History : 60-year-old woman with progressive breathlessness and episodic ‘flushing’. Life-long non smoker. No relevant past medical or exposure history Case of the Month 8 February 2016

Case of the Month 8

What is your diagnosis ? Case of the Month 8

History : 60-year-old woman with progressive breathlessness and episodic ‘flushing’. Life-long non smoker. No relevant past medical or exposure history Case of the Month 8 February 2016 Authors: A Utrera, S Tran, SR Desai, London

Case of the Month 8

Question: What is shown on the HRCT?

Case of the Month 8  Mosaic attenuation pattern  Multiple round pulmonary nodules  cm diameter  Non-calcified Question: What is shown on the HRCT?

Case of the Month 8 Diagnosis : Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH)

Case of the Month 8 Discussion  Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) is characterised by proliferation of neuroendocrine cells confined by the epithelial basement membrane  Epidemiology:  Rare  F > M  th decade  Non-smokers  Symptomatic in ~50% (cough, dyspnoea, wheezing)  Irreversible airflow obstruction on PFTs caused by luminal obliteration

HIGH-RESOLUTION CT  Mosaic attenuation (±air trapping on expiratory CT)  Multiple round/oval pulmonary nodules  Airway abnormalities  Bronchial wall thickening  Airway plugging  Bronchiectasis Case of the Month 8 Discussion

Case of the Month 8 Further Reading Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia DIPNECH 1)Chassagnon G et al. DIPNECH: when to suggest this diagnosis on CT Clinical Radiology 2015;70:317–325 2) Davies SJ et al. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under- recognised spectrum of disease Thorax 2007;62:248–252 3) Gorshtein A et al. Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia and the Associated Lung Neuroendocrine Tumors Cancer 2012;118: ) Koo CW et al. Spectrum of pulmonary neuroendocrine cell proliferation: diffuse idiopathic pulmonary neuroendocrine cell hyperplasia, tumorlet and carcinoids AJR Am J Roentgenol 2010; 195:661–668 5) Benson REC et al. Spectrum of Pulmonary neuroendocrine proliferations and neoplasms RadioGraphics 2013; 33:1631–1649