Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: A Rare Case Involving the Trachea

Abstract

SESSION TITLE: Lung Pathology 2 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare primary pulmonary disorder with good prognosis and is a preneoplastic condition for carcinoid tumors with around 100 cases reported in literature. It is being recognized with increasing incidence and is considered the initial manifestation of pulmonary neuroendocrine disease. CASE PRESENTATION: A 50 year old non-smoker woman with history of shortness of breath for 2 years after which she was diagnosed with asthma, presented to an urgent care with increase shortness of breath. During that time she was treated with inhalers and antibiotics without improvement after which she decided to follow up with a primary care physician who did further testing. Echocardiogram and stress testing were unremarkable. Chest X-Ray demonstrated a curious right mid lung zone mass. Bronchoscopy was performed and revealed a bloody, partially obstructing endobronchial polypoid mass in the anterior basal segment of the RLL [figure 1]. Endobronchial fine needle aspiration and surgical lung biopsy showed low grade neuroendocrine tumor most consistent with carcinoid neoplasm. Interestingly, during bronchoscopy, a curious small non-obstruction infiltrative, hyperemic and friable lesion was found proximally in the upper Trachea [figure 2]. DISCUSSION: DIPNECH is a rare disorder with predilection for middle-aged, non-smoking women. Diagnosis is challenging and frequently delayed for years due to insidious presentation and is often confused with asthma. High degree of suspicion is needed for early diagnosis, management and long term observation of patients with this disorder that can progress to severe airway obstruction and even death. Our case is unique given that DIPNECH is a very rare and underdiagnosed pulmonary disease and to our knowledge, this is the first reported case of DIPNECH involving the Trachea. CONCLUSIONS: DIPNECH is a rare pulmonary disorder. Diagnosis is extremely challenging and frequently delayed for years. Present understanding of the clinical course of DIPNECH is still poor and based primarily on small case reports and thus evidence-based diagnosis, treatment and follow-up haven’t been established. Reference #1: Laurie L. Carr, Jonathon H. Chung, Rosane Duarte, et al.: The Clinical Course of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia, Journal of the American College of Chest Physicians, Vol. 147, No.2, 2015. DISCLOSURE: The following authors have nothing to disclose: Ala Nijim, Yousef Shweihat, Nancy Munn No Product/Research Disclosure Information