Raras
Buscar doenças, sintomas, genes...
Tumor carcinoide e síndrome carcinoide
ORPHA:100093CID-10 · E34.0CID-11 · 5B10DOENÇA RARA

Telotristat etil, etiltelotristato,, telotristato de etila ou telotristat ethyl é um pró-fármaco de telotristat, o qual é um inibitor de triptofano hidroxilase. É formulado como etiprato de telotristat — um sal hipurato do telotristat etil.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

Tumor carcinoide e síndrome carcinoide são condições associadas a tumores neuroendócrinos, frequentemente pulmonares, que produzem excesso de serotonina. Isso pode levar a sintomas como diarreia, rubor, sopros cardíacos e rinorreia, com mutações no gene SDHD sendo um fator de risco.

Pesquisas ativas
5 ensaios
39 total registrados no ClinicalTrials.gov
Publicações científicas
2.818 artigos
Último publicado: 2026 Apr 11
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: E34.0
Você se identifica com essa condição?
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

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

Partes do corpo afetadas

🫃
Digestivo
8 sintomas
🫁
Pulmão
4 sintomas
❤️
Coração
3 sintomas
😀
Face
1 sintomas
🩸
Sangue
1 sintomas
💪
Músculos
1 sintomas

+ 9 sintomas em outras categorias

Características mais comuns

100%prev.
Tumor carcinoide
55%prev.
Placa eritematosa
Frequente (79-30%)
55%prev.
Diarreia prolongada
Frequente (79-30%)
55%prev.
Dor abdominal episódica
Frequente (79-30%)
55%prev.
Carcinoide do intestino delgado
Frequente (79-30%)
55%prev.
Carcinoide intestinal
Frequente (79-30%)
27sintomas
Muito frequente (1)
Frequente (6)
Ocasional (15)
Muito raro (5)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 27 características clínicas mais associadas, ordenadas por frequência.

Tumor carcinoideCarcinoid tumor
Muito frequente100%
Placa eritematosaErythematous plaque
Frequente (79-30%)55%
Diarreia prolongadaProtracted diarrhea
Frequente (79-30%)55%
Dor abdominal episódicaEpisodic abdominal pain
Frequente (79-30%)55%
Carcinoide do intestino delgadoSmall intestine carcinoid
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico2.818PubMed
Últimos 10 anos200publicações
Pico202565 papers
Linha do tempo
2026Hoje · 2026🧪 1996Primeiro ensaio clínico📈 2025Ano 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

Genes associados

1 gene identificado com associação a esta condição.

Not applicable
SDHDSuccinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrialCandidate gene tested inRestrito
FUNÇÃO

Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q) (PubMed:10482792, PubMed:9533030). SDH also oxidizes malate to the non-canonical enol form of oxaloacetate, enol-oxaloacetate (By similarity). Enol-oxaloacetate, which is a potent inhibitor of the succinate dehydrogenase activity, is further isomerized into keto-oxaloacetate

LOCALIZAÇÃO

Mitochondrion inner membrane

VIAS BIOLÓGICAS (3)
Maturation of TCA enzymes and regulation of TCA cycleCitric acid cycle (TCA cycle)Respiratory electron transport
MECANISMO DE DOENÇA

Pheochromocytoma/paraganglioma syndrome 1

A form of pheochromocytoma/paraganglioma syndrome, a tumor predisposition syndrome characterized by the development of neuroendocrine tumors, usually in adulthood. Pheochromocytomas are catecholamine-producing tumors that arise from chromaffin cells in the adrenal medulla. Paragangliomas develop from sympathetic paraganglia in the thorax, abdomen, and pelvis, as well as from parasympathetic paraganglia in the head and neck. PPGL1 inheritance is autosomal dominant.

EXPRESSÃO TECIDUAL(Ubíquo)
Rim - Medula
91.4 TPM
Músculo esquelético
85.3 TPM
Cólon transverso
81.0 TPM
Cólon sigmoide
78.9 TPM
Linfócitos
75.5 TPM
OUTRAS DOENÇAS (8)
Carney-Stratakis syndromepheochromocytoma/paraganglioma syndrome 1mitochondrial complex 2 deficiency, nuclear type 3sporadic pheochromocytoma/secreting paraganglioma
HGNC:10683UniProt:O14521

Medicamentos aprovados (FDA)

2 medicamentos encontrados nos registros da FDA americana.

💊 Lanreotide Acetate (LANREOTIDE ACETATE)
💊 Xermelo (TELOTRISTAT ETHYL)
Ver no DailyMed/FDA

Variantes genéticas (ClinVar)

267 variantes patogênicas registradas no ClinVar.

🧬 SDHD: NM_003002.4(SDHD):c.174del (p.Ser59fs) ()
🧬 SDHD: NM_003002.4(SDHD):c.196del (p.Trp66fs) ()
🧬 SDHD: NM_003002.4(SDHD):c.334dup (p.Thr112fs) ()
🧬 SDHD: NM_003002.4(SDHD):c.233del (p.Gly78fs) ()
🧬 SDHD: NM_003002.4(SDHD):c.170-1G>C ()
Ver todas no ClinVar

Vias biológicas (Reactome)

3 vias biológicas associadas aos genes desta condição.

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.
Aprovado1
3Fase 34
2Fase 28
1Fase 11
·Pré-clínico6
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 20 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Tumor carcinoide e síndrome carcinoide

🗺️

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

🟢 Recrutando agora

2 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

39 ensaios clínicos encontrados, 5 ativos.

