Diffuse Idiopathic Pulmonary Neuroendocrine Cell Research Articles

S3611 Double Trouble: Primary Lung and Colon Cancer in a Patient With Crohn’s Disease

Introduction: This vignette highlights the development of localized rectal adenocarcinoma and concomitant metastatic large cell neuroendocrine tumor of the lung in a patient with longstanding Crohn’s disease (CD) on Vedolizumab (VDZ). Case Description/Methods: A 61 year old male with a 2 year history of severe, uncontrolled colonic inflammatory CD presented to the ED with weakness, loss of appetite, and a 30lb weight loss over a 1 month period. He had been on VDZ 300mg every 8 weeks for 1 year. A CT chest showed a new 2cm pulmonary nodule with increasing mediastinal and perihilar lymphadenopathy. Bronchoscopy and endobronchial ultrasound were performed for lymph node sampling and revealed a large cell neuroendocrine tumor, which was later found to be metastatic to the brain. The patient was discharged with plans to optimize the VDZ therapy and start cancer treatment. Anticipating the use of immunotherapy, a colonoscopy was performed and revealed active inflammation in the sigmoid colon. Incidentally, an 18mm Is (sessile) polyp was found in the rectum and removed by piecemeal snare polypectomy. Pathology was consistent with invasive colonic adenocarcinoma. A colonoscopy one year earlier had been negative for malignancy. After a careful discussion in the GI tumor board, the decision was made to treat the lung cancer first. Unfortunately, the patient developed sepsis after his first chemotherapy dose and passed away. Discussion: This case illustrates the challenges with CD therapy during active malignancy. CD patients are at increased risk for both colonic adenocarcinoma and pulmonary malignancy. There have been isolated cases linking CD to premalignant diffuse idiopathic pulmonary neuroendocrine cell hyperplasia, suggesting a possible correlation between CD and the highly aggressive large cell neuroendocrine tumor of the lung seen, though data is limited. VDZ has been associated with 24%-31% clinical remission of CD after induction. It was shown to be well tolerated and had a low risk of malignancy in patients with and without a history of prior malignancy. Data is limited on the use of VDZ concurrently with active chemotherapy, though it is presumed have a favorable safety profile given the gut-selective nature of the drug. The development of malignancies in this case is thought to be possibly related to CD and likely unrelated to VDZ. With limited data available, a multidisciplinary discussion between oncology and GI is ideal to develop a tailored treatment plan for CD patients with active malignancy.

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.

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

SummaryWe describe the case of a 56 year-old woman with the almost simultaneous appearance of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) and a carotid body paraganglioma. Of interest, 6 years earlier, the patient underwent total thyroidectomy due to papillary thyroid carcinoma and, in the meantime, she was submitted to mastectomy to treat an invasive ductal carcinoma of the breast. In order to explain these lesions, an extensive genetic study was performed. Results showed positivity for the presence of the tumor suppressor gene PALB2, whose presence had already been detected in a niece with breast cancer. The patient underwent different procedures to treat the lesions and currently she is symptom-free over 2 years of follow-up.Learning points:The presence of two rare neoplasms in a single person should raise the suspicion of a common etiology.To the best of our knowledge, this is the first case that shows the coexistence of DIPNECH and paraganglioma.The contribution of the PALB2 gene in the etiology of these rare neoplasms is a possibility.

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: A Clinical Case.

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary condition characterized by diffuse proliferation of neuroendocrine cells in the epithelium of the bronchial wall. DIPNECH may be easily missed in daily clinical practice and diagnosis is often delayed, which may impair prognosis since this condition is considered a pre-invasive lesion for lung carcinoid tumours. We report a clinical case of DIPNECH in order to discuss the diagnostic and therapeutic approach for this entity, the management of which is not yet well established in the literature.LEARNING POINTSDIPNECH is a poorly understood lung condition characterized histologically by diffuse proliferation of pulmonary neuroendocrine cells in the bronchial wall, clinically by obstructive respiratory symptoms and radiologically by small airway disease features and pulmonary nodules.DIPNECH is considered to be a preneoplastic lesion within the spectrum of pulmonary neuroendocrine tumours.Treatment is usually guided by the symptoms and prognosis is highly variable.

