Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia A Systematic Overview (original) (raw)
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Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia
American Journal of Respiratory and Critical Care Medicine, 2011
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIP-NECH) is being recognized with increasing frequency. Diagnostic and treatment information is limited. A systematic review is presented, focusing on patient demographics, clinical presentation, diagnosis, treatment options, and outcomes. A systematic electronic literature search was conducted for adult DIPNECH cases reported in the English literature during the past 6 years. Twenty-four DIPNECH cases were identified. Another case from our institution is contributed. Women represent 92% (23 of 25). Mean age at diagnosis was 58 years (range, 36-76 yr). Most were nonsmokers (16 of 24). Symptoms included cough (71%), dyspnea (63%), and wheezing (25%) occurring days to years before diagnosis. Pulmonary function testing showed obstructive ventilatory disease in 54%. Lung nodules were seen in 15 patients (63%), ground-glass attenuation in 7 patients (29%), and bronchiectasis in 5 patients (21%). Histological confirmation required surgical lung biopsy for 88%; however, transbronchial biopsies alone were diagnostic in three patients. Treatments strategies included systemic and inhaled corticosteroids, bronchodilators, and lung resection. Available followup data in 17 patients showed 6 clinically improved, 7 who remained stable, and 4 clinically deteriorated. The majority of patients presenting with DIPNECH are middle-aged females with symptoms of cough and dyspnea; obstructive abnormalities on pulmonary function testing; and radiographic imaging showing pulmonary nodules, ground-glass attenuation, and bronchiectasis. In general, the clinical course remains stable; however, progression to respiratory failure does occur. Long-term follow-up and treatment remains incomplete. Establishment of a national multicenter DIPNECH registry would allow formulation of optimal evidence-based guidelines for management of these patients.
Thorax, 2007
A review was undertaken of 19 patients diagnosed with diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) between 1992 and 2006. Most patients were women (n = 15) and non-smokers (n = 16). Clinical presentation was either with symptomatic pulmonary disease (group 1; n = 9) or as an incidental finding during investigation for another disorder, most frequently malignant disease (group 2; n = 10). In group 1, cough and dyspnoea were the most frequent symptoms, with an average duration of 8.6 years before diagnosis. Both groups showed mainly stable disease without treatment, although one patient progressed to severe airflow obstruction and one was diagnosed at single lung transplantation. Mosaicism with nodule(s) was the typical pattern of DIPNECH on high-resolution computed tomography, but one case had normal imaging despite airflow obstruction. Lung function tests showed obstructive (n = 8), mixed (n = 3) or normal (n = 5, all group 2) physiology. Two patients under...
Diffuse idiopathic pulmonary neuroendocrine hyperplasia
Introduction: Diffuse idiopathic pulmonary neuroendocrine cells hyperplasia (DIPNECH) is an extremely rare lesion of the lung, less than 400 cases are reported in the literature since its discovery in 1992. Observation: 51-year-old Patient hospitalized after the fortuitous discovery of a small nodule of the lobe apical right. The surgical pathologic examination of the scannoguided biopsy found a lung parenchyme the bronchial cells of which are replaced by neuroendocrine hyperplasiques making cells posing the diagnosis of a Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia with a tumor typical carcinoïde. The patient benefited a surgical resection of the nodule. The contrôle by a thoracic computed tomography scans don't objectivited a parenchymal recurrence, or appearance of other nodule. Conclusion: The current data of this disease concerning its diagnosis, treatment and prognosis are still little known. Several studies will be necessary to clear up the various persistent points about this pathology.
A Rare Case of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia
2018
We describe the case of a 53-year-old woman who visited many pneumologists without a diagnosis until finally being diagnosed with diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH). It is a relatively new disease characterized by neuroendocrine cell hyperplasia in small airways. She has stable DIPNECH and neuroendocrine carcinoma with somatostatin therapy.