Distribuição por fase
NCT04993261 · An Investigational Scan (Dual Energy CT) in Detecting Gastro…Ativo
EARLY_PHASE1
NCT05064514 · Investigation of a Transcatheter Tricuspid Valved Stent Graf…Ativo
NA
NCT07450287 · CardioNETPOL National Registry of Carcinoid Heart DiseaseEm breve
NCT05361668 · Study to Evaluate the Safety, PK, and Dose Response of Paltu…Concluído
PHASE2
NCT03453489 · AMT-PET in Monitoring Telotristat Etiprate Treatment in Part…Concluído
PHASE2
NCT03223428 · Real-world Evidence Study EvaLuating PAtient-Reported Outcom…Concluído
NCT04713202 · Prospective Assessment of Patients With Neuroendocrine Tumor…Cancelado
PHASE2
NCT03722511 · Evaluating an Amino Acid Based Medical Food w/ Diarrhea in C…Concluído
PHASE2
NCT00774930 · An Efficacy and Safety Study of Somatuline Depot (Lanreotide…Concluído
PHASE3
NCT04776876 · Retifanlimab (INCMGA00012) and Telotristat Ethyl for the Tre…Cancelado
PHASE2
NCT04073017 · Enterade in Carcinoid/Non-Carcinoid Syndrome Neuroendocrine …Encerrado
NA
NCT01018953 · Study to Assess the Efficacy and Safety of Different Doses o…Encerrado
PHASE2
NCT01234168 · A Study to Assess Neuroendocrine Tumour (NET) Patients Curre…Concluído
NCT04039516 · Carcinoid Heart Disease and Peptide Receptor Radiotargetted …UNKNOWN
PHASE2
NCT00092287 · Comparison of Lanreotide Autogel® and Sandostatin LAR Depot …Encerrado
PHASE3
NCT04140409 · Sandostatin (Octreotide LAR) May Lead to Clinical Improvemen…Encerrado
PHASE4
NCT02026063 · Telotristat Etiprate - Expanded Treatment for Patients With …Concluído
PHASE3
NCT01104415 · Study of Telotristat Etiprate (LX1606) in Participants With …Concluído
PHASE2
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
733 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 733

#1

Oxalate nephropathy from untreated pancreatic insufficiency in a neuroendocrine tumour patient.

BMJ case reports2026 Feb 26

Pancreatic exocrine insufficiency (PEI) is an under-recognised cause of chronic diarrhoea in patients with neuroendocrine tumours (NETs). If left untreated or undertreated, PEI can lead to complications such as oxalate nephropathy (ON), which may result in loss of kidney function and potentially limit oncologic treatment options, including peptide receptor radionuclide therapy (PRRT). We present a man in his late 60s with a small bowel NET who developed persistent, uncontrolled diarrhoea for several years. The diarrhoea was attributed to multiple factors, including secretory NET (ie, carcinoid syndrome), bile acid diarrhoea and PEI secondary to somatostatin analogue therapy. Due to financial constraints, the patient was unable to consistently take pancreatic enzyme replacement therapy (PERT). Years after the diagnosis of NET, the patient developed a new onset worsening of kidney function. Further work-up, including a kidney biopsy, confirmed ON, which significantly limited subsequent treatment options, including PRRT. Routine screening for PEI and early initiation of PERT should be considered in all NET patients with chronic diarrhoea to prevent ON and preserve future treatment options.

#2

Real-World Observational Study of Somatostatin Analogs and Rescue Medication Usage for Neuroendocrine Tumors in Canada.

Neuroendocrinology2026 Mar 17

Neuroendocrine tumors (NETs) are uncommon malignancies with increasing incidence in Canada. Long-acting somatostatin analogs (SSAs) such as Lanreotide Autogel (LAN-ATG) and Octreotide Long-Acting Release (OCT-LAR) are first-line treatments for well-differentiated GEP-NETs either with or without carcinoid syndrome (CS). Despite their efficacy, many patients require short-acting SSAs for breakthrough symptoms. We investigated the impact of switching between long-acting SSAs on the usage of breakthrough medications and the incidence of above maximum recommended dose (AMRD) prescriptions. This population-based study used claims data from the IQVIA Canadian Private Drug Plan (PDP) that includes data from all Canadian provinces and public drug programs from Ontario and Québec. We identified NET patients who switched SSAs between September 2015 and December 2021. We analyzed breakthrough medication usage and AMRD prescriptions before and after the switch. Among 170 patients who switched SSAs, there was a 59.1% overall reduction in breakthrough medication claims post-switch. Patients switching from OCT-LAR to LAN-ATG experienced a 66.5% reduction, while those switching from LAN-ATG to OCT-LAR saw a 21.9% decrease. Pre-switch, significantly more patients on OCT-LAR exceeded the AMRD threshold compared to those on LAN-ATG (43.8% vs. 18.2%, p value = 0.008). Switching between long-acting SSAs can reduce the need for breakthrough medications and lower the incidence of AMRD prescriptions, with a more pronounced effect observed when switching from OCT-LAR to LAN-ATG. These findings have potential implications for improving quality of life and reducing treatment costs for NET patients, which should be evaluated in a formal health economic analysis.

#3

Recurrent ventricular asystole during octreotide infusion in a patient with pancreatic neuroendocrine tumor: a case report.

Journal of medical case reports2026 Mar 16

Somatostatin analogs such as octreotide are frequently used in the prevention of carcinoid syndrome in the perioperative period following neuroendocrine tumor resection. This is the first reported case of ventricular asystole associated with postoperative intravenous octreotide infusion in a patient with previous congenital cardiac surgery. We present the case of a 62-year-old white British male patient with a history of previous congenital cardiac surgery who sustained multiple episodes of ventricular asystole and associated loss of cardiac output during octreotide infusion. Recurrent asystole ceased following discontinuation of the octreotide infusion. Physicians caring for patients receiving intravenous octreotide should be aware of the potential arrhythmogenic adverse effects. While specific risk factors for octreotide-associated arrhythmias are not generally understood, previous cardiac surgery could potentially be one of these. Risk stratification guidance regarding patient groups requiring heightened monitoring while receiving intravenous octreotide is required. Monitoring recommendations may involve invasive blood pressure monitoring and telemetry in these cases.