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

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare lung disease associated with proliferation of neuroendocrine cells in the lung and multifocal neuroendocrine tumorlets/tumors. Although usually considered an indolent condition, DIPNECH causes chronic, progressive cough and dyspnea which can adversely impact quality of life. There is very limited information on the treatment of this condition. The objective of this study was to assess changes in symptoms and pulmonary function tests (PFTs) in response to somatostatin analog (SSA) treatment. Patients with clinical and/or pathologic diagnosis of DIPNECH and chronic respiratory symptoms were treated with SSAs at the H. Lee Moffitt Cancer Center and Research Institute, Hadassah-Hebrew University Medical Center, and Mayo Clinic Cancer Center. Their charts were reviewed to assess changes in symptoms and PFTs. Forty-two patients were identified who had either chronic cough or dyspnea because of proven or suspected DIPNECH and who had received treatment with an SSA. Thirty-three patients experienced symptomatic improvement. Additionally, 14 of 15 patients in whom PFTs were checked were noted to have an improvement in FEV1 after treatment. SSA treatment can improve chronic respiratory symptoms and PFTs in patients with DIPNECH.

Hiperplasia difusa idiopática de células neuroendocrinas pulmonares con formación de tumorlets: presentación de un caso clínico y revisión de la literatura

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia is an infrequent lesion recently classified by the WHO as preinvasive. It can present with the formation of tumorlets (neuroendocrine cell groups up to 5 mm) which result in a typical histological and radiological image. We report a case of a 67-year-old women who presented with a chronic cough. The CT scan showed bilateral minute, multiple pulmonary nodules. A biopsy revealed a diffuse idiopathic pulmonary neuroendocrine cell hyperplasia with several tumorlets. After two years of follow-up, imaging studies showed no significant changes.

A CASE REPORT OF DIFFUSE IDIOPATHIC PULMONARY NEUROENDOCRINE CELL HYPERPLASIA (DIPNECH)

SESSION TITLE: Wednesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: The World Health Organization(WHO) classification of lung tumors defines diffuse idiopathic pulmonary neuroendocrine cell hyperplasia(DIPNECH) as a preinvasive lesion of pulmonary carcinoids characterized by a proliferation of neuroendocrine cells confined to the mucosa of bronchi or forming tumorlets and carcinoids. Here, we present a case of DIPNECH. CASE PRESENTATION: 72-year-old female, former smoker, with PMHx of morbid obesity, OSA on CPAP and chronic cough (on albuterol) and was sent for abnormal images. She recently traveled to Aruba and noticed increased dyspnea on exertion. She had a CXR done which showed “water in her lungs” and was referred to pulmonology. She reported dry cough and progressive dyspnea on exertion for the last year, and all other ROS were negative. She was saturating 100% on RA and physical exam was unremarkable. CT chest showed multiple subpleural and bilateral upper lobe predominant nodules with ground-glass-opacities of unclear etiology. All labs including rheumatological were WNL. PFT suggested moderate restriction. Bronchoscopy showed no endobronchial lesions but transbronchial biopsy revealed small airway neuroendorcrine cell proliferation and no granulomas or malignant cells consistent with DIPNECH. DISCUSSION: DIPNECH was first defined in 1992 by Aguayo et al. and as studies have continued exploring the disease, there has been some discussion into what classified as DIPNECH. It has been used to describe two types of patients, one is based on their clinical, radiological, and pathological presentation, and the other is defined purely histologically in asymptomatic patients. Symptomatic DIPNECH has been diagnosed mostly in female non-smokers who present with cough, exertional dyspnea and wheezing. Due to their similar presentation, patients may be misdiagnosed with asthma or GERD. Abnormal LFTs is a typical lab finding. CT Images show air trapping or mosaicism. Diagnosis of DIPNECH requires histological confirmation of diffuse neuroendocrine cell proliferation and the presence of constrictive bronchiolitis by transbronchial biopsy, which is found in both types of DIPNECH described in literature. The course of DIPNECH is typically indolent but there are cases that report slow progression of DIPNECH leading to respiratory failure. Octreotide has been used for symptomatic coughing, but reports have not shown that it improves the course of the disease. Symptomatic treatment for obstruction and lung transplantation in select patients are the current therapeutic options for DIPNECH. CONCLUSIONS: DIPNECH is a rare disease and clinicians should have a high index of suspicion for patients presenting with indolent pulmonary symptoms. Reference #1: Davies, Susan J., et al. "Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognised spectrum of disease." Thorax 62.3 (2007): 248-252. Reference #2: Aguayo, Samuel M., et al. "Idiopathic diffuse hyperplasia of pulmonary neuroendocrine cells and airways disease." New England Journal of Medicine 327.18 (1992): 1285-1288. Reference #3: Wirtschafter, Eric, et al. "Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia of the lung (DIPNECH): current best evidence." Lung 193.5 (2015): 659-667. DISCLOSURES: No relevant relationships by Mona Alipour, source=Web Response No relevant relationships by Khoi Paul Dang-Ho, source=Web Response No relevant relationships by Louis Gerolemou, source=Web Response No relevant relationships by Nabil Mesiha, source=Web Response