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: a rare preneoplastic condition
Revue des Maladies Respiratoires
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is an uncommon preneoplastic condition, often associated with typical carcinoid tumours. The observations reported below concern two women, both suffering from chronic pulmonary symptoms. These patients underwent computed tomography that showed a solitary nodule in the first patient and multiple sub centimetre nodules in the second. In both cases histological studies of the pulmonary biopsies revealed: a proliferation of neuroendocrine cells dispersed in the bronchial and bronchiolar epithelium, more specifically superficial to the basement membrane; some tumourlets; a typical carcinoid tumour was also found in the first patient's biopsy. The choice of treatment remains difficult, mainly because the existing studies are restricted to small numbers of patients or isolated cases, a consequence of the low prevalence of this disease. Considering its slow evolution, management by long-term clinical, endoscopic and...
JTO Clinical and Research Reports, 2020
Introduction: Diffuse idiopathic pulmonary neuroendocrine hyperplasia (DIPNECH) is a rare condition that is likely underdiagnosed owing to the lack of established and validated diagnostic criteria. These clinical guidelines are empirical and created on the basis of a limited number of studies. This study was designed to validate the existing criteria and to identify new clinical parameters that can accurately diagnose DIPNECH. Methods: Patients with DIPNECH were identified from a cohort that underwent surgical lung resection for pulmonary carcinoids. The study cohort included a total of 105 consecutive cases with neuroendocrine lesions. Initial diagnostic predictors of DIPNECH were selected from the literature. We employed univariate and multivariate models to evaluate the association of clinical, pathologic, radiologic variables with the likelihood of DIPNECH. Results: Univariate analysis identified age, sex, chronic obstructive pulmonary disease diagnosis, obstructive abnormalities, pulmonary nodules, mosaicism, absolute numbers of pulmonary neuroendocrine lesions (PNELs), and the number of tumorlets as significant DIPNECH predictors (for p < 0.05). After adjustment for sampling variations, the ratio of the total number of PNELs to the number of bronchioles was found to be considerably higher in DIPNECH category. Multivariate analysis identified the total number of PNELs and multiple pulmonary nodules (>10) as independent predictors of DIPNECH. The performance of our criteria revealed an accuracy of 76% in detecting DIPNECH cases. Conclusions: We proposed a set of diagnostic criteria for DIPNECH on the basis of an expert-panel approach integrating pathological features, radiology, and clinical data. Our findings will help identify DIPNECH patients, without a pathological confirmation of a neuroendocrine lesion. Before the implementation of these criteria in clinical practice, they require further validation in multiinstitutional cohorts.
Oxford Medical Case Reports, 2022
We present a case of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) in a 56-year-old woman, who presented to our emergency department with a 7-day history of exertional dyspnoea. Due to profound haemodynamic compromise, pulmonary embolism (PE) was suspected, and the patient underwent emergency thrombolysis on admission. A subsequent computerized tomography pulmonary angiogram revealed extensive bilateral PE. Incidentally, a 1.3 cm lesion within the right upper lobe, associated with pleural tethering, was identified. Positron emission tomography computerized tomography and, subsequently, histopathology revealed this lesion to be primary DIPNECH, a rare pre-invasive hyperplasia of neuroendocrine cells. While studies are scarce and cohort numbers are low, somatostatin analogues and protein kinase inhibitors have been proven to reduce symptoms and increase progression-free survival in DIPNECH, respectively.