#4

Determining Prognosis in Patients With Carcinoid Syndrome: A Retrospective Single-Center Cohort Study.

Journal of the National Comprehensive Cancer Network : JNCCN2026 Mar 09

Carcinoid syndrome (CS), the most prevalent neuroendocrine tumor (NET)-related hormonal syndrome, is associated with impaired survival. However, the contribution of NET-specific death in patients with this syndrome is currently unknown. This study aimed to evaluate overall survival (OS), prognostic factors, and causes of death in patients with CS. We retrospectively included patients with CS treated between 1995 and 2021 at our ENETS Center of Excellence. We used Kaplan-Meier curves and log-rank tests to perform survival analyses, and a Cox proportional hazards model to calculate mortality hazard ratios (HRs). A total of 295 of 427 included patients with CS died during follow-up, and cause of death was recorded in 255 patients. In 231 (90.6%) patients, the cause of death was NET-specific. Median OS was 7.1 years, and 65.1% and 34.1% of CS patients were alive at 5 and 10 years, respectively. Multivariate analysis identified age (HR, 1.06; P<.001), WHO tumor grade 2 (HR, 2.11; P=.01), plasma chromogranin A (CgA) level of 200 to 940 µg/L (HR, 1.64; P=.04), CgA level >940 µg/L (HR, 3.18; P<.001), and primary lung NET (HR, 1.77; P=.008) as negative predictors of survival. The presence of carcinoid heart disease (HR, 1.01; P=.97) was not associated with OS in a multivariate model due to interaction with CgA levels. Patients with CS are likely to succumb to their disease, with >90% of mortality being NET-specific. Age, tumor grade, primary lung origin, and CgA levels were independent predictors of mortality. These findings indicate that there is an urgent need for advances in therapeutic options for patients with CS.

#5

Multivalvular Cardiac Involvement from Giant Hepatic Metastases of an Ileal Neuroendocrine Tumor.

Cureus2026 Feb

Intestinal neuroendocrine tumors (NETs) are rare, slow-growing neoplasms arising from enterochromaffin cells, capable of secreting vasoactive substances that may cause carcinoid syndrome (CS) and clinically significant valvular heart disease. These tumors are often diagnosed at advanced stages due to nonspecific gastrointestinal symptoms, and distant metastases, particularly to the liver and lymph nodes, are common at presentation. Hormonal dysregulation can lead to chronic diarrhea, flushing, and bronchospasm, while carcinoid heart disease (CHD) contributes substantially to morbidity and mortality, typically affecting right-sided valves, with left-sided involvement being uncommon and more frequently associated with intracardiac shunts than exceptionally high serotonin exposure. Recent advances in management emphasize a multidisciplinary approach integrating systemic therapy, surgery, and targeted radionuclide treatment. Somatostatin analog therapy remains the cornerstone for controlling hormonal symptoms and slowing tumor progression. Aggressive cytoreductive surgery to achieve hormonal stabilization prior to surgical valve replacement has been associated with improved survival, symptomatic relief, and cardiac function. Targeted peptide receptor radionuclide therapy provides additional treatment for residual or metastatic disease, enhancing biochemical and radiological control. We report the case of a 61-year-old woman with chronic diarrhea, weight loss, and recurrent flushing, diagnosed with a well-differentiated ileal NET with extensive hepatic metastases and severe right-sided valvular disease with mild left-sided involvement. She underwent somatostatin analog therapy to control hormonal symptoms, cytoreductive surgery, and surgical replacement of the pulmonary and tricuspid valves. Subsequent targeted peptide receptor radionuclide therapy further reduced tumor burden and stabilized biochemical markers. This combined multimodal strategy resulted in sustained clinical improvement, normalization of neuroendocrine markers, and long-term oncologic stability, as the patient remains asymptomatic and oncologically stable after four years of follow-up from initial presentation. This case highlights the importance of early recognition, comprehensive evaluation, and a multidisciplinary strategy in advanced NETs. Coordinated care integrating endocrinology, oncology, cardiology, nuclear medicine, and surgery can significantly improve survival, hormonal control, and quality of life in patients with complex metastatic disease.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC1.041 artigos no totalmostrando 194

2026

Real-World Observational Study of Somatostatin Analogs and Rescue Medication Usage for Neuroendocrine Tumors in Canada.

Neuroendocrinology
2026

Recurrent ventricular asystole during octreotide infusion in a patient with pancreatic neuroendocrine tumor: a case report.

Journal of medical case reports
2026

Determining Prognosis in Patients With Carcinoid Syndrome: A Retrospective Single-Center Cohort Study.

Journal of the National Comprehensive Cancer Network : JNCCN
2026

Multivalvular Cardiac Involvement from Giant Hepatic Metastases of an Ileal Neuroendocrine Tumor.

Cureus
2026

Persistent facial flushing as a cutaneous manifestation of carcinoid syndrome.

Medicina clinica
2026

Oxalate nephropathy from untreated pancreatic insufficiency in a neuroendocrine tumour patient.

BMJ case reports
2026

Oncological Outcomes after Elective and Emergency Resection of Small Intestinal Neuroendocrine Tumours.

