Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Review of the Literature and a Single-center Experience

Abstract

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare disorder that is commonly underdiagnosed. In 2015, it was recognized by the World Health Organization (WHO) classification of lung tumors as a premalignant lesion. DIPNECH syndrome is characterized by cough, exertional dyspnea, wheezing, and, less frequently, hemoptysis. We report the clinical and histological features and imaging findings in four cases of DIPNECH from our institution (Torrejon University Hospital, Madrid, Spain) between the years 2012 and 2019. DIPNECH represents a rare and poorly understood pulmonary disorder. Our limited single-center experience shows the slow and stable evolution of the disease. However, some exceptional cases may progress poorly if distant metastases occur.

Constrictive bronchiolitis in diffuse idiopathic pulmonary neuroendocrine cell hyperplasia

“…Similar to others, our study demonstrates that DIPNECH remains stable or progresses very slowly in most patients, but can result in severe airflow limitation and respiratory impairment [ 8 , 10 , 13 , 26 , 28 , 35 , 36 ].…”

“…Similar to others, our study demonstrates that DIPNECH remains stable or progresses very slowly in most patients, but can result in severe airflow limitation and respiratory impairment. [8,10,13,26,28,35,36] Our study has limitations inherent to the retrospective single-center design and the rarity of the disease under study. The number of subjects in this study is modest and the features described within may reflect the more severe end of the disease spectrum due to referral bias.…”

“…A small case series reported drastic improvement of cough in all four patients treated with SSA [ 35 ]. In a small case series, one patient with DIPNECH received an SSA, with temporary improvement in her cough [ 36 ]. In our study, 14 patients received an SSA; 4 (29%) experienced improvement in their cough.…”

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: clinical characteristics and progression to carcinoid tumour

“…After its first case report in 1992, 6 a limited number of small case series have since been published. 3,4,[7][8][9][10] To our knowledge, this study is one of the largest to report the characteristics of patients with pathological and clinical DIPNECH in comparison to those with secondary NECH/tumorlets, and one of the first to report the growth kinetics of these preinvasive lesions to guide surveillance planning.…”

Air trapping in small airway diseases: A review of imaging technique and findings with an overview of small airway diseases

“…The majority of patients with DIPNECH are middle‐aged (5th and 6th decades) non‐smoking females 15 . Only half of the patients are symptomatic, usually presenting with non‐productive cough, dyspnoea and wheeze 27 . Asymptomatic patients are usually diagnosed incidentally 26 .…”