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Hiperplasia difusa idiopática das células neuroendócrinas pulmonares
ORPHA:617916CID-10 · J84.8CID-11 · 2F00.YDOENÇA RARA
Início adulta

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.

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Introdução

O que você precisa saber de cara

Mantido pelo Disease Twin100% com fonte · atualizado 27/09/2026Revisão médica pendente
Informacoes curadas por IA — podem conter imprecisoes

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

No contexto diagnóstico laboratorial e molecular, existem 5 testes genéticos catalogados para a avaliação associada a essa condição.[1][2]

Fontes numeradas em Referências.

Conteúdo informativo gerado e mantido automaticamente a partir de fontes oficiais (Orphanet, HPO, OMIM, SUS). Não substitui avaliação médica.

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EM LINGUAGEM SIMPLES

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.

Informacoes curadas por IA — podem conter imprecisoes
[1] Orphanet [17] Ministério da Saúde — lista oficial de…
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Informacoes curadas por IA — podem conter imprecisoes

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.

Publicações científicas
159 artigos
Último publicado: 2026 Apr 7
Medicamentos
20 com registro
CLORIDRATO DE AMBROXOL, ESPECBAC, TOSSLAB

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CLORIDRATO DE AMBROXOLESPECBACTOSSLABFLUIBRONFLUISOLVANAMBROLSEDAVANMUCOXOLAN

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
Worldwide
Casos conhecidos
100
pacientes catalogados
Início
Adult
+ elderly
🏥
SUS: Cobertura mínimaScore: 0%
CID-10: J84.8
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

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Sinais e sintomas

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

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico159PubMed
Últimos 10 anos103publicações
Pico202213 papers
Linha do tempo
2026Hoje · 2026📈 2022Ano 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

Carregando informações de tratamento...

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

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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.

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Publicações mais relevantes

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

Update on diffuse idiopathic pulmonary neuroendocrine cell hyperplasia.

Current opinion in pulmonary medicine2026 Mar 04

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.

#2

Clinical characteristics of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia.

ERJ open research2026 Jan

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.

#3

LUNG CARCINOIDS (Lung Neuroendocrine Tumors).

Endocrine reviews2026 Jan 12

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.

#4

Transbronchial cryobiopsy to diagnose diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH).

BMJ case reports2025 Jul 17

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.

#5

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia Mimicking Lung Cancer: A Case Report.

Cureus2025 Dec

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

Ver todas no PubMed

📚 EuropePMC109 artigos no totalmostrando 102

2026

Update on diffuse idiopathic pulmonary neuroendocrine cell hyperplasia.

Current opinion in pulmonary medicine
2026

Clinical characteristics of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia.

ERJ open research
2026

LUNG CARCINOIDS (Lung Neuroendocrine Tumors).

Endocrine reviews
2025

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia Mimicking Lung Cancer: A Case Report.

Cureus
2025

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia associated with lung adenocarcinoma: clinical, pathological and oncological outcomes from a multicentre analysis.

ERJ open research
2025

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: An Uncommon Clinical Challenge.

Cureus
2025

DIPNECH: The neuroendocrine enigma behind persistent respiratory symptoms in the post-COVID era.

Respiratory medicine case reports
2025

Ectopic Cushing's syndrome in a patient with DIPNECH and metastatic lung carcinoid.

Endocrinologia, diabetes y nutricion
2025

Transbronchial cryobiopsy to diagnose diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH).

BMJ case reports
2025

Pulmonary neuroendocrine cells: Spectrum of diseases and their radiological-pathological correlations.

Radiologia
2025

[Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH)].

Revue des maladies respiratoires
2025

Embolization of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia.

Journal of vascular and interventional radiology : JVIR
2025

Diffuse 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 Tisilogia
2025

Asthma and respiratory comorbidities.

The Journal of allergy and clinical immunology
2024

Coexistence of DIPNECH with pulmonary adenocarcinoma: Coincidence or by design?

Heliyon
2024

A Case of Unknown Pulmonary Nodules.

Journal of investigative medicine high impact case reports
2024

Dipnech: 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 medicine
2024

A Unique Association of Right-Sided Aortic Arch With Kommerell's Diverticulum, Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia, and Typical Carcinoid: A Case Report.

Cureus
2024

A Rare Case of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: A Case Report.

Cureus
2024

[Pulmonary neuroendocrine tumors : Current review].

