Uma condição muito rara caracterizada pela proliferação generalizada de células neuroendócrinas pulmonares. Manifesta-se como tosse progressiva e dispneia. É considerada um precursor para tumor carcinoide pulmonar.
Introdução
O que você precisa saber de cara
Visão geral
A hiperplasia difusa idiopática das células neuroendócrinas pulmonares (código MONDO: MONDO:0022986, CID-10 J84.8) é uma condição muito rara, com prevalência estimada inferior a 1 em 1.000.000 na população.[1]
Trata-se de um quadro caracterizado pela proliferação generalizada de células neuroendócrinas pulmonares, tipicamente com idade de início observada em adultos e idosos. A condição é considerada um precursor para tumor carcinoide pulmonar.[1]
Sinais e sintomas
A condição manifesta-se clinicamente de forma respiratória, apresentando tosse progressiva e dispneia (falta de ar).[1]
Diagnóstico
Conteúdo informativo gerado e mantido automaticamente a partir de fontes oficiais (Orphanet, HPO, OMIM, SUS). Não substitui avaliação médica.
A **hiperplasia difusa idiopática das células neuroendócrinas pulmonares** é uma condição respiratória muito rara em que ocorre um aumento generalizado de células neuroendócrinas nos pulmões. As pessoas afetadas costumam sentir **tosse progressiva** e **dispneia** (falta de ar ou dificuldade para respirar), geralmente com início na fase adulta ou mais velha. Essa proliferação é considerada por especialistas como precursora do tumor carcinoide do pulmão. No Brasil, a doença não conta com Protocolo Clínico e Diretrizes Terapêuticas (PCDT) do Ministério da Saúde nem com medicamentos específicos fornecidos pelo SUS, sendo o cuidado conduzido pela rede assistencial especializada.
Uma condição muito rara caracterizada pela proliferação generalizada de células neuroendócrinas pulmonares. Manifesta-se como tosse progressiva e dispneia. É considerada um precursor para tumor carcinoide pulmonar.
Tem tratamento?
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
Encontrou um erro ou informação desatualizada? Sugira uma correção →
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
Linha do tempo da pesquisa
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
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Hiperplasia difusa idiopática das células neuroendócrinas pulmonares
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
Nenhum ensaio clínico registrado para esta condição.
Publicações mais relevantes
Update on diffuse idiopathic pulmonary neuroendocrine cell hyperplasia.
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a disorder characterized by neuroendocrine cell hyperplasia, tumorlets and tumors, manifesting as lung nodules, chronic cough, and airflow obstruction. Often misdiagnosed as asthma, DIPNECH lies at the cross-section of oncology, obstructive airway disease and interstitial lung disease. With the increasing use of CT scans leading to higher rates of lung nodule detection, it is important for clinicians to be familiar with DIPNECH. The evidence base in DIPNECH is sparse and limited to retrospective case series. In the last 5 years, clinical experiences in large academic centers have been published describing variability in clinical presentation and pulmonary function, use of DOTATATE scans, efficacy of somatostatin analogs, and principles of surveillance imaging. More awareness of DIPNECH is needed among pulmonary clinicians. Apart from the usual presentation of cough with lung nodules and spirometric obstruction in women, patients also present with dyspnea and/or restrictive patterns on PFTs. Variability in diagnosis and management is widespread. Multidisciplinary assessment is helpful in guiding management. Multicenter and multispecialty collaboration is needed to establish best practices and improve clinical management.
Clinical characteristics of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia.
