Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia Research Articles

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia in adults

Diffuse Idiopathic Pulmonary NeuroEndocrine Cell Hyperplasia (DIPNECH) is an understudied rare disease that should be taken into account in the differential diagnosis of therapy-resistant bronchial obstruction combined with focal pulmonary lesions and mosaic attenuation on chest CT. The aim of this article is to discuss the aspects of clinical and morphological diagnosis, therapeutic options, prognosis assessment, and the need for follow-up using a clinical case as an example. The article describes a clinical case of DIPNECH in a 55-year-old woman, and also provides a brief review of the scientific literature on DIPNECH. DIPNECH is a rare disease that most often affects non-smoking women aged around 60 years. The most frequent clinical manifestations are cough, dyspnea, stridor, and broncho-obstructive syndrome. Only histological and immunohistochemical assays can confirm the diagnosis. DIPNECH should be considered as part of the differential diagnosis in patients with prolonged cough, dyspnea of unclear genesis and changes on chest CT in the form of pulmonary focal lesions and mosaic attenuation. Conclusion. The presented clinical case shows the challenges and importance of timely diagnosis.

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

To conduct a multi-lesional computed tomography (CT) analysis of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) patients to determine volumetric changes in lesions over 5 years. A retrospective case-note review was undertaken to identify 16 patients with histological and radiological features of DIPNECH between 2012-2021. Area and volume were calculated for 17 sets of lesions identified on high-resolution CT. Clinical data were extracted from electronic patient records, which included demographic data, outpatient clinic letters, histology reports, and imaging reports. One hundred and twenty-eight lesions were identified in 16 patients (one male, 15 female) and followed-up annually over a median 1,985 days (range 1,450-2,290). At year 1 follow-up, lesion area ranged from 1-48 mm2, and lesion volume ranged from 8-18,380 mm3; lesion area ranged from 1-45mm2 and lesion volume ranged from 11-17,800 mm3 and year 5. Half (8/16) of the patients had concomitant typical carcinoid tumours and one patient had an atypical carcinoid tumour. No statistically significant correlation (p<0.05) was found between lesion cross-sectional area or volume and duration of follow-up (years and days). No metastatic spread was observed at the time of analysis. No significant increase was observed in the size of over 100 lesions in patients with DIPNECH over a 5-year period and no metastasis occurred during the study period affirming the relatively indolent course of the disease.

Congenital Pulmonary Airway Malformation Type 1 with Pulmonary Neuroendocrine Cell Hyperplasia in an Adult Male: a Rare Case Report

Abstract Introduction/Objective Congenital pulmonary airway malformations (CPAM) is rare, usually diagnosed in the pediatric population, and may occur in adults. Pulmonary neuroendocrine cell hyperplasia (PNECH) mimicking diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) associated with CPAM has not been described previously. We report a case of CPAM Type 1 with PNECH. Methods/Case Report A 33-year-old male, with past medical history of latent tuberculosis (TB) by Quantiferon blood testing, recurrent pneumonia, recurrent lung abscesses, and lung cavitary lesion for three years, presented with fever, cough with hemoptysis, shortness of breath, and left-sided chest pain. Computerized tomography of chest displayed a 10.8 cm cavitary lesion in the left lower lobe of the lung. The patient underwent left lower lobectomy of the lung. Grossly, the lobectomy specimen revealed a 10.5 cm irregular cavity communicating to the superior segmental bronchus of the left lower lobe. Microscopically, the dominant large cyst was surrounded by lung parenchyma and small cystically dilated spaces resembling bronchioles under the fibrotic pleura. The large cyst was lined by respiratory epithelium with rare pathognomonic mucous cells in clusters. Cyst walls contained smooth muscle, small islands of cartilage, and focal intense acute and chronic inflammatory cell infiltrates with hemorrhage. There were multiple foci of proliferation of pulmonary neuroendocrine cells centered around bronchioles surrounding the large cyst. The neuroendocrine cells were arranged from individual cells and cellular aggregates to larger tumorlets, highlighted by Synaptophysin and Chromogranin immunostains. No granuloma or necrosis was present. No acid-fast bacilli or fungal microorganisms were identified by special stains. The findings are consistent with CPAM type 1, with PNECH. Results (if a Case Study enter NA) NA Conclusion Our study demonstrates a histologically diagnosed adult congenital pulmonary airway malformations type 1 with an incidental pulmonary neuroendocrine cell hyperplasia, which mimics diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. To the best of our knowledge, this is the first report of such an association.

