| Title: | Clinical characteristics of a Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia Cohort |
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| 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 |
| Category: | Lung Cancer/CF/ILD/Surgery |
| 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. |