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is characterised by diffuse bronchial hyperplasia of pulmonary neuroendocrine cells, which are situated within the walls of bronchi and bronchioles. Presenting symptoms are nonspecific and the clinical course varies, making diagnosis challenging. We sought to describe the clinical characteristics of patients with DIPNECH in a large multinational case series to guide and inform future care and research. Data were collated from 18 international centres. Information collected included disease presentation, pulmonary function testing, histopathology, radiological patterns and outcomes. The relationship between clinical features, radiology and symptoms were explored in parametric and nonparametric group-wise analyses, univariate linear regressions, and multivariate binomial logistic regression. The mean±sd age of the 258 patients in this study was 63.3±10.6 years and 93.4% were female. Diffuse pulmonary nodules (98.8%) and mosaic attenuation (59.1%) were the most common radiological findings and 29.5% had obstructive spirometry with a mean±sdforced expiratory volume in 1 s (FEV1) % pred of 69.0±23.7%. There was a significant association between the number of nodules and a reduction in FEV1 % pred (p<0.001), while the presence of bronchial wall thickening on imaging was most closely associated with cough (OR 4.97, p=0.001) dyspnoea (OR 3.14, p=0.003) and bronchodilator responsiveness (OR 3.09, p=0.013). Approximately half of patients treated with inhaled beta agonist and corticosteroids (46.3%) or somatostatin analogue (54.1%) reported improvement in symptoms. The presence of radiological bronchial wall thickening is associated with the presence of symptoms, while mosaic attenuation is correlated with airflow obstruction; hence, the presence of these radiological findings has the potential to guide possible treatment decisions.
LUNG CARCINOIDS (Lung Neuroendocrine Tumors).
This narrative review written by experts aims to provide a comprehensive synthesis of current knowledge and guidelines on lung carcinoids, emphasizing the multidimensional approach required for effective diagnosis and management. The manuscript will: i) Define and classify lung carcinoids in accordance with the latest WHO classification system, and emphasize the importance of molecular markers for precise subtyping; ii) Analyze epidemiological trends, discuss prevalence, incidence, and mortality rates, alongside key risk factors including genetic predispositions (MEN1) and precursor lesions such as diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH); iii) Investigate the etiology and carcinogenesis of lung carcinoids, including insights into the molecular hallmarks and pathways that underlie tumor development and progression; iv) Detail clinical presentations, distinguishing pulmonary symptoms from secretory syndromes (carcinoid syndrome and Cushing's syndrome), supported by visual aids to elucidate symptom prevalence and implications; v) Present an integrated diagnostic approach, encompassing state-of-the-art imaging modalities (CT, MRI, 68Ga-SSA-PET-CT) and advanced molecular pathology techniques; vi) Outline evidence-based treatment strategies, addressing early-stage interventions, systemic therapies, locoregional therapies, and emerging modalities such as immunotherapy and agnostic approaches; vii) Explore the role of multidisciplinary management, highlighting the critical importance of tumor boards dedicated to lung carcinoids and provide a checklist of key items for comprehensive case discussion and decision-making; viii) Conclude with future perspectives, identifying gaps in current knowledge and suggest directions for research to enhance diagnosis, treatment, and overall patient quality of life.
Transbronchial cryobiopsy to diagnose diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH).
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare precursor of neuroendocrine malignancy. Diagnosis is difficult as clinical and radiological findings can be non-specific. Histological confirmation is necessary, and surgical lung biopsy is still considered the gold standard technique to aid multidisciplinary discussion. There are increasing reports of using transbronchial lung cryobiopsy (TBLC) for tissue sampling, allowing a feasible and safe alternative to diagnosis. Here we present a case of cough, dyspnoea and hypoxia in a woman ultimately diagnosed with DIPNECH using TBLC.
Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia Mimicking Lung Cancer: A Case Report.
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is an extremely uncommon and incompletely elucidated pulmonary condition arising from neuroendocrine cells within the airway epithelium. We report the case of a 65-year-old woman who presented to our thoracic surgery department with a history of hemoptysis following the incidental discovery of a solitary pulmonary nodule in the right middle lobe, highly suspicious for lung cancer. After a thorough diagnostic workup, including positron emission tomography-computed tomography (PET-CT), our multidisciplinary team recommended a right middle lobectomy. The histopathological examination of the resected specimen confirmed DIPNECH without evidence of an associated carcinoid tumor. Given the potential for progression to neuroendocrine neoplasm, the development of evidence-based management protocols for DIPNECH remains essential. This case highlights the atypical and often silent presentation of this rare lung pathology and emphasizes the importance of considering DIPNECH in the differential diagnosis of asymptomatic patients presenting with solitary pulmonary nodules.
