Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia

Definition

A rare pulmonary neuroendocrine tumor, most often presenting in women over 50 years and no smokers, characterized by long-lasting cough (>10 years), and less frequently by dispnea or wheezing. A proportion of patients are asymptomatic, the disease being an incidental finding during routine check-ups. Diffuse pulmonary nodules and mosaic attenuation can be detected by CT-scan however only histopathology can confirm the diagnosis detecting a multifocal hyperplasia of pulmonary neuroendocrine cells (carcinoid tumorlets). Ventilatory dysfunction can be obstructive and less often restrictive, mixed or non-specific. Symptoms can progress slowly but sometimes lead to respiratory failure. It is considered as a precursor for pulmonary carcinoid tumor.