: urinary stone excretion, elevated blood glucose, pancreatic mass,and co-secretion of multiple hormones].
    Zhonghua nei ke za zhi· 2025· PMID 41338562mais citado
  6. [Necrolytic migratory erythema as the key to the diagnosis of pancreatic neoplasms].
    Medicina (B Aires)· 2026· PMID 41961613recente
  7. Somatostatin Receptor PET/CT in a Glucagonoma Presenting With Necrolytic Migratory Erythema and Normoglucagonemia: A Diagnostic Dilemma.
    Clin Nucl Med· 2025· PMID 41937553recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:97280(Orphanet)
  2. MONDO:0019959(MONDO)
  3. GARD:2496(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Artigo Wikipedia(Wikipedia)
  6. 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.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Glucagonoma
Compêndio · Raras BR

Glucagonoma

ORPHA:97280 · MONDO:0019959
Prevalência
<1 / 1 000 000
Herança
Not applicable
CID-10
D37.7 · Neoplasia de comportamento incerto ou desconhecido de outros órgãos digestivos
CID-11
Ensaios
2 ativos
Início
Adult
Prevalência
0.0 (Europe)
MedGen
UMLS
C0017689
EuropePMC
Wikidata
Wikipedia
Papers 10a
Evidência
🥉 Relato de caso
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