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VIPoma
ORPHA:97282CID-10 · D37.7CID-11 · 2C10.1DOENÇA RARA

VIPoma é um tipo muito raro de tumor neuroendócrino do pâncreas que produz o polipeptídeo intestinal vasoativo (VIP). Isso causa sintomas como diarreia aquosa, baixos níveis de potássio no sangue e ausência ou diminuição do ácido do estômago, condição conhecida como síndrome WDHA.

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Introdução

O que você precisa saber de cara

📋

VIPoma é um tipo muito raro de tumor neuroendócrino do pâncreas que produz o polipeptídeo intestinal vasoativo (VIP). Isso causa sintomas como diarreia aquosa, baixos níveis de potássio no sangue e ausência ou diminuição do ácido do estômago, condição conhecida como síndrome WDHA.

Pesquisas ativas
1 ensaio
23 total registrados no ClinicalTrials.gov
Publicações científicas
370 artigos
Último publicado: 2000

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
All ages
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: D37.7
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Entender a doença

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Sinais e sintomas

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

Partes do corpo afetadas

🫃
Digestivo
11 sintomas
📏
Crescimento
9 sintomas
💪
Músculos
3 sintomas
🧠
Neurológico
1 sintomas
🫘
Rins
1 sintomas
🧬
Pele e cabelo
1 sintomas

+ 14 sintomas em outras categorias

Características mais comuns

90%prev.
Diarreia secretora
Muito frequente (99-80%)
90%prev.
Neoplasia do pâncreas
Muito frequente (99-80%)
90%prev.
Hipocalemia
Muito frequente (99-80%)
55%prev.
Espasmo muscular
Frequente (79-30%)
55%prev.
Eritema
Frequente (79-30%)
55%prev.
Dor abdominal episódica
Frequente (79-30%)
41sintomas
Muito frequente (3)
Frequente (15)
Ocasional (6)
Muito raro (17)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 41 características clínicas mais associadas, ordenadas por frequência.

Diarreia secretoraSecretory diarrhea
Muito frequente (99-80%)90%
Neoplasia do pâncreasNeoplasm of the pancreas
Muito frequente (99-80%)90%
HipocalemiaHypokalemia
Muito frequente (99-80%)90%
Espasmo muscularMuscle spasm
Frequente (79-30%)55%
EritemaErythema
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico370PubMed
Últimos 10 anos120publicações
Pico201915 papers
Linha do tempo
2026Hoje · 2026🧪 1996Primeiro ensaio clínico📈 2019Ano 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 22
1Fase 11
·Pré-clínico1
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 4 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — VIPoma

🗺️

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

23 ensaios clínicos encontrados, 1 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
111 papers (10 anos)
#1

Elevated vasoactive intestinal peptide concentrations poorly predict VIPoma.

Endocrine-related cancer2026 Jan 01

VIPoma is a rare neuroendocrine tumor (NET) that is challenging to diagnose. While VIP concentrations are elevated in VIPoma, the optimal threshold for diagnostic purposes is not well defined. We aimed to study this in a single-institution population. We obtained results from vasoactive intestinal peptide (VIP) tests from 2011 to 2023 and reviewed the medical record of patients who had concentrations greater than our established assay-specific reference limit of 75 pg/mL. We compared plasma VIP concentrations between patient cohorts with and without VIPoma and determined the optimal threshold for VIP concentrations to predict a VIPoma in this population. Seventy-six patients met the selection criteria. Of these, nine cases of VIPoma were diagnosed. All patients had chronic diarrhea, and five patients had a previous diagnosis of a pancreatic neuroendocrine tumor (PNET). VIP concentrations drawn for acute/episodic diarrhea or flushing/diaphoresis did not lead to a diagnosis of a VIPoma. Mean VIP concentration was increased in patients with a VIPoma relative to those without, but the difference was not statistically significant (508 pg/mL vs 223 pg/mL, P = 0.31). Using the threshold of 75 pg/mL, the positive predictive value for a VIPoma was 12%. The optimal VIP threshold was 442 pg/mL (OR: 11.96, 95% CI: 2.00-79.69, P = 0.01), with statistically significant odds ratios starting at 200 pg/mL. Our findings suggest that elevated VIP concentrations are not predictive for a VIPoma and most patients with elevated VIP do not have a VIPoma. We recommend that VIP only be drawn in certain clinical scenarios to avoid unnecessary medical investigations.

#2

Severe secretory diarrhea due to VIPoma.