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia associated with bronchiectasis: A rare case

Noroendokrin hucre hiperplazisi cogunlukla havayolu mukozasinda bazal membrani penetre etmeyecek sekilde ortaya cikan nadiren tum akcigerde yaygin odaksal ekstraluminal nodul olusturan noroendokrin tumor onculu proliferasyonlardir. Bu calismada 30 yasinda, genisletilmis sakkuler bronsektazi nedeniyle opere edilen ve cerrahi spesmenin histopatolojik degerlendirmesinde ek olarak diffuz idiyopatik pulmoner noroendokrin hucre hiperplazisi saptanan olgu sunuldu.

DIPNECH: The Mayo experience.

e20029 Background: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a pulmonary disorder with neuroendocrine cell proliferation with potential progression to lung neuroendocrine tumor (Lu-NET). Optimal diagnostic and treatment strategies have yet to be well defined. Herein, we aim to describe the Mayo Clinic experience with DIPNECH. Methods: A retrospective analysis was performed of patients diagnosed with DIPNECH within Mayo Clinic between January 2000-Febuary 2019. Cases were identified from clinical databases at Mayo Clinic. Data on demographics, disease characteristics, time of diagnosis, surgery, and last follow up date were extracted. Extent of symptom burden, treatment approaches, disease progression, and disease-free survival (DFS) were evaluated by chart review. Results: A total of 59 patients were identified with a median age of 63(43-81) years. The cohort was predominantly female (93.2%) and non-smoking (76.3%). Most patients (86.4%) had symptomatic disease with chronic cough being the most common (71.2%) followed by exertional dyspnea (44.1%). Imaging typically showed bilateral lung nodules (93.2%) with mosaic attenuation noted 69.5% of the time. Surgical resection was frequently completed to confirm diagnosis (94.9%). Most patients received inhaled glucocorticoids combined with a beta agonist (79.7%). Oral steroid use was seen in 49.2% of patients whereas a somatostatin analog was used in 15.3% following the diagnosis of DIPNECH. These medical interventions led to symptom relief in 23.7% of the patients. The median follow up for all patients was 19.5 months. Progression of tumorlets was seen in 48.7% of patients with only 7(17.9%) patients progressing to a diagnosis of Lu-NET. The 3-year DFS was 90.5%. Conclusions: This is amongst the largest studies completed evaluating DIPNECH patients. DIPNECH remains a rare disease more commonly diagnosed in women in their early 60’s. DIPNECH appears to have an indolent course with obstructive symptoms being the most common finding. A minority of patients experienced symptom relief with therapy. Overall, DIPNECH appears to have a low risk of progressing to Lu-NET based on the observation of this study.

