Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia
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A carcinoid tumourlet, as may be seen in the context of DIPNECH. (WC/Nephron)
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia, abbreviated DIPNECH, is an uncommon lung pathology that is considered pre-neoplastic by the WHO.[1]
General
- Rare - less than 50 reported based on a 2010 paper.[2]
- Usually women (15 of 19 cases[3]).
- Typically non-smokers (16 of 19 cases[3]).
- DIPNECH has been described in the context of multiple endocrine neoplasia 1.[3]
Clinical:[4]
- Typical patient in 60s.
- Cough.
Treatment:
- Somatostatin analogs[4] - improves cough.
Criteria
Marchevsky and Walts criteria - both 1 & 2 are required:[5]
- Three or more separate small airways pulmonary neuroendocrine cell clusters.
- Each cluster should have five or more pulmonary neuroendocrine cells.
- Three or more carcinoid tumourlets.
Notes:
- The Marchevsky and Walts definition is not universally accepted.
- World Health Organization (WHO) definition is vague.
Gross
- Multiple small pulmonary nodules.[1]
DDx:
Microscopic
See also
References
- ↑ 1.0 1.1 Wirtschafter, E.; Walts, AE.; Liu, ST.; Marchevsky, AM. (Jun 2015). "Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia of the Lung (DIPNECH): Current Best Evidence.". Lung. doi:10.1007/s00408-015-9755-1. PMID 26104490.
- ↑ Tippett, VM.; Wathen, CG. (2010). "Diffuse idiopathic neuroendocrine cell hyperplasia: an unusual cause of breathlessness and pulmonary nodules.". BMJ Case Rep 2010. doi:10.1136/bcr.05.2010.3006. PMID 22798306.
- ↑ 3.0 3.1 3.2 Davies, SJ.; Gosney, JR.; Hansell, DM.; Wells, AU.; du Bois, RM.; Burke, MM.; Sheppard, MN.; Nicholson, AG. (Mar 2007). "Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognised spectrum of disease.". Thorax 62 (3): 248-52. doi:10.1136/thx.2006.063065. PMID 17099078.
- ↑ 4.0 4.1 Chauhan, A.; Ramirez, RA. (Jun 2015). "Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) and the Role of Somatostatin analogs: A Case Series.". Lung. doi:10.1007/s00408-015-9754-2. PMID 26112453.
- ↑ Marchevsky, AM.; Walts, AE. (Nov 2015). "Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH).". Semin Diagn Pathol 32 (6): 438-44. doi:10.1053/j.semdp.2015.08.002. PMID 26472691.