Publicações recentes
Pulmonary Carcinoid Tumors and DIPNECH: Surgical Perspectives on a Rare and Evolving Spectrum of Lung Neuroendocrine Disease.
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) mimicking poorly controlled asthma: CT findings.
🥉 Relato de casoUpdate on diffuse idiopathic pulmonary neuroendocrine cell hyperplasia.
Clinical characteristics of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia.
LUNG CARCINOIDS (Lung Neuroendocrine Tumors).
📚 EuropePMC109 artigos no totalmostrando 102
Update on diffuse idiopathic pulmonary neuroendocrine cell hyperplasia.
Current opinion in pulmonary medicineClinical characteristics of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia.
ERJ open researchLUNG CARCINOIDS (Lung Neuroendocrine Tumors).
Endocrine reviewsDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia Mimicking Lung Cancer: A Case Report.
CureusDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia associated with lung adenocarcinoma: clinical, pathological and oncological outcomes from a multicentre analysis.
ERJ open researchDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: An Uncommon Clinical Challenge.
CureusDIPNECH: The neuroendocrine enigma behind persistent respiratory symptoms in the post-COVID era.
Respiratory medicine case reportsEctopic Cushing's syndrome in a patient with DIPNECH and metastatic lung carcinoid.
Endocrinologia, diabetes y nutricionTransbronchial cryobiopsy to diagnose diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH).
BMJ case reportsPulmonary neuroendocrine cells: Spectrum of diseases and their radiological-pathological correlations.
Radiologia[Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH)].
Revue des maladies respiratoiresEmbolization of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia.
Journal of vascular and interventional radiology : JVIRDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia in a male patient associated with pulmonary adenocarcinoma.
Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e TisilogiaAsthma and respiratory comorbidities.
The Journal of allergy and clinical immunologyCoexistence of DIPNECH with pulmonary adenocarcinoma: Coincidence or by design?
HeliyonA Case of Unknown Pulmonary Nodules.
Journal of investigative medicine high impact case reportsDipnech: A Rare Cause of Slow-Growing Pulmonary Nodules in a Dyspnoeic Patient with a History of Breast Cancer.
European journal of case reports in internal medicineA Unique Association of Right-Sided Aortic Arch With Kommerell's Diverticulum, Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia, and Typical Carcinoid: A Case Report.
CureusA Rare Case of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: A Case Report.
Cureus[Pulmonary neuroendocrine tumors : Current review].
Radiologie (Heidelberg, Germany)Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: A Blanked Out Pulmonary Neuroendocrine Tumor.
Case reports in oncologyMultiple pulmonary nodules with diffuse idiopathic pulmonary neuroendocrine cell hyperplasia and minute pulmonary meningothelial-like nodules.
Respirology case reportsA multi-lesional analysis of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: a single-centre analysis.
Clinical radiologyRare Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) Finding in a 9/11 World Trade Center Survivor: A Case Report.
CureusRadiological-pathological correlation in diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH): imaging and histopathology.
Clinical radiologyDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) Progressing to Carcinoid Tumor: A Case of Chronic Cough.
CureusDIPNECH: pragmatic approach, uncertainties, notable associations, and a proposal for an improved definition.
Endocrine-related cancerDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognized entity-a report of two cases.
Indian journal of thoracic and cardiovascular surgeryPulmonary Function Tests as a Biomarker in Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia Patients Treated With Somatostatin Analogues.
CureusA multidisciplinary approach to the work up and management of pulmonary carcinoid tumors and DIPNECH: a narrative review.
Translational lung cancer researchQuantitative Airway Assessment of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) on CT as a Novel Biomarker.
Diagnostics (Basel, Switzerland)Plasma Progastrin-Releasing Peptide and Chromogranin A Assays for Diagnosing and Monitoring Lung Well-Differentiated Neuroendocrine Tumors: A Brief Report.