Endokrynologia Polska2025

Not required for Clinical Vignette.

#3

Severe Idiopathic Secretory Diarrhoea With a Profound Sustained Response to Somatostatin Analogues: A Case Report.

Case reports in gastrointestinal medicine2025

Chronic secretory diarrhoea is a diagnostic challenge with a broad differential and significant impact on patient's quality of life. While common causes include microscopic colitis, bile acid diarrhoea, and laxative use, rarer aetiologies such as vasoactive intestinal peptide (VIP)-secreting neuroendocrine tumours (VIPomas) must be considered when standard investigations fail. We present a 35-year-old woman with a two-year history of progressively worsening, fasting-persistent, high-volume watery diarrhoea leading to severe electrolyte abnormalities and weight loss requiring resuscitation in intensive care. Extensive biochemical, endoscopic, and radiological investigations-including faecal analysis, colonoscopy, neuroendocrine markers, multiphase CT, endoscopic ultrasound, and Ga-68 Dotatate PET imaging-failed to identify an underlying cause. Serum VIP levels remained within the normal range. Despite the absence of a definitive diagnosis, empirical treatment with the somatostatin analogue octreotide led to rapid and sustained symptom resolution. The patient was subsequently maintained on long-acting lanreotide with complete remission. Notably, diarrhoea recurred upon cessation of therapy, again resolving with reinitiation. After 4 years, the patient self-ceased lanreotide without symptom recurrence, and follow-up imaging remained unremarkable. This case highlights a diagnostic dilemma: clinical and biochemical features were highly suggestive of a VIPoma, yet no tumour was identified despite repeated advanced imaging and biochemical workup. The patient's remarkable therapeutic response to somatostatin analogue therapy, in the absence of confirmed neuroendocrine neoplasia, suggests that somatostatin analogues may have a broader role in the management of idiopathic secretory diarrhoea than currently appreciated. We present a rare case of chronic secretory diarrhoea with suspected but unproven VIPoma, demonstrating sustained and reproducible response to somatostatin analogue therapy. This case supports the consideration of therapeutic trials of somatostatin analogues in refractory secretory diarrhoea of unknown origin.

#4

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

Diagnostics (Basel, Switzerland)2025 Aug 28

Background: Functional pancreatic neuroendocrine tumors (FpNETs) are extremely rare in childhood and adolescence, with an incidence of less than 0.1 per million. Since there is currently no systematic review of the literature on FpNETs in children, this study aims to summarize findings from studies focusing on clinical characteristics, diagnostics, treatment modalities, and outcomes. Methods: A systematic review was conducted following the PRISMA guidelines. A literature search was performed using three electronic databases: PubMed, Scopus, and Web of Science. An age filter was used during the search to limit results to childhood and adolescence. There was no limit set in relation to the type and the language of the article. Results: Out of 80,742 records identified, 91 studies met the inclusion criteria and were included in the review. Two studies included patients with insulinoma and gastrinomas, that is, insulinomas and glucagonoma. Of the included studies, 71 were insulinomas, 10 were gastrinomas, 3 were glucagonomas, 6 were VIPomas, and 3 were mixed FpNETs. A total of 163 children with FpNETs were analyzed, with a median age of 12 years. A total of 48 cases were reported in childhood, while 115 cases were reported in adolescence. The results indicate that FpNETs were more prevalent in males. Almost all patients presented with symptoms appropriate to the type of tumor. A significant proportion of tumors were associated with MEN1. In almost all patients, the symptomatology was accompanied by elevated levels of specific hormones. US, CT, PET-CT, MRI, and EUS were the dominant imaging modalities. Surgical approaches and types of resections, depending on the type, association with the syndrome, location, and size of the tumor, were quite heterogeneous. Grade 1 and Grade 2 tumors were nearly equally represented. There was no recurrence in most patients. Conclusions: Early suspicion based on specific clinical symptomatology is essential for timely diagnosis. Accurate localization and size based on modern radiological diagnostics, accompanied by biochemical and genetic testing, are essential for optimal management. Adequate surgical resection offers the best chance of cure, with the lowest risk of recurrence. Additional multicenter registries and studies are needed in the future to better understand tumor behavior, optimal management, and outcomes of FpNETs.

#5

Stereotactic Body Radiation Therapy Facilitating Debulking Surgery in Metastatic VIPoma with Severe Diarrhea and Hypovolemic Shock.