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

To review histologically confirmed diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) cases and carry out a detailed pathological-radiological correlation to see if computed tomography (CT) can be used to confidently identify DIPNECH. Twenty-three histologically confirmed DIPNECH patients in the shared database of two NHS Trusts were reviewed. CT images were reviewed by two independent radiologists, each of them with >10 years of experience in thoracic imaging. All histological specimens were reviewed by a single pathologist with >25 years of experience. The diagnosis of DIPNECH was made according to the current World Health Organization (WHO) definition included in the WHO 2015 classification of pulmonary tumours. The results on histology were compared to the presence of nodules and air trapping on CT. Demographic information and, when available, molecular imaging studies and pulmonary function tests were also considered. There are prototypal clinical and radiological findings reflecting the presence of underlying histological DIPNECH: middle-aged women with multiple small and scattered nodules due to the clustering and proliferation of neuroendocrine cells. At least one larger, dominant, lung nodule reflecting a carcinoid tumour is very common and mosaic attenuation/air trapping is seen approximately in 50% of cases in inspiratory scans. Airflow obstruction is rarely associated with histological bronchial or peribronchial fibrosis, which suggests other mechanisms must be involved in its development. CT can be used to predict pathological DIPNECH in the appropriate clinical setting. It is important to consider DIPNECH to avoid overdiagnosis of more sinister conditions such as lung cancer or metastases.

(857) Bilateral Lung Transplant with Recognition of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) of rhe Donor Lungs in the Immediate Postoperative Period

(857) Bilateral Lung Transplant with Recognition of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) of rhe Donor Lungs in the Immediate Postoperative Period

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

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) describes an indolent, under-recognised and poorly understood pulmonary condition with fewer than 200 reported cases across the literature. Currently, cases are diagnosed following a biopsy of the primary lesion, with treatment options centring on symptomatic benefit as opposed to targeting the underlying aetiology. Classically, DIPNECH lesions have been described as slow growing and benign, but with growing awareness of the condition, reports of metastatic disease with significant symptomatic burden have been reported. However, effectively addressing the subset of DIPNECH patients with greater metastatic potential remains an unmet clinical need. Due to the similarities between DIPNECH and carcinoid patients, several centres have considered using somatostatin analogues to not only help symptomatically but also to initiate tumour regression. However, to date, there are limited biomarkers to help evaluate the benefit of such options. In this review, we consider the use of pulmonary function tests (PFTs) to help quantify the benefit of somatostatin analogues. Although much of the evidence stems from small single-centre studies, the use of PFTs within the treatment pathway for both localised and metastatic DIPNECH represents a meaningful improvement from subjective monitoring of disease.

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

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) occurs due to abnormal proliferation of pulmonary neuroendocrine cells. We hypothesized that performing a quantitative analysis of airway features on chest CT may reveal differences to matched controls, which could ultimately help provide an imaging biomarker. A retrospective quantitative analysis of chest CTs in patients with DIPNECH and age matched controls was carried out using semi-automated post-processing software. Paired segmental airway and artery diameters were measured for each bronchopulmonary segment, and the airway:artery (AA) ratio, airway wall thickness:artery ratio (AWTA ratio) and wall area percentage (WAP) calculated. Nodule number, size, shape and location was recorded. Correlation between CT measurements and pulmonary function testing was performed. 16 DIPNECH and 16 control subjects were analysed (all female, mean age 61.7 +/- 11.8 years), a combined total of 425 bronchopulmonary segments. The mean AwtA ratio, AA ratio and WAP for the DIPNECH group was 0.57, 1.18 and 68.8%, respectively, compared with 0.38, 1.03 and 58.3% in controls (p &lt; 0.001, &lt;0.001, 0.03, respectively). DIPNECH patients had more nodules than controls (22.4 +/- 32.6 vs. 3.6 +/- 3.6, p = 0.03). AA ratio correlated with FVC (R2 = 0.47, p = 0.02). A multivariable model incorporating nodule number, AA ratio and AWTA-ratio demonstrated good performance for discriminating DIPNECH and controls (AUC 0.971; 95% CI: 0.925-1.0). Quantitative CT airway analysis in patients with DIPNECH demonstrates increased airway wall thickness and airway:artery ratio compared to controls. Quantitative CT measurement of airway wall thickening offers a potential imaging biomarker for treatment response.