Journal of thoracic oncology : official publication of the International Association for the Study of Lung CancerDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH): An Underrecognized Cause of Multiple Lung Nodules and Mosaic Perfusion Pattern.
Journal of the Belgian Society of RadiologyDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia: a rare cause of airflow limitation.
BMJ case reportsDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH): a little known cause of thoracic lesions.
BMJ case reportsDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia presenting as a solitary lung nodule: a rare histopathological diagnosis.
Oxford medical case reportsLung adenocarcinoma coexisting with diffuse idiopathic pulmonary neuroendocrine cell hyperplasia manifesting as multiple pulmonary nodules: A case report.
Thoracic cancerDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH): Prevalence, clinicopathological characteristics and survival outcome in a cohort of 311 patients with well-differentiated lung neuroendocrine tumours.
Journal of neuroendocrinologyA case of multiple lung carcinoid tumors localized in the right lower lobe.
Respiratory medicine case reportsNot All Multifocal Pulmonary Neuroendocrine Cell Proliferations Represent Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia.
The Annals of thoracic surgeryDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia: A rare and under-diagnosed condition.
Pulmonology[Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia/tumorlets].
MedicinaDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH): A Case of Indolent Pulmonary Nodules Diagnosed with Robotic-Assisted Navigational Bronchoscopy.
Case reports in pulmonologyIs the combination of bilateral pulmonary nodules and mosaic attenuation on chest CT specific for DIPNECH?
Orphanet journal of rare diseasesDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia: clinical characteristics and progression to carcinoid tumour.
The European respiratory journal[Your images. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia].
La Revue du praticienWell-Differentiated Bronchopulmonary Neuroendocrine Tumors: More Than One Entity.
Journal of thoracic oncology : official publication of the International Association for the Study of Lung CancerLung carcinoid tumors with Diffuse Idiopathic Pulmonary NeuroEndocrine Cell Hyperplasia (DIPNECH) exhibit pejorative pathological features.
Lung cancer (Amsterdam, Netherlands)Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: DIPNECH.
Current opinion in pulmonary medicineDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia With Progression to Neuroendocrine Tumor.
Cureus[Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia with tumorlet formation: A case report and review of the literature].
Revista espanola de patologia : publicacion oficial de la Sociedad Espanola de Anatomia Patologica y de la Sociedad Espanola de CitologiaAn Update on the Management of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH).
Current treatment options in oncologyConstrictive bronchiolitis in diffuse idiopathic pulmonary neuroendocrine cell hyperplasia.
ERJ open researchRare diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: one case report and literature review.
Translational cancer researchDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Imaging and Clinical Features of a Frequently Delayed Diagnosis.
AJR. American journal of roentgenologyClinical Characteristics of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: A Retrospective Analysis.
ChestRecent advances in the understanding of bronchiolitis in adults.
F1000ResearchCoexistence of DIPNECH and carotid body paraganglioma: is it just a coincidence?
Endocrinology, diabetes & metabolism case reportsDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: A Clinical Case.
European journal of case reports in internal medicineSomatostatin Analogs Improve Respiratory Symptoms in Patients With Diffuse Idiopathic Neuroendocrine Cell Hyperplasia.
ChestDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia in a young man with hypoxia: a case report and review of the literature.
Acta bio-medica : Atenei Parmensis[A case of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) syndrome].
Revue des maladies respiratoiresDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia, Mimicking Pulmonary Metastasis.
International journal of surgical pathologyDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: An Asthma Mimicker.
The American journal of medicineDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Review of the Literature and a Single-center Experience.
CureusDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) with liver metastases.
BMJ case reportsDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia: Unusual presentation.
Annals of thoracic medicineDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia: Case series and a review of the literature.
MedicineManagement of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Review and a Single Center Experience.
LungDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia on Somatostatin Receptor Imaging.
American journal of respiratory and critical care medicineA Rare Case of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia.
CureusDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia Causing Chronic Cough.
Archivos de bronconeumologiaDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia: Report of two cases.
Medicina clinicaSeries of rare lung diseases mimicking imaging patterns of common diffuse parenchymal lung diseases.
Lung India : official organ of Indian Chest SocietyDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia Syndrome Treated With Sirolimus.
Annals of internal medicineDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia mimicking metastatic disease.
PulmonologyDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) Syndrome and Carcinoid Tumors With/Without NECH: A Clinicopathologic, Radiologic, and Immunomolecular Comparison Study.
The American journal of surgical pathologyMolecular alterations of neuroendocrine tumours of the lung.
HistopathologyPrognostic Factors in Typical and Atypical Pulmonary Carcinoids.
Ochsner journalAtypical carcinoid localized at the bronchus accompanied by diffuse idiopathic pulmonary neuroendocrine cell hyperplasia in the distal lung: a rare case report.
Journal of thoracic disease[Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (dipnech) - an overview of the cases diagnosed at the department of thoracic surgery in the years 2010-2014].
Wiadomosci lekarskie (Warsaw, Poland : 1960)Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia diagnosed by tranbronchoscopic cryoprobe biopsy technique.
Respirology case reportsDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: A Case Report and Review of the Literature.
The American journal of case reportsAssociation of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) with lung adenocarcinoma: A radiologist's perspective.
Lung cancer (Amsterdam, Netherlands)68Gallium DOTANOC-PET Imaging in Lung Carcinoids: Impact on Patients' Management.
NeuroendocrinologyDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia diagnosed by transbronchial lung cryobiopsy: a case report.
Journal of medical case reportsDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia and Granulomatous Inflammation Mimicking High-Grade Malignancy on FDG-PET/CT.
Clinical nuclear medicineDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: A Case With Long-term Follow-up.
Journal of thoracic imagingDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia and Neuroendocrine Hyperplasia of Infancy.
Clinics in chest medicineExpression of developing neural transcription factors in diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH).
Virchows Archiv : an international journal of pathologyDiffuse Neuroendocrine Cell Hyperplasia: Report of Two Cases.
Case reports in pathologyA case of DIPNECH presenting as usual interstitial pneumonia.
Pneumonologia i alergologia polskaDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome.
The European respiratory journal[Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: Usefulness of espiratory chest CT].
Medicina clinica[Multifocal Pulmonary Neuroendocrine Tumours: Genesis, Diagnostics and Treatment].
Pneumologie (Stuttgart, Germany)DIPNECH: Association Between Histopathology and Clinical Presentation.
LungDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH)--An uncommon precursor of a common cancer?
Pathology, research and practicePulmonary Peripheral Carcinoids after Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia and Tumorlets: Report of 3 Cases.
Case reports in pulmonologyDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) and multiple pulmonary epithelioid hemangioendothelioma (PEH): a case report.
PathologicaDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH).
Seminars in diagnostic pathologyDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) and the Role of Somatostatin analogs: A Case Series.
LungDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia of the Lung (DIPNECH): Current Best Evidence.
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Doenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Ainda não achamos doenças com sintomas parecidos o suficiente.
Perguntas frequentes
O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu
Trata-se de uma proliferação difusa de células neuroendócrinas no tecido pulmonar. A condição é considerada precursora de tumor carcinoide pulmonar e acomete adultos e idosos.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
3 fontes citadas no texto · 7 publicações do grafo RarasNet (PubMed) · 3 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
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.
Citar este verbete
Raras. (2026). Hiperplasia difusa idiopática das células neuroendócrinas pulmonares. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/hiperplasia-difusa-idiopatica-das-celulas-neuroendocrinas-pulmonares
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Hiperplasia difusa idiopática das células neuroendócrinas pulmonares
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- Codificação WHO/SUS
- fonte: WHO ICD-10 / DATASUS
- CID-11 (futuro)
- fonte: WHO ICD-11
- NIH/GARD
- fonte: GARD (NIH)