The American journal of case reports2025 Sep 06

BACKGROUND Treatment of metastatic vasoactive intestinal peptide tumors (VIPoma) is challenging and requires a careful multidisciplinary approach to achieve optimal disease control. We present a case of metastatic VIPoma with recurring episodes of life-threatening diarrhea necessitating multiple intensive care unit (ICU) admissions. CASE REPORT A 54-year-old man presented with severe watery diarrhea and metabolic acidosis with MRI showing a necrotic pancreatic body mass, and multiple liver lesions. Histopathologic and biochemical findings were consistent with VIPoma. Due to the multifocal liver lesions, he was deemed unsuitable for curative surgical intervention, shifting focus to other therapies. Initial treatments over 7 months included octreotide, transarterial chemoembolization, yttrium-90 radioembolization, and systemic therapy with everolimus, with decreases in serum vasoactive intestinal peptide (VIP) levels from a peak of 3180 pg/mL to undetectable levels corresponding to temporary relief of diarrhea. However, the temporary relief was followed by a rapid increase in VIP levels into the 200s and 300s and recurrence of 3-11 liters of diarrhea per day. He had multiple ICU admissions due to severe diarrhea/hypovolemic shock until he was treated with stereotactic body radiation therapy (SBRT). SBRT of the pancreatic site immediately yielded long-term hemodynamic stability, prevented further ICU admissions, and enabled the patient to receive debulking surgery. CONCLUSIONS Although radiation is not typically a primary treatment modality for VIPoma, in this case, the high dose per fraction delivered with stereotactic technique to a pancreatic mass played a crucial role in alleviating his catastrophic volume loss through intravenous octreotide-refractory diarrhea, making the patient a suitable candidate for debulking surgery. This case highlights the importance of considering all available treatment options, even those not traditionally employed, when managing complex cases with refractory symptoms.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC183 artigos no totalmostrando 118

2026

Elevated vasoactive intestinal peptide concentrations poorly predict VIPoma.

Endocrine-related cancer
2025

Severe secretory diarrhea due to VIPoma.

Endokrynologia Polska
2025

Severe Idiopathic Secretory Diarrhoea With a Profound Sustained Response to Somatostatin Analogues: A Case Report.

Case reports in gastrointestinal medicine
2025

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

Diagnostics (Basel, Switzerland)
2025

Stereotactic Body Radiation Therapy Facilitating Debulking Surgery in Metastatic VIPoma with Severe Diarrhea and Hypovolemic Shock.

The American journal of case reports
2025

Nutritional Management of Functioning GEP-NENs.

Nutrients
2025

Octreotide infusion pump in patients with functional neuroendocrine tumors and refractory hormonal syndrome.

Endocrine oncology (Bristol, England)
2025

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

Endocrine pathology
2024

Case report: Resolution of VIPoma-related symptoms with peptide receptor radionuclide therapy.

Frontiers in oncology
2024

Vasoactive Intestinal Polypeptide Secreting MS Neuroblastoma.

Journal of Indian Association of Pediatric Surgeons
2024

Carcinoid heart findings in vasoactive intestinal peptide-secreting tumour.

BMJ case reports
2024

VIPoma: An Unusual Cause of Chronic Diarrhea.

Acta medica (Hradec Kralove)
2024

Efficacy of Racecadotril in a Patient Affected by a Therapy-Refractory VIPoma and Carcinoid Syndrome.

JCEM case reports
2024

Vasoactive Intestinal Peptide-Producing Neuroblastic Tumors: A Rare Cause of Refractory Diarrhea.

Cureus
2024

Endoscopic Ultrasound-Guided Radiofrequency Ablation of Metastatic Pancreatic VIPoma: A Novel Treatment.

Cureus
2024

Management of functional neuroendocrine tumors.

Current problems in cancer
2024

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

Scientific reports
2024

Interference With VIP to Distinguish Between Real and False VIPoma: National Study From the French Endocrine Tumors Group.

Journal of the Endocrine Society
2024

Successful liver transplantation as rescue therapy in a patient with metastases from a vasoactive intestinal peptide producing neuroendocrine tumor.

Journal of surgical case reports
2024

A Rare Presentation of Lymph Node Metastasis of VIPoma After Three Years of Resection: A Case Report.

Cureus
2024

All you need to know about VIPoma: Review on the latest studies.

Presse medicale (Paris, France : 1983)
2024

Pancreatic VIPomas From Colombia.

Pancreas
2023

Synchronous Insulinoma and Glucagonoma: A Review of the Literature.

