Médecine pulmonaire et respiratoire

DIPNECH (Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia): A Rare Syndrome or Undiagnosed?

Abstract

Rafaela B. Campanha, Cristina Matos and Fernando Nogueira

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is considered a rare syndrome and knowledge of its pathophysiology is scarce. Classified as pre-neoplastic lesion of carcinoid tumours currently there are many questions regarding the best approach to follow-up and treatment this patients. Mainly affects females, there is no apparent relationship to smoking habit and is often confused with obstructive airway disease. The diagnosis is histological and sometimes can be observed simultaneously a triple pattern consisting of DIPNECH, tumourlets and carcinoid tumour. We report the case of a 35-years-old woman, non-smoker with DIPNECH in association with typical carcinoid tumour and tumourlets.