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

Low and intermediate grade neuroendocrine tumors of the lung are uncommon malignancies representing 2% of all lung cancers. These are termed typical and atypical pulmonary carcinoid tumors. These can arise in the setting of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH). The presentation, workup, management and outcomes of patients with these tumors can overlap with more common lung cancers but differ in that many of these patients have a prolonged clinical course. The objective of this narrative review is to summarize the literature and provide evidence and expert-based algorithms for work up and treatment of pulmonary carcinoids and DIPNECH. A search of PubMed and Web of Science databases ending April 15, 2022, with the following keywords "lung carcinoid", "DIPNECH", "lung neuroendocrine," and "bronchopulmonary carcinoid". Pulmonary carcinoid tumors benefit from a multidisciplinary approach. Pre-treatment imaging with contrast-enhanced computed tomography, and DOTATATE positron emission tomography is required. Surgical resection is the gold standard for curative intent, and possibly including sublobar resections. Patients can recur or develop new primaries thus emphasizing the importance of surveillance; national guidelines recommend at least a 10-year follow up. A growing body of literature support the use of endobronchial therapy, with long responses documented. Systemic therapy consists of everolimus, somatostatin analogs, peptide receptor radionuclide therapy, and chemotherapy. Diffuse idiopathic pulmonary neuroendocrine tumor cell hyperplasia is rare, but series suggest somatostatin analogs may confer clinical benefit. Pulmonary carcinoid tumors and DIPNECH are rare. Despite lack of regulatory approvals for advanced disease, multiple options are available but should be sequenced according to the clinical status and disease biology. Each patient should be discussed in a multidisciplinary setting and clinical trials should be considered if available.

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.

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is considered to be a rare condition associated with lung neuroendocrine tumours (NET), and its natural history is poorly described. We aimed to assess the prevalence and clinicopathologic characteristics of DIPNECH in the lung NET population, and to investigate predictors of time-to-progression (TTP) and overall survival (OS). We retrospectively identified patients diagnosed with DIPNECH between April 2005 and December 2020. Clinical data were collected from medical records. The relationship between baseline characteristics and TTP and OS was analysed using the Kaplan-Meier method. Univariate analysis was performed using the Cox proportional hazards model. Of 311 patients with well-differentiated lung NETs, 61 (20%) had DIPNECH and were included in the study. Baseline demographics described 95% female, 59% never smokers and mean body mass index 34.4 kg m-2 ; 77% were typical carcinoids (TC), 13% atypical carcinoids (AC), and 10% both TC and AC (multicentric). At presentation, 54% of patients were asymptomatic. Multicentric NETs were demonstrated in 16 (26%) on histopathology, and a further 32 (52%) had synchronous NETs suggested on imaging (multiple nodules ≥ 5 mm). Seven (11%) patients developed metastases and the median OS from time of first metastasis was 37 months. AC histopathology and NET TNM stage ≥ IIA were associated with poorer TTP and OS. Of the DIPNECH cohort, the 15-year survival rate was 86%. DIPNECH may be more prevalent in the lung NET population than previously appreciated, especially in women. Although our results confirm that DIPNECH is predominantly an indolent disease associated with TC, 23% developed AC and these patients may warrant closer observation.

March 2022 Medical Image of the Month: Pulmonary Nodules in the Setting of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH)

No abstract available. Article truncated after 150 words. A 62-year-old woman presented to an outside hospital with chronic cough, prompting a CXR. Findings further prompted unenhanced chest CT to evaluate possible pulmonary nodules. The CT demonstrated multiple scattered, solid and centrilobular pulmonary nodules, most of which were small but there were two &gt;1 cm nodules, one in the right upper lobe (RUL) and a second in the lingula (Figure 1A,B). A subsequent FDG PET-CT was performed demonstrating increased metabolic activity in the RUL nodule with no activity in the lingular nodule (Figure 1C,D). Biopsy of the RUL nodule was consistent with a carcinoid. At this point the patient was referred to our center for further management. A repeat chest CT failed to demonstrate any significant change in the nodules. MIP and MinIP reconstructions from that examination demonstrate multiple small, solid pulmonary nodules (arrows) (Figure 2A), many of which were associated with air-trapping resulting in pulmonary mosaicism (circled) (Figure …