In vivo (Athens, Greece)
2023

Chronic Diarrhea Caused by Vasoactive Intestinal Peptide-Secreting Tumor.

Life (Basel, Switzerland)
2023

Pancreatic Vasoactive Intestinal Peptide-Producing Tumor as a Rare Cause of Acute Diarrhea and Severe Hypokalemia.

Journal of medical cases
2024

Calcium-sensing receptor activator cinacalcet for treatment of cyclic nucleotide-mediated secretory diarrheas.

Translational research : the journal of laboratory and clinical medicine
2023

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

Journal of neuroendocrinology
2022

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

Pancreas
2023

Recurrent primary hepatic VIPoma treated with a combination of surgical resection and loco-regional therapy.

Future science OA
2023

Hypercalcemia as the Presenting Finding in VIPoma.

The Brown journal of hospital medicine
2023

A case of vasoactive intestinal peptide-secreting tumor (VIPoma) arising from MEN1 inactivation which recurred 15 years after the initial resection.

Endocrine journal
2023

An Insight on Functioning Pancreatic Neuroendocrine Neoplasms.

Biomedicines
2022

Vasoactive Intestinal Peptide Tumor as the Cause of Persistent Diarrhea: A Diagnostic Challenge.

Cureus
2022

Circulating Neuroendocrine Tumor Biomarkers: Past, Present and Future.

Journal of clinical medicine
2022

Metastatic mixed VIPoma/PPoma-induced diarrhoea causing renal failure.

Endocrinology, diabetes &amp; metabolism case reports
2022

Treatment options of metastatic and nonmetastatic VIPoma: a review.

Langenbeck's archives of surgery
2022

A clinical analysis on functioning pancreatic neuroendocrine tumors (focusing on VIPomas): a single-center experience.

Endocrine journal
2022

Vasoactive intestinal peptide secreting tumour: An overview.

World journal of gastrointestinal oncology
2023

Localization of VIPoma with NETSpot.

The American surgeon
2022

Vasoactive Intestinal Peptide-Secreting Pancreatic Neuroendocrine Tumor: A Case Report.

Cureus
2022

VIPoma: an unsuspecting culprit of severe secretory diarrhoea in a human immunodeficiency virus-infected patient.

Internal medicine journal
2022

Laparoscopic resection of VIPoma presenting at an unusual location.

Journal of minimal access surgery
2021

Surgical treatment of metastatic VIPoma: a case report.

Therapeutic advances in gastroenterology
2021

Life-threatening diarrhea in neuroendocrine tumors: two case reports.

Journal of medical case reports
2021

[VIPoma of the Pancreas].

Praxis
2021

Pancreatic VIPoma as a Differential Diagnosis in Chronic Pediatric Diarrhea: A Case Report and Review of the Literature.

Journal of medical cases
2021

Efficacy of treatments for VIPoma: A GTE multicentric series.

Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.]
2021

Evaluation of the Octreotide Acetate Pen Injector and its Instructions for Use in a Formative Human Factors Study.

Advances in therapy
2021

A Case of Metastatic VIPoma With Complete Response to Peptide Radionuclide Receptor Therapy.

Pancreas
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

Repurposing calcium-sensing receptor agonist cinacalcet for treatment of CFTR-mediated secretory diarrheas.

JCI insight
2021

Clinical-Pathological Conference Series from the Medical University of Graz : Case No 173: A 77-year-old patient with adenocarcinoma of the prostate, liver metastases and watery diarrhea.

Wiener klinische Wochenschrift
2020

MORPHOLOGICAL DIAGNOSIS OF PANCREATIC NEUROENDOCRINE TUMORS (REVIEW AND CASE REPORT).

Georgian medical news
2021

Pancreatic neuroendocrine tumors: Surgical outcomes and survival analysis.

American 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

Vasoactive intestinal peptide-oma causing refractory diarrhea in a young woman.

Proceedings (Baylor University. Medical Center)
2021

An Unusual Cause of Watery Diarrhea.

Gastroenterology
2021

Percutaneous Cryoablation of Recurrent Pancreatic Mass for Life-Threatening Pancreatic VIPoma Syndrome: A Case Report.

Cardiovascular and interventional radiology
2020

Rare Cases of Pediatric Vasoactive Intestinal Peptide Secreting Tumor With Literature Review: A Challenging Etiology of Chronic Diarrhea.

Frontiers in pediatrics
2020

Incidental 68Ga-DOTATATE uptake in the pancreatic head: A case report and a unique opportunity to improve clinical care.