Journal of gastrointestinal cancer
2026

Nonmetastatic Pulmonary Carcinoid Presenting With Carcinoid Syndrome Despite Negative 5-HIAA: A Case Report.

Case reports in endocrinology
2026

Carcinoid Heart Disease: Diagnostic Value of Cardiac MRI in a Patient With Metastatic Small‑Intestinal Neuroendocrine Tumor.

Journal of the Belgian Society of Radiology
2026

Contrast-Enhanced Ultrasound and Somatostatin Receptor Scintigraphy Unveil an Occult Neuroendocrine Tumor With Carcinoid Syndrome and Presumed Small Intestinal Origin - A Case Report.

Gastro hep advances
2026

LUNG CARCINOIDS (Lung Neuroendocrine Tumors).

Endocrine reviews
2026

Intravenous adrenaline (epinephrine): its use in the critical care setting.

Postgraduate medical journal
2025

Diagnosis and Treatment of Right-Sided Valve Disease in Cardiac Carcinoid Syndrome: A Systematic Review.

Cardiology
2025

Ovarian carcinoid tumor with carcinoid heart syndrome: A case report and literature review.

Gynecologic oncology reports
2025

Exploring the carcinoid crisis: insights from a cancer-specific centre.

Endocrine oncology (Bristol, England)
2026

Flushing in Carcinoid Syndrome.

Actas dermo-sifiliograficas
2025

Paraneoplastic Neurological Syndromes in Oncology: Clinical Features, Mechanisms, and Educational Perspectives.

Journal of cancer education : the official journal of the American Association for Cancer Education
2025

Metastatic neuroendocrine neoplasms of the breast: a systematic review.

Endocrine-related cancer
2026

ASO Visual Abstract: Carcinoid Syndrome Impacts Long-Term Outcomes After Hepatectomy for Small Bowel Neuroendocrine Tumor Liver Metastasis.

Annals of surgical oncology
2025

Carcinoid Heart Disease Revealing the Burden of a Neuroendocrine Tumour: A Case Report.

Cureus
2025

Uncommon Origin, Common Presentation: A Rare Case of Mesenteric Carcinoid Tumor Mimicking Functional Gastrointestinal Disorders.

Cureus
2025

Paltusotine versus octreotide: different effects on radioligand uptake in neuroendocrine tumours.

Endocrine oncology (Bristol, England)
2025

Complications of ovarian metastases from well-differentiated small bowel neuroendocrine neoplasms: a focus on bowel and ureteral obstruction.

Surgical oncology
2026

AIMN, ITANET, SIE joint position paper for the recognition, prevention and management of NET carcinoid syndrome and crises during peptide receptor radionuclide therapy.

European journal of nuclear medicine and molecular imaging
2026

Carcinoid Syndrome Impacts Long-Term Outcomes After Hepatectomy for Small Bowel Neuroendocrine Tumor Liver Metastasis.

Annals of surgical oncology
2025

Bilateral carcinoid heart disease secondary to a massive right-to-left shunt through a patent foramen ovale: a case report.

European heart journal. Case reports
2025

Telotristat ethyl affects tumour-fibroblast crosstalk in small intestinal neuroendocrine tumours.

Journal of neuroendocrinology
2025

Case report of carcinoid syndrome with multi-organ involvement.

Journal of gastrointestinal oncology
2025

Outcomes of surgical management of carcinoid heart disease in patients with primary gonadal neuroendocrine tumors.

JTCVS open
2025

Clinical presentation of bone metastases in neuroendocrine tumors: a retrospective cohort study.

Endocrine-related cancer
2025

[Primary hepatic neuroendocrine neoplasms: a case series analysis of 10 patients and literature review].

Zhejiang da xue xue bao. Yi xue ban = Journal of Zhejiang University. Medical sciences
2025

Carcinoid syndrome mimicking irritable bowel syndrome: don't fall into the trap.

Expert review of gastroenterology &amp; hepatology
2025

Concomitant carcinoid heart disease and patent foramen ovale: a case report and review of literature.

Cardio-oncology (London, England)
2025

A Rare Case of Idiopathic Angioedema Associated With Amlodipine Use.

Cureus
2025

A Rare Discovery of Neuroendocrine Tumor: Cardiac Mass.

Journal of community hospital internal medicine perspectives
2025

A Royal Flush: Carcinoid Heart Disease Complicated by Severe Tricuspid and Pulmonic Valve Regurgitation.

Cureus
2025

Diagnostic Dilemma of a Neuroendocrine Tumour Complicated by Simultaneous Retroperitoneal Fibrosis and Carcinoid Heart Disease in a Perimenopausal Woman.

Cureus
2025

A deep foundation model for electrocardiogram interpretation: enabling rare disease detection through transfer learning.

European heart journal. Digital health
2025

Impact of treatment on quality of life in neuroendocrine neoplasm survivors.

Endocrine-related cancer
2025

Nutritional Management of Functioning GEP-NENs.

Nutrients
2025

Carcinoid Tumor of the Duodenum Presenting as Iron Deficiency Anemia.

Oman medical journal
2025

Pulmonary Sclerosing Pneumocytoma Masquerading as a Carcinoid Tumor.

Clinical lung cancer
2025

Looking beyond the heart: carcinoid syndrome.

European heart journal. Case reports
2025

A Rare Case of a Neuroendocrine Tumor With Intracardiac Metastasis: Management and Literature Review.

Cureus
2025

Atypical Presentation of Small Bowel Neuroendocrine Carcinoma Leading to Acute Obstruction.

Cureus
2025

Hiding in Plain Sight: Pulmonic Valve Involvement in Carcinoid Heart Disease.

The American journal of cardiology
2025

Orthotopic transcatheter tricuspid valve replacement in a patient with carcinoid syndrome.