Incidental finding of pulmonary tumorlet in a case of surgically treated bronchiectatic cavity superimposed by aspergilloma

Introduction. Intracavitary aspergilloma is the consequence of a saprophytic infection of the lung with cavitary disease. Pulmonary tumorlet are nodular proliferations of the neuroendocrine cells less than 5 mm in diameter. Both aspergilloma in the bronchiectatic cavity of the lung and pulmonary tumorlet has rare been seen. Case outline. We present a 71-year-old woman with a medical history of recurrent pneumonia complicated with cough and hemoptysis. Computed tomography (CT) scan of the chest showed pulmonary soft tissue mass in the right lower lobe of the lung 42 ? 50 mm in diameter. Direct microscopy of the specimens of bronchioalveolar lavage showed spores of Aspergillus. Galactoman Ag test was also positive. Right lower lobectomy and mediastinal lymph node sampling was performed via thoracotomy. Pathohistological findings showed aspergilloma with the presence of pulmonary tumourlet and diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) in the lymphovascular spaces. Five years follow up showed no abnormalities on the CT scan, and the patient remained alive without medical problems. Conclusion. The concomitant occurrence of bronchiectasis, aspergilloma, and precancerous lesions such as pulmonary tumourlet and DIPNECH is rare and further increases the risk of developing malignant tumors as well as recurrent infections. Therefore, surgical treatment can prevent the development of premalignant lesions and the occurrence of recurrent infections accompanied by dyspnea and hemoptysis as the main symptoms. The question of the connection between pulmonary tumourlet and chronic inflammatory lung diseases is raised. We hope that future researches will provide answers to this question.

A RARE CASE OF CHRONIC COUGH

TOPIC: Lung Cancer TYPE: Medical Student/Resident Case Reports INTRODUCTION: Chronic cough is one of the most common symptoms for which outpatient care is sought. The differential diagnosis is broad, and many patients undergo extensive evaluation. We here report a rare case of chronic cough with progressive dyspnea due to Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia( DIPNECH), followed by a brief literature review of DIPNECH. CASE PRESENTATION: 70-year-old female, non smoker, history of Crohn's disease presented to the Pulmonology clinic in 2005 with cough and shortness of breath. PFT's showed restrictive pattern. CT scan showed multiple bilateral pulmonary nodules. Bronchoscopy could not identify cause. On periodic CT, lung nodules remained stable. In the meantime, she continued to have chronic cough and progressively worsening shortness of breath. She received multiple trials of steroids, antibiotics, and inhalers with mild improvement. Repeat PFT's now showed severe obstructive disease. Her coughing spells made it difficult to perform on PFTs. She underwent repeat bronchoscopy with extensive testing, including bacterial, fungal, malignancy, and autoimmune causes of her symptoms, all of which came back negative. The working diagnosis was necrobiotic nodules secondary to her Crohn's disease vs. Asthma. She was put on a prolonged steroid course and developed severe side effects like muscle weakness, mood changes, tremors and worsening kidney function. The patient was hesitant to go back on steroids. She had suffered for 17 years. A trial of steroid-sparing medication CellCept was considered. However, before committing the patient to these, a wedge biopsy of the pulmonary nodule was performed and revealed neuroendocrine cells that stained positive for synaptophysin, chromogranin, and CD56.8 confirming the diagnosis of DIPNECH. The patient was subsequently referred to oncology and started on Octreotide infusions causing significant improvement in symptoms. DISCUSSION: DIPNECH is a rare condition that is becoming increasingly recognized. It is typically characterized by an insidious onset with chronic nonproductive cough, exertional dyspnoea, and wheezing, spuriously attributed to Asthma, chronic obstructive pulmonary disease, or GERD. Indeed, the diagnosis of DIPNECH is usually made several years after the onset of clinical symptoms, often following the incidental discovery of a lung nodule (or nodules) on imaging, usually computed tomography (CT). It is characterized histologically by a diffuse, bronchiolo-centric proliferation of neuroendocrine cells at the periphery of the lung, radiologically by ground-glass opacity, mosaic attenuation with air trapping, bronchial wall thickening, and pulmonary nodules and, Clinically by symptoms of airflow limitation. CONCLUSIONS: We hope this case can increase awareness of DIPNECH among clinicians as patients may otherwise be misdiagnosed and undergo unnecessary treatments or procedures causing permanent harm. REFERENCE #1: Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia 1Margaret Wei, MD and 2Mehri Manouchehr-Pour CLINICAL VIGNETTE1UCLA Department of Medicine2UC Riverside Department of Cell, Molecular, and Developmental Biology DISCLOSURES: No relevant relationships by Venkatkiran Kanchustambham, source=Web Response No relevant relationships by Swetha Saladi, source=Web Response No relevant relationships by Khurram Shafique, source=Web Response No relevant relationships by Vinita Vaidya, source=Web Response

