Lung Pathology: Student/Resident Case Report Poster - Lung Pathology I |

Not All That Wheezes Is Asthma FREE TO VIEW

Areeb Zamir, MD; Noor Salam, MD; Andy Iriza, DO; Andres Zirlinger, MD
Author and Funding Information

Lehigh Valley Health Network, Emmaus, NJ

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):797A. doi:10.1016/j.chest.2016.08.893
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SESSION TITLE: Student/Resident Case Report Poster - Lung Pathology I

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare condition characterized by abnormal growth of lung neuroendocrine cells. DIPNECH has been commonly misdiagnosed as asthma and other pulmonary conditions.1 Given its increasing prevalence, improving its awareness is vital and may lead to more prompt diagnosis. Here, we present a case of a 71-year-old lady with DIPNECH.

CASE PRESENTATION: A 71-year-old non-smoking white female with no significant occupational exposure presents with cough and shortness of breath for many years. She was previously treated for asthma with inhaled corticosteroids and a long acting beta agonist with partial success. During a suspected asthma exacerbation, a CT of the chest to rule out pulmonary embolism revealed multiple sub-centimeter peripheral nodules and marked mosaic attenuation suggestive of air trapping. Further work-up with a PET CT showed mild metabolic activity of the larger nodules. Her pulmonary functioning tests revealed mild obstruction and a normal diffusion capacity. The patient underwent a surgical lung biopsy, which revealed multiple typical carcinoid tumorlets measuring less than 1 cm and proliferation of neuroendocrine cells consistent with neuroendocrine cell hyperplasia.

DISCUSSION: The diagnosis of DIPNECH can be challenging. Review of the literature shows a limitation in the use of serum biomarkers because neuroendocrine products cannot be broken down into readily measured levels. CT scan of the chest usually reveals small sub-centimeter nodules, which are less than 5mm in 60% of cases. Mosaic attenuation is also commonly found. Less common findings are ground glass opacities as well as bronchiectasis; however, no radiological finding is pathognomonic. The role of bronchoscopy is unclear as there are no specific findings on bronchiolar lavage although some reports of diagnoses of DIPNECH through trans-bronchial biopsies have been described. The gold standard for diagnosis is a surgical lung biopsy.2 In order to ensure histopathological diagnosis of DIPNECH, neuroendocrine cell hyperplasia must be seen. Carcinoid tumorlets are seen in more than 70% of patients and as was seen in our patient. Various treatment strategies have been described including systemic/inhaled steroids, bronchodilators and lung resection.

CONCLUSIONS: The diagnosis and management of DIPNECH remains challenging because of varying presentation and lack of non-invasive testing. It requires a high index of suspicion in patients who have multiple small pulmonary nodules, especially in women with associated symptoms of cough and wheezing. Further case studies would be beneficial in order to better understand the diagnosis and management of this rare pulmonary condition.

Reference #1: Diffuse Idiopathic Pulmoanry Neuroenocrine Cell Hyperplasia: A Systemic Overview. Adrienne A. Nassar, Dawn E. Jaroszewski, Richard A. Helmers. American Journal of Respiratory and Critical Care Medicine. Volume 184, 2011.

DISCLOSURE: The following authors have nothing to disclose: Areeb Zamir, Noor Salam, Andy Iriza, Andres Zirlinger

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