Lung Cancer, 2017
Association of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) with lung adenocarcinoma: A radiologist's perspective To the Editor, We describe the case of an asymptomatic 66-year-old woman who was incidentally diagnosed with a lung nodule on a chest radiograph. A thoracic CT confirmed a 12-mm suspicious spiculated partly-solid lung nodule in the right lower lobe and unexpectedly detected multiple small nodules scattered throughout both lungs (Fig. 1). Given this presentation, small pulmonary metastases could not be completely ruled out. A videoassisted thoracoscopic lobectomy surgery (right lower lobectomy and a right middle lobe wedge resection) was performed, confirming a right lower lobe pT1a low-grade invasive mucinous adenocarcinoma and findings consistent with diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH). These findings were found both in the right lower lobectomy and right middle lobe wedge resection and encompassed the presence of nodular proliferations of neuroendocrine cells (including multiple tumorlets and even a small 6-mm carcinoid tumor) and areas of constrictive obliterative bronchiolitis. DIPNECH is a rare and poorly understood preneoplastic pulmonary disorder with less than 100 documented cases in the scientific literature and with no consolidated diagnostic radiological and/or pathological criteria that typically affects women [1]. According to the original description, DIPNECH is an entity characterized histologically by a diffuse proliferation of neuroendocrine cells and presence of constrictive obliterative bronchiolitis, radiologically by the presence of small pulmonary nodules, mosaic attenuation due to air trapping, and bronchial wall thickening, and clinically by symptoms of airflow limitation (dyspnea, chronic cough) [2]. However, the term DIPNECH is also used in asymptomatic patients with carcinoid tumors without radiological features of airway disease in whom incidental foci of neuroendocrine cell hyperplasia and tumorlets are found adjacent to the carcinoid tumor on the histopathological analysis [3]. To our knowledge this is the sixth reported case (third publication) describing the association of a lung adenocarcinoma with DIPNECH. Previous reports have focused on the pathologic findings of this rare association and have suggested that a subset of lung adenocarcinomas may have concomitant DIPNECH within the lung cancer, implying a causal relationship. Interestingly, this subset of lung adenocarcinomas may be low-grade tumors and exhibit a better prognosis than conventional (not DIPNECH-associated) lung adenocarcinomas [4,5]. This case reminds us that DIPNECH, whether it represents a fortuitous association with lung adenocarcinoma or not, may mimic metastatic pulmonary nodules in lung cancer patients. Therefore, we believe that DIPNECH should be considered in the differential diagnosis of patients with potentially resectable primary lung cancers presenting with small pulmonary nodules on imaging studies.
Neuroendocrine cell hyperplasia as an unusual form of interstitial lung disease
Respiratory Medicine, 2007
Study objectives: Two patients diagnosed with interstitial lung disease (ILD) secondary to pulmonary neuroendocrine cell (PNEC) hyperplasia are presented. Background: Pulmonary neuroendocrine cell hyperplasia (PNECH) is a rare entity worldwide and few cases presenting as ILD have been reported. Following the consensus criteria established by the American Thoracic Society (ATS) and the European Respiratory Society (ERS), three patients from among 500 ILD patients over the last 10 years in our institution were diagnosed with PNECH. The diagnosis was established by open lung biopsy using specific stains to demonstrate neuroendocrine cells in lung tissue. Methods: Patients were questioned on their medical and pathological history, occupational or environmental exposure to toxic substances and any relationship with smoking. In addition, were recorded symptoms at presentation, physical signs, analytic and respiratory function results, chest X-ray and CT scan features, bronchoscopy findings including bronchoalveolar lavage and transbronchial biopsy, and open lung biopsy findings. Results: In these two patients, PNECH was the only cause of their diffuse lung disease. Clinical signs and symptoms (cough, expectoration and progressive dyspnea) were similar to other idiopathic interstitial pneumonias and radiologic features (ground-glass infiltrates in mosaic pattern) were consistent with those of nonspecific interstitial pneumonia or the onset of hypersensitivity pneumonitis.
Southwest Journal of Pulmonary and Critical Care, 2017
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary disease, where carcinoid tumorlets invade the pulmonary parenchyma and bronchioles. These nests of cells release a variety of mediators including bombesin and gastrin releasing peptide that cause heterogeneous bronchoconstriction, creating a mosaic appearance on chest imaging studies, especially on expiratory scans. Clinically patients usually have long standing symptoms of shortness of breath (SOB) and cough that are difficult to distinguish from asthma. In this article we describe a case of DIPNECH in a patient with several years' history of SOB and cough, and review 179 cases of DIPNECH reported in the literature since 1992. Case Presentation A 72-year-old, non-smoking lady was admitted to the hospital in preparation for bilateral mastectomy. She recently received a diagnosis of bilateral breast invasive ductal carcinoma grade 2, estrogen receptor/progesterone receptor/human epidermal growth factor receptor 2 (HER-2) positive in the left tumor but negative in the right tumor. Her past medical history was significant for hypertension, long standing cough and dyspnea on exertion labeled as asthma poorly responsive to nebulizers. Socially, she was a house wife with no history of occupational exposure. The patient was found to be tachypneic (respiratory rate 22 breaths/minute) and hypoxemic (oxygen saturation 86% on room air). Heart rate and blood pressure were within normal limits. She had bilateral decreased breath sounds and diffuse expiratory wheezes. Chest CT scan revealed diffuse mosaic pattern and multiple pulmonary nodules in both lungs suggestive of metastases (Figure 1).