Well-Differentiated Bronchopulmonary Neuroendocrine Tumors: More Than One Entity

IntroductionUntil now, well-differentiated bronchopulmonary neuroendocrine tumors (bpNET) occurring either sporadically (sp-bpNET) or in the context of multiple endocrine neoplasia type 1 (MEN1) and diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) are regarded as similar entities. However, in contrast to sp-bpNET: MEN1-related and DIPNECH-related bpNET rarely metastasize or lead to bpNET-related death. We aimed to describe and compare the course of the disease of sp-bpNET, DIPNECH- and MEN1-related bpNET. MethodsAll patients with histologically confirmed MEN1-related bpNET from the DutchMEN Study Group database (1990-2017), patients with resected sp-bpNET and DIPNECH patients referred to a Dutch European Neuroendocrine Tumor Society center between 2000 and 2018 were included. Fisher’s exact test was used for comparison between groups. The primary end point was disease-specific mortality (DSM). Kaplan-Meier and logrank test were used to compare survival. Cox regression was used to identify risk factors for DSM in the sp-bpNET subgroup. ResultsWe included 112 sp-bpNET, 29 MEN1, and 27 DIPNECH patients. Tumor classification was similar across subgroups. A total of 20 patients (18%) with sp-bpNET died because of bpNET, compared with none in the MEN1 group and DIPNECH group. Median disease-specific survival was 12.3 (confidence interval: 6.3–18.3) years for patients with sp-bpNET, and not estimable for the other subgroups (p < 0.001). Differences in baseline characteristics did not explain worse survival in sp-bpNET. Tumor classification and age at diagnosis were independent risk factors for DSM in sp-bpNET. ConclusionsPatients with sp-bpNET have a significantly higher DSM compared with MEN1 or DIPNECH-related bpNET, unexplained by differences in baseline characteristics. This implies that not all bpNET are similar entities.

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

IntroductionDiffuse Idiopathic Pulmonary NeuroEndocrine Cell Hyperplasia (DIPNECH) is a rare disease often associated with carcinoid tumors. We aimed at evaluating the impact of DIPNECH on characteristics and prognosis of patients who underwent radical treatment of pulmonary carcinoid tumors. Material and methodsWe reviewed all patients operated on for curative-intent resection of carcinoid tumor in our department from 2001 to 2020. Cases exhibiting both pathological and radiological features of DIPNECH, as assessed by respective thoracic expert physicians, were analyzed separately. Results172 cases of resected carcinoid tumors were identified, including 25 (14.5 %) harboring pathological criteria of DIPNECH and radiologic features like mosaic attenuation (92.0 %), multiple nodules < 5 mm (76.0 %), and mucoid impactions (32 %). In DIPNECH patients, major pulmonary resections were usually performed (92.0 %) and resected tumors were mostly classified as pT1 (92 %). Mean Ki67 staining was 3.7 ± 5.2 %. The early postoperative period was mostly uneventful (96.0 %) and 5-year survival was 92.9 ± 6.9 %. Compared to non-DIPNECH cases, we found that patients were older (mean 65.6 ± 9.3 versus 54.1 ± 17.9, p = 0.002), more frequently female (84.0 % versus 56.5 %, p = 0.009), and exhibiting diabetes mellitus (45.8 % versus 18.5 %, p < 0.001) or hypertension (45.8 % versus 24.1 %, p = 0.039). The rate of atypical carcinoid tumors was significantly higher in DIPNECH patients (40.0 % versus 19.9 %, p = 0.027), as well as rate of mediastinal lymph-nodes involvement (pN2+) (36.0 % versus 4.1 %, p < 0.001). At multivariate analysis, only DIPNECH pattern and atypical histology were independent factors of pN2 invasion which was the only predictor of poorer prognosis on Log-Rank test. ConclusionCarcinoid tumors with proven DIPNECH are associated with negative pathological features and may deserve a dedicated perioperative management.