The Journal of invasive cardiology
2025

Red Emitting Fluorescent EuDPA Complex as a Probe for the Detection of Serotonin, A Neurotransmitter.

Journal of fluorescence
2025

PFO closure in a patient with hypoxia due to tricuspid valve regurgitation and flow-driven shunt.

BMJ case reports
2025

Echocardiographic Evaluation of Carcinoid Heart Disease.

Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography
2025

A Tumultuous Journey of Metastatic Pancreatic Neuroendocrine Tumor with Carcinoid Syndrome.

Journal of the ASEAN Federation of Endocrine Societies
2025

Psychiatric and cognitive function in patients with serotonin producing neuroendocrine tumors.

Translational psychiatry
2025

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

Endocrine oncology (Bristol, England)
2026

Symptom Management for Well-Differentiated Gastroenteropancreatic Neuroendocrine Tumors: ASCO Guideline.

JCO oncology practice
2025

[Treatment options in carcinoid heart disease].

Lakartidningen
2025

Serum 5-Hydroxyindoleacetic Acid Measurements for the Diagnosis and Follow-up of Carcinoid Syndrome.

The Journal of clinical endocrinology and metabolism
2025

Post-marketing Safety Evaluation of Lutathera ( 177 Lu-DOTATATE) : A Pharmacovigilance Analysis of FDA Adverse Event Reporting System.

Clinical nuclear medicine
2025

Hypertension and Carcinoid Heart Disease as Initial Manifestations of Ovarian Carcinoid Tumor.

JCEM case reports
2025

Mesenteric Fibrosis in Neuroendocrine Neoplasms: a Systematic Review of New Thoughts on Causation and Potential Treatments.

Current oncology reports
2025

Congestive Heart Failure and Arrhythmias Among Hospitalized Patients With Carcinoid Syndrome.

Cureus
2025

Frozen tricuspid valve - A case of a carcinoid syndrome.

Revista portuguesa de cardiologia : orgao oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of Cardiology
2025

Exploring the association between 91 circulating inflammatory proteins and the risk of carcinoid syndrome: a Mendelian randomization analysis.

Discover oncology
2025

Contemporary Management of Severe Carcinoid Heart Disease.

JACC. Case reports
2025

Recurrent transient visual loss in secretory carcinoid tumor.

American journal of ophthalmology case reports
2025

Endocrine paraneoplastic syndromes in lung cancer: A call for clinical vigilance (Review).

Molecular and clinical oncology
2025

Burden of Comorbidities and Concomitant Medications and Their Associated Costs in Patients with Gastroenteropancreatic or Lung Neuroendocrine Tumors: Analysis of US Administrative Data.

Advances in therapy
2025

Oral paltusotine, a nonpeptide selective somatostatin receptor 2 agonist: Mass balance, absolute bioavailability and metabolism in healthy participants.

British journal of clinical pharmacology
2025

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

Cancer treatment reviews
2025

Three strikes in a row: a challenging case of carcinoid syndrome.

European heart journal
2025

A Rare Case of Gastric Outlet Obstruction Caused by a Duodenal Carcinoid.

Cureus
2025

Factors Influencing Costs of Cancer Care for Patients with Neuroendocrine Neoplasms.

Neuroendocrinology
2025

Mixed Trabecular and Mucinous Carcinoid Arising in the Background of a Mature Ovarian Cystic Teratoma.

Cureus
2025

The exceptionally rare phenomenon of well-differentiated colon neuroendocrine tumors.

Journal of neuroendocrinology
2025

[An unusual tricuspid regurgitation].

Giornale italiano di cardiologia (2006)
2024

The Value of Repeat 5-HIAA Measurements as a Predictor of Carcinoid Heart Disease: A Prospective 5-Year Follow-Up Study in Patients with Small Intestinal Neuroendocrine Tumors.

Cancers
2025

A post-modified lanthanide metal-organic frameworks as ratiometric luminescent sensor for the visual detection of 5-hydroxytryptamine.

Journal of hazardous materials
2025

Gut microbial and functional alterations lead to metagenomic signatures for midgut neuroendocrine tumor patients and for carcinoid syndrome.

Endocrine-related cancer
2024

Carcinoid Heart Disease Associated with Primary Ovarian Carcinoid Tumor: A Rare Presentation.

Heart views : the official journal of the Gulf Heart Association
2024

Exploring Carcinoid Syndrome in Neuroendocrine Tumors: Insights from a Multidisciplinary Narrative Review.

Cancers
2024

Cardiac Surgery and Transcatheter Intervention for Valvular Heart Disease in Carcinoid Syndrome: Risk Factors, Outcomes, and Evolving Therapeutic Strategies.

Journal of cardiovascular development and disease
2024

Skin as a Reflection of Gut Health: An Overview of Dermatological Manifestations in Primary Neoplastic and Autoimmune Gastrointestinal Disorders.

Cureus
2025

Surgical Management of Neuroendocrine Tumor Liver Metastases.

Hematology/oncology clinics of North America
2024

Carcinoid heart findings in vasoactive intestinal peptide-secreting tumour.

BMJ case reports
2024

Synchronous bilateral typical pulmonary carcinoid tumours diagnosed by robotic navigation bronchoscopy: A unique case.

Respirology case reports
2025

Percutaneous bicaval valve implantation for severe tricuspid regurgitation due to carcinoid syndrome.

Singapore medical journal
2025

Carcinoid syndrome presenting as diffuse violaceous cutaneous flushing.

The British journal of dermatology
2024

Carcinoid heart disease in patients with advanced small-intestinal neuroendocrine tumors and carcinoid syndrome: a retrospective experience from two European referral centers.