Medicine
2020

A Rare Cause of Refractory Chronic Diarrhea and Cachexia: A Case Report.

Iranian journal of public health
2020

Neuroendocrine pancreatic tumor causing chronic diarrhea in young adult, a case report.

AME case reports
2020

Hypercalcemia in a Patient Diagnosed with a Vasoactive Intestinal Peptide Tumor.

Cureus
2020

Metastatic VIPoma, Cosecreting Insulin, With Complete Response to Lanreotide, Capecitabine, and Temozolomide.

Pancreas
2020

[Use of a somatostatin analog to improve a patient's condition and the subsequent diagnosis of pancreatic VIPoma:a case report].

Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterology
2019

Successful Treatment of Hypokalemic Rhabdomyolysis Caused by a Pancreatic VIPoma: A Case Report.

The American journal of case reports
2019

Prevalence of Diagnostic Methods and Treatment Modalities in Vipoma Patients: A Rare Cause of Hormone-Mediated Diarrhea.

Indian journal of endocrinology and metabolism
2019

Vasoactive Intestinal Peptide-Secreting Tumors: A Review.

Pancreas
2019

[A Case of WDHA Water Diarrhea Hypokalemia Achlorhydria Syndrome that Developed after Multimodal Therapy for Retroperitoneal Paraganglioma].

Hinyokika kiyo. Acta urologica Japonica
2019

Pancreatic neuroendocrine microadenomatosis presenting as a functional VIPoma.

Journal of surgical case reports
2019

Diagnostic and Management Challenges in Vasoactive Intestinal Peptide Secreting Tumors: A Series of 15 Patients.

Pancreas
2019

Life-threating diarrhea and acute renal failure secondary to pancreatic VIPoma treated by surgery.

Revista espanola de enfermedades digestivas
2019

Clinicopathological data and treatment modalities for pancreatic vipomas: a systematic review.

Journal of B.U.ON. : official journal of the Balkan Union of Oncology
2019

Chronic Diarrhea Secondary to Newly Diagnosed VIPoma.

Case reports in gastroenterology
2019

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

Current radiopharmaceuticals
2019

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

The Journal of clinical endocrinology and metabolism
2018

Octreotide reverses shock due to vasoactive intestinal peptide-secreting adrenal pheochromocytoma: A case report and review of literature.

World journal of clinical cases
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

Pancreatic VIPomas from China: Case reports and literature review.

Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.]
2019

Perioperative Hypotensive Crisis in an Adolescent with a Pancreatic VIPoma and MEN1-Gene Variant.

Hormone research in paediatrics
2019

Results of Duodenopancreatic Reoperations in Multiple Endocrine Neoplasia Type 1.

World journal of surgery
2018

Paediatric VIPoma: A Jamboree of Electrolytes.

Turkish journal of anaesthesiology and reanimation
2018

Flushing Disorders Associated with Gastrointestinal Symptoms: Part 1, Neuroendocrine Tumors, Mast Cell Disorders and Hyperbasophila.

Clinical medicine &amp; research
2018

[Surgical strategies for small sporadic neuroendocrine pancreatic tumors].

Der Chirurg; Zeitschrift fur alle Gebiete der operativen Medizen
2018

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

Reviews in endocrine &amp; metabolic disorders
2017

[A Surgical Resected Case of VIPoma with Para-Aortic Lymph Node Involvement].

Gan to kagaku ryoho. Cancer &amp; chemotherapy
2018

[VIPoma : a rare etiology of diarrhea with hypokalemia].

Revue medicale suisse
2017

Prevalence of Vibrio cholerae O1 serogroup in Assam, India: A hospital-based study.

The Indian journal of medical research
2017

Verner-Morrison syndrome. Literature review.

Romanian journal of morphology and embryology = Revue roumaine de morphologie et embryologie
2017

Management of the hormonal syndrome of neuroendocrine tumors.

Archives of medical science : AMS
2017

Watery stools and metabolic acidosis.

Internal and emergency medicine
2017

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

Neuroendocrinology
2017

Percutaneous Irreversible Electroporation for Pancreatic VIPoma: A Case Report.

Pancreas
2016

Pancreatic Neuroendocrine Tumor Secreting Vasoactive Intestinal Peptide and Dopamine With Pulmonary Emboli: A Case Report.

The Journal of clinical endocrinology and metabolism
2016

Emergency therapy for liver metastases from advanced VIPoma: surgery or transarterial chemoembolization?