Radiologie (Heidelberg, Germany)
2024

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: A Blanked Out Pulmonary Neuroendocrine Tumor.

Case reports in oncology
2024

Multiple pulmonary nodules with diffuse idiopathic pulmonary neuroendocrine cell hyperplasia and minute pulmonary meningothelial-like nodules.

Respirology case reports
2024

A multi-lesional analysis of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: a single-centre analysis.

Clinical radiology
2024

Rare Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) Finding in a 9/11 World Trade Center Survivor: A Case Report.

Cureus
2024

Radiological-pathological correlation in diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH): imaging and histopathology.

Clinical radiology
2023

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) Progressing to Carcinoid Tumor: A Case of Chronic Cough.

Cureus
2023

DIPNECH: pragmatic approach, uncertainties, notable associations, and a proposal for an improved definition.

Endocrine-related cancer
2023

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognized entity-a report of two cases.

Indian journal of thoracic and cardiovascular surgery
2022

Pulmonary Function Tests as a Biomarker in Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia Patients Treated With Somatostatin Analogues.

Cureus
2022

A multidisciplinary approach to the work up and management of pulmonary carcinoid tumors and DIPNECH: a narrative review.

Translational lung cancer research
2022

Quantitative Airway Assessment of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) on CT as a Novel Biomarker.

Diagnostics (Basel, Switzerland)
2023

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 Cancer
2022

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH): An Underrecognized Cause of Multiple Lung Nodules and Mosaic Perfusion Pattern.

Journal of the Belgian Society of Radiology
2022

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: a rare cause of airflow limitation.

BMJ case reports
2022

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH): a little known cause of thoracic lesions.

BMJ case reports
2022

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia presenting as a solitary lung nodule: a rare histopathological diagnosis.

Oxford medical case reports
2022

Lung adenocarcinoma coexisting with diffuse idiopathic pulmonary neuroendocrine cell hyperplasia manifesting as multiple pulmonary nodules: A case report.

Thoracic cancer
2022

Diffuse 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 neuroendocrinology
2022

A case of multiple lung carcinoid tumors localized in the right lower lobe.

Respiratory medicine case reports
2023

Not All Multifocal Pulmonary Neuroendocrine Cell Proliferations Represent Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia.

The Annals of thoracic surgery
2022

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: A rare and under-diagnosed condition.

Pulmonology
2022

[Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia/tumorlets].

Medicina
2021

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH): A Case of Indolent Pulmonary Nodules Diagnosed with Robotic-Assisted Navigational Bronchoscopy.

Case reports in pulmonology
2021

Is the combination of bilateral pulmonary nodules and mosaic attenuation on chest CT specific for DIPNECH?

Orphanet journal of rare diseases
2022

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: clinical characteristics and progression to carcinoid tumour.

The European respiratory journal
2021

[Your images. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia].

La Revue du praticien
2021

Well-Differentiated Bronchopulmonary Neuroendocrine Tumors: More Than One Entity.

Journal of thoracic oncology : official publication of the International Association for the Study of Lung Cancer
2021

Lung carcinoid tumors with Diffuse Idiopathic Pulmonary NeuroEndocrine Cell Hyperplasia (DIPNECH) exhibit pejorative pathological features.

Lung cancer (Amsterdam, Netherlands)
2021

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: DIPNECH.

Current opinion in pulmonary medicine
2021

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia With Progression to Neuroendocrine Tumor.

Cureus
2021

[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 Citologia
2021

An Update on the Management of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH).

Current treatment options in oncology
2020

Constrictive bronchiolitis in diffuse idiopathic pulmonary neuroendocrine cell hyperplasia.

ERJ open research
2020

Rare diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: one case report and literature review.

Translational cancer research
2020

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Imaging and Clinical Features of a Frequently Delayed Diagnosis.

AJR. American journal of roentgenology
2021

Clinical Characteristics of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: A Retrospective Analysis.

Chest
2020

Recent advances in the understanding of bronchiolitis in adults.

F1000Research
2020

Coexistence of DIPNECH and carotid body paraganglioma: is it just a coincidence?

Endocrinology, diabetes &amp; metabolism case reports
2020

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: A Clinical Case.

European journal of case reports in internal medicine
2020

Somatostatin Analogs Improve Respiratory Symptoms in Patients With Diffuse Idiopathic Neuroendocrine Cell Hyperplasia.