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: DIPNECH.

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare premalignant condition. Over the past decade, there has been increased recognition and reporting of DIPNECH in the literature. Currently, our understanding is that DIPNECH has a predilection to nonsmoking females around their sixth decade of life. The patients usually present with chronic cough, dyspnea, and computed tomography (CT) showing multifocal pulmonary nodules with associated mosaic attenuation. The clinic history is largely driven by constrictive obliterative bronchiolitis, which typically has an indolent course with progressive respiratory decline and difficult to treat symptoms. DIPNECH has been found to be associated with carcinoid tumors. Recent data has found that symptomatic DIPNECH patients respond to somatostatin analog (SSA). SSAs provide improvement in symptoms and pulmonary function tests. According to small studies and case series SSAs can be used in conjunction with steroids and bronchodilators for the treatment of respiratory symptoms. DINPNECH is a premalignant condition that can transform into carcinoid tumors. Although the recent data suggest the potential efficacy of SSA, further studies are needed to validate such results in prospective fashion in addition to investigating other therapeutic agents.

ES17.03 Updates in Squamous Cell Carcinoma and Neuroendocrine Tumors of the Lung

ES17.03 Updates in Squamous Cell Carcinoma and Neuroendocrine Tumors of the Lung

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

OBJECTIVE. The purpose of this study was to assess features of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) on CT, clinical presentation, and delays in radiologic and clinical diagnosis in a series of 32 patients. MATERIALS AND METHODS. Medical records of patients with DIPNECH from the years 2000-2017 were obtained from an institutional data warehouse. Inclusion criteria were an available CT examination and either a pathologic diagnosis of DIPNECH or pathologic findings of multiple carcinoid tumorlets or carcinoid tumor with CT features suggesting DIPNECH. Two thoracic radiologists with 10 and 14 years of experience reviewed CT examinations and scored cases in consensus. RESULTS. All 32 patients were women, and most had never smoked (69%). The mean age at presentation was 61 years. Symptoms included chronic cough (59%) or dyspnea (28%), and the initial clinical diagnosis was asthma in 41%. DIPNECH was clinically suspected at presentation in only one case and was mentioned by the interpreting radiologist in only 31% of cases. CT characteristics included numerous nodules with a lower zone and peribronchiolar predominance, mosaic attenuation, and nodular bronchial wall thickening. Number of nodules at least 5 mm in diameter showed strong inverse correlations with the percentage predicted for both forced vital capacity and forced expiratory volume in 1 second and a moderate inverse correlation with total lung capacity percentage predicted. In cases with a follow-up CT interval of 3 years or longer, 85% of patients showed an increase in size of the largest nodule, and 70% had an increase in size in multiple nodules. CONCLUSION. Many cases of DIPNECH are originally missed or misdiagnosed by radiologists and clinicians. Awareness of the typical clinical and imaging features of DIPNECH may prompt earlier diagnosis of this condition.

