|Title:||Clinical characteristics of a Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia Cohort|
|Author(s):||M. Gleeson, A. Fabre, D. O’ Toole, R. Crowley, D. Murphy, C. Mc Carthy|
|Institution:||St. Vincent's University Hospital|
|Poster:||Click to view poster|
|Abstract:||Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) is a rare disease typically of middle aged non smoking women, with few cases described. After first recognitition in 1992, the WHO described it as a precursor to pulmonary carcinoid tumours. Currently there is no consensus on radiological and/or pathological criteria for diagnosis. Herein we aim to describe our experience of DIPNECH in St. Vincent’s University Hospital. |
A retrospective analysis of 15 cases was performed. Data collected included: Age, gender, smoking status, CT findings, PFTs, treatment and assocaited neuroendocrine tumours.
All patients were female with a mean age of 60.2years. 9 patients had respiratory symptoms at diagnosis. 10 patients had lung function tested with an obstructive pattern seen in 40%. CT findings included a dominant nodule(n=7), associated nodules(n=13) and mosaicism(n=5). 13 cases were associated with a pulmonary carcinoid. Treatment included somatostatin analogues and inhaled corticosteroids in 5 and 5 patients respectively.
This is amongst the largest studies evaluating DIPNECH patients in a single centre. DIPNECH is a histological diagnosis, often asymptomatic and therefore underdiagnosed. Obstructive symptoms and lung function were most commonly seen. Given its rarity and current lack of evidence based guidelines a specialist centre is most appropraite for managing such patients.