ESMO open
2024

Atypical morphoea, a herald of two malignancies: Lung adenocarcinoma and a neuroendocrine tumour.

Journal of scleroderma and related disorders
2024

Biomarkers to Inform Prognosis and Treatment for Unresectable or Metastatic GEP-NENs.

JAMA oncology
2024

Horizontal partial laryngectomy for laryngeal neuroendocrine tumor: A case report.

International journal of surgery case reports
2024

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

JCEM case reports
2024

The challenging differential diagnosis of recurrent flushing episodes: systemic mastocytosis mimicking carcinoid syndrome.

Endokrynologia Polska
2024

Management of functional neuroendocrine tumors.

Current problems in cancer
2025

Severe tricuspid regurgitation and right heart failure secondary to carcinoid heart disease.

Acta cardiologica
2024

A case of carcinoid syndrome after CT guided lung biopsy of a neuroendocrine tumor.

Radiology case reports
2024

Surgical treatment of neuroendocrine tumors.

Current problems in cancer
2024

Management of carcinoid heart disease.

Current problems in cancer
2024

Ileo-Ileal Intussusception Secondary to Neuroendocrine Tumor in Adult Patient.

The American journal of case reports
2024

Anesthetic key points in a patient with a terminal ileum neuroendocrine tumor and a rare carcinoid left heart disease presented for non-cardiac surgery: case report.

BMC anesthesiology
2024

Sarcopenia in Patients with Advanced Gastrointestinal Well-Differentiated Neuroendocrine Tumors.

Nutrients
2024

[Anaphylactic shock after mistletoe therapy].

Innere Medizin (Heidelberg, Germany)
2024

Carcinoid Heart Disease and a Complicated Course of Progressive Gastroenteropancreatic Neuroendocrine Neoplasia: A Case Report.

Case reports in oncology
2024

[Appendix ... it's not just appendicitis: neuroendocrine tumors.].

Recenti progressi in medicina
2024

Occult Pancreatic Neuroendocrine Tumor Presenting as Carcinoid Syndrome.

Cureus
2024

Rare Case of Neuroendocrine Metastasis to the Left Ventricle.

JACC. Case reports
2024

Carcinoid crisis: The challenge is still there.

Endocrinologia, diabetes y nutricion
2024

European Neuroendocrine Tumor Society (ENETS) 2024 guidance paper for the management of well-differentiated small intestine neuroendocrine tumours.

Journal of neuroendocrinology
2024

Unmasking Carcinoid Syndrome in a Chronic Obstructive Pulmonary Disease (COPD) Patient: A Rare Presentation with Wheezing and Angioedema.

Cureus
2024

Primary Neuroendocrine Tumor of the Testis Associated With Cardiac Symptoms: A Case Report and Literature Review.

Cureus
2024

Unique Presentation of Adult Ileocecal Intussusception Unveiling a Rare Culprit: A Carcinoid Tumor.

Cureus
2024

[Clinical significance of neuroendocrine tumors : Incidence, symptoms, diagnosis, stage, and prognostic factors and their influence on disease management].

Radiologie (Heidelberg, Germany)
2024

Carcinoid syndrome with right-sided valve involvement - a case report and review of the literature.

Klinicka onkologie : casopis Ceske a Slovenske onkologicke spolecnosti
2024

Elevated sortilin expression discriminates functional from non-functional neuroendocrine tumors and enables therapeutic targeting.

Frontiers in endocrinology
2024

Normalization of Prediabetic Hemoglobin A1c (HbA1c) Levels After the Surgical Removal of a Serotonin-Secreting Neuroendocrine Tumor.

Cureus
2024

Value of Surgical Cytoreduction in Patients with Small Intestinal Neuroendocrine Tumors Metastatic to the Liver and Peritoneum.

Annals of surgical oncology
2024

Liver-Directed Locoregional Therapies for Neuroendocrine Liver Metastases: Recent Advances and Management.

Current oncology (Toronto, Ont.)
2025

Gastric neuroendocrine tumor presenting with carcinoid syndrome.

Revista espanola de enfermedades digestivas
2025

Perioperative management of a double valve replacement and coronary artery bypass graft in a patient with carcinoid syndrome; a case report and literature review.

Perfusion
2024

Comparison of Octreotide and Vasopressors as First-Line Treatment for Intraoperative Carcinoid Crisis.

Annals of surgical oncology
2024

Managing end-stage carcinoid heart disease: A case report and literature review.

World journal of gastrointestinal oncology
2024

A systematic review and meta-analysis of the diagnosis and surgical management of carcinoid heart disease.

Frontiers in cardiovascular medicine
2024

From biology to clinical practice: antiproliferative effects of somatostatin analogs in neuroendocrine neoplasms.

Therapeutic advances in medical oncology
2024

Benign or by Chance: A Case Report on Incidental Appendectomy Revealing a Neuroendocrine Tumor During Traumatic Exploratory Laparotomy.

Cureus
2024

The skin as a window to the gut: A case of carcinoid syndrome.

Clinical case reports
2024

Does Telotristat Have a Role in Preventing Carcinoid Heart Disease?

International journal of molecular sciences
2024

Use of perioperative telotristat in a patient with carcinoid heart disease.

Endocrinology, diabetes &amp; metabolism case reports
2024

Plasma tryptophan pathway metabolites quantified by liquid chromatography-tandem mass spectrometry as biomarkers in neuroendocrine tumor patients.

Journal of neuroendocrinology
2023

The cause-and-effect relationship between gut microbiota abundance and carcinoid syndrome: a bidirectional Mendelian randomization study.