Therapeutic advances in medical oncology
2017

Primary Pancreatic Secretinoma: Further Evidence Supporting Secretin as a Diarrheogenic Hormone.

Annals of surgery
2016

Medical management of secretory syndromes related to gastroenteropancreatic neuroendocrine tumours.

Endocrine-related cancer
2016

Pancreatic neuroendocrine tumors: contemporary diagnosis and management.

Hospital practice (1995)
2016

Epidemiological features of gastroenteropancreatic neuroendocrine tumors in Chengdu city with a population of 14 million based on data from a single institution.

Asia-Pacific journal of clinical oncology
2016

Neuroendocrine Differentiation of a Primary BRAF Mutant Colon Cancer in a Patient With a History of Hairy Cell Leukemia.

Clinical colorectal cancer
2016

Ventricular fibrillation resulting from electrolyte imbalance reveals vipoma in MEN1 syndrome.

Familial cancer
2016

Clinical Presentation and Diagnosis of Pancreatic Neuroendocrine Tumors.

Surgical oncology clinics of North America
2016

Surgical resection of vasoactive intestinal peptideoma with hepatic metastasis aids symptom palliation: A case report.

Experimental and therapeutic medicine
2016

A short history of neuroendocrine tumours and their peptide hormones.

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

Neuralgic amyotrophy mimicking Vernet syndrome.

Journal of the neurological sciences
2016

When Symptomatic Treatment Becomes Antitumor Treatment for Vipoma: Opportunity for Frail Elderly Adults.

Journal of the American Geriatrics Society
2015

[VIPoma in retroperitoneum of elderly, cause of chronic diarrhea in uncommon location].

Revista de gastroenterologia del Peru : organo oficial de la Sociedad de Gastroenterologia del Peru
2016

Malignant-functioning neuroendocrine tumors of the pancreas: A survival analysis.

Surgery
2015

Metastatic VIPoma presenting as an ovarian mass.

International journal of surgery case reports
2015

Multiple-phase spiral CT findings of pancreatic vasoactive intestinal peptide-secreting tumor: A case report.

Oncology letters
2015

VIPoma with multiple endocrine neoplasia type 1 identified as an atypical gene mutation.

BMJ case reports
2015

A 25-Year Experience of Gastroenteropancreatic Neuroendocrine Tumors and Somatostatin (Congeners) Analogs: From Symptom Control to Antineoplastic Therapy.

Frontiers of hormone research
2015

Pancreatic VIPoma visualized by 68Ga DOTA-NOC PET-CT.

Nuklearmedizin. Nuclear medicine
2015

Watery diarrhea-hypopotassemia-acidosis syndrome like diarrhea in a case with X-linked lissencephaly with abnormal genitalia.

Minerva pediatrica
2015

Clinical features of pancreatic neuroendocrine tumors.

Journal of hepato-biliary-pancreatic sciences
2015

Intractable and dramatic diarrhea in liver transplantation recipient with vasoactive intestinal peptide-producing tumor after split liver transplantation: a case report.

Transplantation proceedings
Ver todos os 183 no EuropePMC

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Referências e fontes

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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. Elevated vasoactive intestinal peptide concentrations poorly predict VIPoma.
    Endocrine-related cancer· 2026· PMID 41498509mais citado
  2. Severe secretory diarrhea due to VIPoma.
    Endokrynologia Polska· 2025· PMID 41340353mais citado
  3. Severe Idiopathic Secretory Diarrhoea With a Profound Sustained Response to Somatostatin Analogues: A Case Report.
    Case reports in gastrointestinal medicine· 2025· PMID 41195063mais citado
  4. Diagnosis and Management of Functional Pancreatic Neuroendocrine Tumors in Children-A Systematic Review.
    Diagnostics (Basel, Switzerland)· 2025· PMID 40941664mais citado
  5. Stereotactic Body Radiation Therapy Facilitating Debulking Surgery in Metastatic VIPoma with Severe Diarrhea and Hypovolemic Shock.
    The American journal of case reports· 2025· PMID 40913783mais citado
  6. Approach to Hypercalcemia.
    · 2000· PMID 25905352recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:97282(Orphanet)
  2. MONDO:0019960(MONDO)
  3. GARD:3787(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q1518637(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

VIPoma
Compêndio · Raras BR

VIPoma

ORPHA:97282 · MONDO:0019960
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
1 ativos
Início
All ages
Prevalência
0.0 (Europe)
MedGen
UMLS
C0011993
EuropePMC
Wikidata
Papers 10a
DiscussaoAtiva

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