A case series of 7 patients with Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) in New South Wales Australia

Lisa Basler, Marsa Hosseinzadeh, Dariush Daneshvar, Peter Wu

Abstract

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) is a rare and poorly understood entity, defined as a generalised proliferation of pulmonary neuroendocrine cells arranged in scattered single cells, small nodules or linear pattern that do not invade beyond the bronchial epithelial basement membrane.

The diagnosis of DIPNECH requires clinical, radiological and histopathology correlation. However, it is often diagnosed incidentally on histopathological examination of lung biopsy specimen performed for other reasons or discovered due to investigations for unexplained respiratory symptoms.

In the first publication of a case series of DINPNECH and NECH in Australia, we describe seven histopathologically confirmed cases of DIPNECH or NECH. All patients were females, aged 57–70, mostly non-smoker. The most common presenting symptoms were dry cough and progressive dyspnoea. 71 per cent of patients had typical radiological findings with nodular changes.
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