Chest
2019

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia in a young man with hypoxia: a case report and review of the literature.

Acta bio-medica : Atenei Parmensis
2020

[A case of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) syndrome].

Revue des maladies respiratoires
2020

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia, Mimicking Pulmonary Metastasis.

International journal of surgical pathology
2020

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: An Asthma Mimicker.

The American journal of medicine
2019

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Review of the Literature and a Single-center Experience.

Cureus
2019

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) with liver metastases.

BMJ case reports
2019

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: Unusual presentation.

Annals of thoracic medicine
2018

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: Case series and a review of the literature.

Medicine
2018

Management of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Review and a Single Center Experience.

Lung
2018

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia on Somatostatin Receptor Imaging.

American journal of respiratory and critical care medicine
2018

A Rare Case of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia.

Cureus
2018

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia Causing Chronic Cough.

Archivos de bronconeumologia
2019

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: Report of two cases.

Medicina clinica
2018

Series of rare lung diseases mimicking imaging patterns of common diffuse parenchymal lung diseases.

Lung India : official organ of Indian Chest Society
2018

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia Syndrome Treated With Sirolimus.

Annals of internal medicine
2018

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia mimicking metastatic disease.

Pulmonology
2018

Diffuse 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 pathology
2018

Molecular alterations of neuroendocrine tumours of the lung.

Histopathology
2017

Prognostic Factors in Typical and Atypical Pulmonary Carcinoids.

Ochsner journal
2017

Atypical 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
2017

[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)
2017

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia diagnosed by tranbronchoscopic cryoprobe biopsy technique.

Respirology case reports
2017

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: A Case Report and Review of the Literature.

The American journal of case reports
2017

Association of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) with lung adenocarcinoma: A radiologist's perspective.

Lung cancer (Amsterdam, Netherlands)
2018

68Gallium DOTANOC-PET Imaging in Lung Carcinoids: Impact on Patients' Management.

Neuroendocrinology
2017

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia diagnosed by transbronchial lung cryobiopsy: a case report.

Journal of medical case reports
2017

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia and Granulomatous Inflammation Mimicking High-Grade Malignancy on FDG-PET/CT.

Clinical nuclear medicine
2016

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: A Case With Long-term Follow-up.

Journal of thoracic imaging
2016

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia and Neuroendocrine Hyperplasia of Infancy.

Clinics in chest medicine
2016

Expression of developing neural transcription factors in diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH).

Virchows Archiv : an international journal of pathology
2016

Diffuse Neuroendocrine Cell Hyperplasia: Report of Two Cases.

Case reports in pathology
2016

A case of DIPNECH presenting as usual interstitial pneumonia.

Pneumonologia i alergologia polska
2016

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome.

The European respiratory journal
2016

[Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: Usefulness of espiratory chest CT].

Medicina clinica
2016

[Multifocal Pulmonary Neuroendocrine Tumours: Genesis, Diagnostics and Treatment].

Pneumologie (Stuttgart, Germany)
2016

DIPNECH: Association Between Histopathology and Clinical Presentation.

Lung
2016

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH)--An uncommon precursor of a common cancer?

Pathology, research and practice
2015

Pulmonary Peripheral Carcinoids after Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia and Tumorlets: Report of 3 Cases.

Case reports in pulmonology
2015

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) and multiple pulmonary epithelioid hemangioendothelioma (PEH): a case report.

Pathologica
2015

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH).

Seminars in diagnostic pathology
2015

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) and the Role of Somatostatin analogs: A Case Series.

Lung
2015

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia of the Lung (DIPNECH): Current Best Evidence.

Lung
Ver todos os 109 no EuropePMC

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Respostas geradas por IA a partir das fontes citadas

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.

  1. MONDO
    purl.obolibrary.org
  2. GARD:10780
    GARD (NIH)

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

Compêndio · Raras BR

Hiperplasia difusa idiopática das células neuroendócrinas pulmonares

ORPHA:617916 · MONDO:0022986
Prevalência
<1 / 1 000 000
Casos
100 casos conhecidos
CID-10
J84.8 · Outras doenças pulmonares intersticiais especificadas
CID-11
Início
Adult, Elderly
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C1333291
EuropePMC
Papers 10a
Evidência
🥉 Relato de caso

📋 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
Rara