Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia is an extremely rare pulmonary lesion, with only 39 cases reported in the literature. We report an additional case and review the literature. The patient is a 41-year-old man with a 5-year history of progressive dyspnea, cough, and wheezing. He was initially diagnosed as having bronchial asthma but did not respond to treatment of bronchodilators and inhaled steroids. Pulmonary function tests showed airflow obstruction. Chest computed tomography revealed a mosaic pattern of air trapping and thickening of bronchial walls. Open lung biopsy showed diffuse proliferation of pulmonary neuroendocrine cells within the bronchiolar epithelium, often bulging into or obliterating the bronchiolar lumen. These cells also breached the basement membrane, forming tumorlets. There was prominent peribronchiolar fibrosis and obliterative bronchiolitis. The pathologic evaluation of lung tissue is currently the gold standard in making a definitive diagnosis of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia, and all the reported cases were diagnosed by either open lung biopsy or lobectomy.
