Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia. A Case Report.

Abstract

Introduction; Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a sporadic but underdiagnosed pulmonary disorder at the benign end of the neuroendocrine cell proliferation spectrum of preinvasive lesions of the lungs. This disease is characterized by hyperplasia of bronchiolar and bronchial pulmonary neuroendocrine cells. DIPNECH can be primary or reactive.

In the WHO - IASLP classification of lung tumors (1999, 2004, 2005, 2015), DIPNECH is considered a preneoplastic lesion in the spectrum of pulmonary tumors. According to the WHO classification, the definition of DIPNECH is purely histological.  

The DIPNECH was initially described in 1992 by Aguayo et al., who reported six non-smoking patients with cough, exertional dyspnea, wheezing, less frequent hemoptysis, and a mixed obstructive/restrictive defect on pulmonary function tests. This disease has a predilection for non-smoking middle-aged women (female to male ratio is approximately 10:1) 

In this article, we present a 62-year-old, non-smoker woman presented with respiratory symptoms ascribable to DIPNECH. After surgery, the morphological study of lung specimens confirmed the DIPNECH, multiple tumors, one peripheral carcinoid, and obliterative bronchiolitis in the right middle pulmonary lobe. 

Conclusions: DIPNECH remains a rare pulmonary condition and is considered a preneoplastic lesion in the spectrum of pulmonary tumors. According to the WHO classification, the definition of DIPNECH is purely histological. While most patients experience a relatively uneventful clinical course, this condition may be associated with tumors, carcinoid tumors, and airway obstruction (Aguayo-Miller disease).