Frontiers in microbiology
2024

NET-Induced Carcinoid Heart Disease Affecting Both Tricuspid and Aortic Valves Due to Patent Foramen Ovale and Right/Left Shunt: A Multi-imaging Challenge to Nuclear Medicine.

Clinical nuclear medicine
2024

Resistant gastroenteropancreatic neuroendocrine tumors: a definition and guideline to medical and surgical management.

Proceedings (Baylor University. Medical Center)
2024

A Diagnostic Dilemma: Metastatic Neuroendocrine Tumor Mimicking Hepatocellular Carcinoma.

Rhode Island medical journal (2013)
2023

Ovarian metastasis in a patient with neuroendocrine tumour of the small intestine.

Endokrynologia Polska
2024

Carcinoid crisis induced by radioligand therapy: a rare but life-threatening complication in patient with neuroendocrine tumor.

Nuclear medicine review. Central &amp; Eastern Europe
2023

Plasma or serum 5-hydroxyindoleacetic acid can be used interchangeably in patients with neuroendocrine tumours.

Scandinavian journal of clinical and laboratory investigation
2024

Midgut neuroendocrine tumor patients have a depleted gut microbiome with a discriminative signature.

European journal of cancer (Oxford, England : 1990)
2023

Treatment Patterns of Long-Acting Somatostatin Analogs for Neuroendocrine Tumors.

Journal of health economics and outcomes research
2024

Smart dual imprinted Origami 3D-ePAD for selective and simultaneous analysis of vanillylmandelic acid and 5-hydroxyindole-3-acetic acid carcinoid cancer biomarkers using graphene quantum dots coated with dual molecularly imprinted polymers.

Talanta
2023

Strongyloidiasis Mimicking Carcinoid Syndrome in an Immunocompetent Host.

Cureus
2023

Carcinoid heart disease: a potentially fatal complication of carcinoid syndrome.

BMJ case reports
2024

What Is Carcinoid Syndrome? A Critical Appraisal of Its Proposed Mediators.

Endocrine reviews
2023

Lung Carcinoids: A Comprehensive Review for Clinicians.

Cancers
2023

Structure-Based Design of Xanthine-Imidazopyridines and -Imidazothiazoles as Highly Potent and In Vivo Efficacious Tryptophan Hydroxylase Inhibitors.

Journal of medicinal chemistry
2023

Pancreatic Neuroendocrine Tumor (Pan-NET) Presented by Abdominal Pain: A Case Report and Literature Review.

Journal of clinical medicine
2024

Gender-related differences in patients with carcinoid syndrome: new insights from an Italian multicenter cohort study.

Journal of endocrinological investigation
2023

Masquerading as Pneumonia: A Lung Neuroendocrine Tumor Case Report.

Cureus
2023

Successful treatment of facial telangiectasia from carcinoid syndrome with pulsed dye laser therapy.

JAAD case reports
2023

Prevalence, one-year-incidence and predictors of carcinoid heart disease.

Cardiovascular ultrasound
2023

Carcinoid syndrome caused by a pulmonary carcinoid mimics intestinal manifestations of ulcerative colitis: A case report.

World journal of gastroenterology
2023

Metastatic well differentiated serotonin-producing pancreatic neuroendocrine tumor with carcinoid heart disease: a case report.

Journal of gastrointestinal oncology
2023

Treatment of idiopathic anaphylaxis with dupilumab: a case report.

Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology
2023

A Case of Functional Bowel Disease Misdiagnosed as Carcinoid Syndrome.

ACG case reports journal
2023

Improving outcomes in carcinoid heart disease - learning from a single centre.

Nuclear medicine communications
2023

Carcinoid syndrome-Symptoms and therapeutic approaches.

Hellenic journal of nuclear medicine
2023

High Tumor Uptake on 18F-FDOPA PET/CT Indicates Poor Prognosis in Patients with Metastatic Midgut Neuroendocrine Tumors: A Study from the Groupe d'étude des Tumeurs Endocrines and ENDOCAN-RENATEN Network.

Journal of nuclear medicine : official publication, Society of Nuclear Medicine
2023

Urinary 5-Hydroxyindolacetic Acid Measurements in Patients with Neuroendocrine Tumor-Related Carcinoid Syndrome: State of the Art.

Cancers
2023

Conventional Transarterial Chemo embolization Using Streptozocin in Patients with Unresectable Neuroendocrine Liver Metastases.

Cancers
2023

Combination Systemic Therapies in Advanced Well-Differentiated Gastroenteropancreatic Neuroendocrine Tumors (GEP-NETs): A Comprehensive Review of Clinical Trials and Prospective Studies.

Biology
2024

A case of carcinoid syndrome probably exacerbated by hemodialysis in which prochlorperazine maleate was effective.

CEN case reports
2023

Patent foramen ovale in carcinoid heart disease: The potential role for and risks of percutaneous closure prior to cardiothoracic surgery.

Journal of neuroendocrinology
2023

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

Journal of neuroendocrinology
2024

Appendiceal Neuroendocrine Neoplasms: A Comprehensive Review.

Journal of computer assisted tomography
2023

A Comprehensive Review on Neuroendocrine Neoplasms: Presentation, Pathophysiology and Management.

Journal of clinical medicine
2023

NT-proBNP as a neuroendocrine tumor biomarker: beyond heart failure.

Endocrine connections
2023

Health care resource use and costs among patients with carcinoid syndrome in France: Analysis of the National Health Insurance Database.

Clinics and research in hepatology and gastroenterology
2023

The role of serotonin inhibition within the treatment of carcinoid syndrome.

Endocrine oncology (Bristol, England)
2023

Breakthrough Symptoms Remain an Unmet Need in Symptomatic Patients With Neuroendocrine Tumors and Carcinoid Syndrome.