A case series of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia

A case series of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia

DIPNECH: A UNIQUE PRESENTATION OF A RARE DISEASE

SESSION TITLE: Diffuse Lung Disease 2 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 pm - 02:15 pm INTRODUCTION: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a very rare disease that consists of a proliferation of scattered single cells, small nodules, or linear proliferations of pulmonary neuroendocrine cells (1). The entity has been described as a precursor to pulmonary carcinoid tumors (2), is thought to be more common in middle-aged women, and can be associated with slowly-progressive cough or shortness of breath with exertion (1,3). CASE PRESENTATION: We present a case of a 42-year-old female with a complicated pulmonary history including a childhood diagnosis of asthma, two spontaneous pneumothoraces, chronic daily cough, dyspnea on exertion, and a seven-year history of incidentally-noted pulmonary nodules. An initial workup for her nodules was negative for fungal or autoimmune etiologies. An interval CT scan was obtained and showed an increase in nodule size which was further evaluated with a PET-CT. While none of the lung nodules were PET-avid, a hypermetabolic thyroid nodule was incidentally noted, which was biopsied and found to be papillary thyroid cancer. Pulmonary function testing was obtained for her dyspnea and showed mild restriction with no evidence of obstruction and normal diffusion capacity. After a non-diagnostic CT-guided biopsy of a right middle lobe lung nodule led to an iatrogenic pneumothorax requiring chest tube placement, she eventually underwent video assisted thoracoscopic surgery and right middle and lower lobe wedge resections. Biopsy showed the presence of a typical carcinoid tumor as well as neuroendocrine hyperplasia consistent with DIPNECH. DISCUSSION: This case represents an atypical presentation of a rare disease. Though pulmonary nodules, shortness of breath, and cough are common findings in DIPNECH, the occurrence of spontaneous pneumothoraces is not. Few case reports have described spontaneous pneumothoraces in patients with large endobronchial carcinoid tumors; however, this patient had no evidence of endobronchial obstruction and thus represents a unique presentation of the disease (4,5). This case also demonstrates the importance of maintaining a broad differential in the approach to patients presenting with dyspnea, cough, and pulmonary nodules. While the DIPNECH is very rare, appropriate diagnosis and close follow-up is important given the association of DIPNECH with subsequent development of carcinoid tumors (6). CONCLUSIONS: The differential for diffuse pulmonary nodules in the setting of chronic cough or dyspnea is incredibly broad. After more common etiologies like metastatic disease, granulomatous disease, autoimmune, and infectious disorders are ruled out, one should consider the diagnosis of DIPNECH, particularly in a middle-aged female with chronic, otherwise-unexplained respiratory symptoms and especially with an associated diagnosis of a carcinoid tumor (2). Reference #1: Travis WD, Brambilla EW, Burke AP, et al. WHO Classification of Tumours of the Lung, Pleura, Thymus, and Heart, IARC Press, Lyon 2015. Reference #2: Gorshtein, A., Gross, D. J., Barak, D., Strenov, Y., Refaeli, Y., Shimon, I. and Grozinsky-Glasberg, S. (2012), Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia and the associated lung neuroendocrine tumors. Cancer, 118: 612–619. https://doi.org/10.1002/cncr.26200. Reference #3: Nassar A. A., Jaroszewski D. E., Helmers R. A., Colby T. V., Patel B. M., Mookadam F. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia—a systematic overview. American Journal of Respiratory and Critical Care Medicine. 2011;184(1):8–16. https://doi.org/10.1164/rccm.201010-1685pp. DISCLOSURES: No relevant relationships by Carleen Risaliti, source=Web Response No relevant relationships by Kyle Stinehart, source=Web Response

1323P - Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) in patients with pulmonary carcinoid tumours: Prevalence and prognosis of an under-recognised disease

1323P - Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) in patients with pulmonary carcinoid tumours: Prevalence and prognosis of an under-recognised disease

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

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary condition, characterized by diffuse proliferation of neuroendocrine cells in the respiratory epithelium. DIPNECH lesions are less than 5mm in size and are limited to the basement membrane with no invasion. There is limited information regarding epidemiology, natural history of disease progression, or the management of this rare entity. We present the experience of a center with extensive expertise in neuroendocrine disease. A cohort of patients (N = 13) with DIPNECH treated and followed at our institution was identified. We describe the our approach to their care, our disease management and also provide a review of DIPNECH pathophysiology. Our patient cohort consisted of twelve females and one male with a mean age of 63years at the time of diagnosis. Dyspnea on exertion and dry cough were the most common presenting symptoms. Two patients were under surveillance without treatment; three patients were treated with a short-acting somatostatin analog; three patients were treated with azithromycin alone; four were treated with a combination of long-acting monthly somatostatin analogs and azithromycin; one patient received a combination of long-acting somatostatin analog and everolimus. Five patients had concomitant bronchial carcinoids. DIPNECH is a rare pathology that can profoundly affect a patient's quality of life. Paroxysmal coughing episodes can be difficult to treat. Our limited single center experience shows encouraging response to use of somatostatin analogs, azithromycin, and everolimus in the management of debilitating DIPNECH associated symptoms.