DIFFUSE PULMONARY MENINGOTHELIOMATOSIS DIAGNOSED BY TRANSBRONCHIAL CRYOBIOPSY

SESSION TITLE: Medical Student/Resident Lung Pathology SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Minute pulmonary meningothelial-like nodules (MPMNs) are typically asymptomatic, solitary lesions incidentally noted on pathologic evaluation of lung tissue. However, diffuse, bilateral pulmonary involvement, termed diffuse pulmonary meningotheliomatosis (DPM), is exceedingly rare and has only been described in a small number of case studies. We report a case of a patient diagnosed with DPM via transbronchial cryobiopsy. CASE PRESENTATION: A 67-year-old non-smoking female with a history of scleroderma was referred for diffuse bilateral pulmonary nodules found on computed tomography (CT) scan of the chest with mosaicism concerning for diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH). Pulmonary function testing was notable for an isolated reduction in diffusing capacity at 58% with a normal echocardiogram. Two transbronchial cryobiopsy samples were obtained from the right lower lobe. Pathology revealed a meningothelial nodule. Immunohistochemistry was positive for epithelial membrane antigen (EMA) as well as progesterone receptor (PR) and negative for synaptophysin and chromogranin. Follow up imaging at 1 year demonstrated stability. Taken in context with her radiographic findings, the patient was diagnosed with DPM. DISCUSSION: DPM is an extremely rare condition characterized by MPMNs diffusely involving lung parenchyma. It predominantly affects females (2.2:1) with a median age of 59-62. DPM has no predilection for specific lobes, and it can be mistaken for metastatic or interstitial disease. Histologically, MPMNs appear as epithelioid cells arranged in small nests in pulmonary interstitium. Immunochemically, MPMNs tend to be positive for vimentin, EMA and PR and negative for S-100 protein, chromogranin A, and synaptophysin. Though the clinical significance of DPM is still unclear, prior studies have noted higher incidence among patients with underlying lung disease, particularly thromboembolism and malignancy. CONCLUSIONS: In summary, we present a case of DPM diagnosed by transbronchial cryobiopsy to highlight the importance of considering DPM in the differential of bilateral micronodular lung disease. Reference #1: Korn D, Bensch K, Liebow AA, Castleman B. Multiple minute pulmonary tumors resembling chemodectomas. Am J Pathol. 1960;37(6):641–672. Reference #2: Gaffey MJ, Mills SE, Askin FB. Minute pulmonary meningothelial-like nodules. A clinicopathologic study of so-called minute pulmonary chemodectoma. Am J Surg Pathol 1988; 12: 167-175. Reference #3: Gleason JB, Valentin R, Almedia P, Martinez N, Bejarano PA. Diffuse pulmonary meningotheliomatosis: a literature review of a rare diffuse parenchymal lung disease with unclear clinical significance. J Assoc Chest Physic 2017; 5: 18-25. DISCLOSURES: Speaker/Speaker's Bureau relationship with Boehringer-Ingelheim Pharmaceuticals Please note: $5001 - $20000 Added 04/07/2020 by Jaime Betancourt, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with Vapotherm, Inc Please note: $5001 - $20000 Added 04/07/2020 by Jaime Betancourt, source=Web Response, value=Honoraria No relevant relationships by I. Obi Emeruwa, source=Web Response No relevant relationships by Scott Oh, source=Web Response No relevant relationships by Megan Trieu, source=Web Response

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

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

Recent advances in the understanding of bronchiolitis in adults.

Bronchiolitis is injury to the bronchioles (small airways with a diameter of 2 mm or less) resulting in inflammation and/or fibrosis. Bronchioles can be involved in pathologic processes that involve predominantly the lung parenchyma or large airways, but, in some diseases, bronchioles are the main site of injury (“primary bronchiolitis”). Acute bronchiolitis caused by viruses is responsible for most cases of bronchiolitis in infants and children. In adults, however, there is a wide spectrum of bronchiolar disorders and most are chronic. Many forms of bronchiolitis have been described in the literature, and the terminology in this regard remains confusing. In clinical practice, a classification scheme based on the underlying histopathologic pattern (correlates with presenting radiologic abnormalities) facilitates the recognition of bronchiolitis and the search for the inciting cause of the lung injury. Respiratory bronchiolitis is the most common form of bronchiolitis in adults and is usually related to cigarette smoking. Currently, the diagnosis of respiratory bronchiolitis is generally achieved based on the clinical context (smoking history) and chest CT findings. Constrictive (obliterative) bronchiolitis is associated with airflow obstruction and is seen in various clinical contexts including environmental/occupational inhalation exposures, transplant recipients (bronchiolitis obliterans syndrome), and many others. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is increasingly recognized and can be associated with progressive airflow obstruction related to constrictive bronchiolitis (“DIPNECH syndrome”). Diffuse aspiration bronchiolitis is a form of aspiration-related lung disease that is often unsuspected and confused for interstitial lung disease. Novel forms of bronchiolitis have been described, including lymphocytic bronchiolitis and alveolar ductitis with emphysema recently described in employees at a manufacturing facility for industrial machines. Bronchiolitis is also a component of vaping-related lung injury encountered in the recent outbreak.