Pancreas
2023

Surgery Improves Patient-Reported Outcomes in Patients with Intestinal and Pancreatic NETs: A Prospective Analysis.

Annals of surgical oncology
2023

Effective strategies for adequate control of hormonal secretion in functioning neuroendocrine neoplasms.

Best practice &amp; research. Clinical endocrinology &amp; metabolism
2023

Late-stage diagnosis of carcinoid heart disease due to lack of access to health care.

Cardio-oncology (London, England)
2023

Therapy Sequencing in Patients With Advanced Neuroendocrine Neoplasms.

American Society of Clinical Oncology educational book. American Society of Clinical Oncology. Annual Meeting
2023

Carcinoid Heart Disease Management: A Multi-Disciplinary Collaboration.

The oncologist
2023

Primary Well-Differentiated Neuroendocrine Tumor of the Kidney.

Journal of kidney cancer and VHL
2023

Testicular Neuroendocrine Tumors: A Case Report and Literature Review.

Cureus
2023

Small bowel neuroendocrine tumours - casting the net wide.

Current opinion in gastroenterology
2023

Impact of Atrial Fibrillation on Patients With Carcinoid Syndrome: A National Inpatient Sample Analysis.

The American journal of cardiology
2023

Targeting neuroendocrine tumors with octreotide and lanreotide: Key points for clinical practice from NET specialists.

Cancer treatment reviews
2023

Inhibition of serotonin biosynthesis in neuroendocrine neoplasm suppresses tumor growth in vivo.

bioRxiv : the preprint server for biology
2023

Outcome of carcinoid heart syndrome in patients enrolled in the SwissNet cohort.

BMC cancer
2023

Sporadic pancreatic neuroendocrine neoplasms: A retrospective clinicopathological and outcome analysis from a Latvian study group.

Frontiers in surgery
2023

Carcinoid syndrome in a HIV-infected patient: where you least expect it.

AIDS (London, England)
2023

Carcinoid heart disease in patients with midgut neuroendocrine tumours.

Journal of neuroendocrinology
2023

Prescribing Characteristics of Octreotide Immediate-Release and Long-Acting Release in Patients with Neuroendocrine Tumors.

The oncologist
2023

Carcinoid Heart Disease-Induced Right-Sided Heart Failure as a Culprit for Significant Ascites.

Case reports in gastroenterology
2023

Diagnosis and management of neuroendocrine tumours.

Clinical medicine (London, England)
2023

Carcinoid syndrome and somatostatin analogues.

Revista espanola de enfermedades digestivas
2023

Telotristat Etiprate alleviates rheumatoid arthritis by targeting LGALS3 and affecting MAPK signaling.

Intractable &amp; rare diseases research
2022

Liver transplantation in gastroenteropancreatic neuroendocrine tumors.

Frontiers in oncology
2023

The Role of Somatostatin Analogues in the Control of Diarrhea and Flushing as Markers of Carcinoid Syndrome: A Systematic Review and Meta-Analysis.

Journal of personalized medicine
2023

Carcinoid Syndrome: Preclinical Models and Future Therapeutic Strategies.

International journal of molecular sciences
2023

Analysis of Patients with NET G1/G2 Neuroendocrine Tumors of the Small Intestine in the Course of Carcinoid Heart Disease-A Retrospective Study.

Journal of clinical medicine
2022

Bioprosthetic valve monitoring in patients with carcinoid heart disease.

Frontiers in cardiovascular medicine
2023

Discovery of Paltusotine (CRN00808), a Potent, Selective, and Orally Bioavailable Non-peptide SST2 Agonist.

ACS medicinal chemistry letters
2023

Balancing carcinoid crisis and right ventricular dysfunction during tricuspid and pulmonic valve replacement for carcinoid heart disease: A case report.

International journal of surgery case reports
2023

Neuroendocrine Tumor Presenting as Hypoxic Respiratory Failure in a Patient with Patent Foramen Ovale.

The Brown journal of hospital medicine
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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. Oxalate nephropathy from untreated pancreatic insufficiency in a neuroendocrine tumour patient.
    BMJ case reports· 2026· PMID 41748253mais citado
  2. Real-World Observational Study of Somatostatin Analogs and Rescue Medication Usage for Neuroendocrine Tumors in Canada.
    Neuroendocrinology· 2026· PMID 41843715mais citado
  3. Recurrent ventricular asystole during octreotide infusion in a patient with pancreatic neuroendocrine tumor: a case report.
    Journal of medical case reports· 2026· PMID 41840662mais citado
  4. Determining Prognosis in Patients With Carcinoid Syndrome: A Retrospective Single-Center Cohort Study.
    Journal of the National Comprehensive Cancer Network : JNCCN· 2026· PMID 41825127mais citado
  5. Multivalvular Cardiac Involvement from Giant Hepatic Metastases of an Ileal Neuroendocrine Tumor.
    Cureus· 2026· PMID 41822627mais citado
  6. Neuroendocrine Tumors and Pregnancy: The French PregNET Study.
    Eur J Endocrinol· 2026· PMID 41968574recente
  7. Impact of Symptom Phenotype on Surgical Quality and Survival in Small-Bowel Neuroendocrine Tumours: A Quality Metrics Analysis.
    J Surg Oncol· 2026· PMID 41913096recente

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Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:100093(Orphanet)
  2. MONDO:0100347(MONDO)
  3. GARD:5994(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Artigo Wikipedia(Wikipedia)
  7. Q339547(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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Tumor carcinoide e síndrome carcinoide
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Tumor carcinoide e síndrome carcinoide

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