Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is an extremely rare pulmonary disorder at the benign end of the neuroendocrine cells proliferation spectrum. It is mainly seen in non-smoker middle age females with a history of chronic cough or asthma.
On imaging, it is characterized by several nodules and extensive mosaic attenuation. It is, however, recognized with increasing frequency.
There may be an increased female predilection 3. Most patients tend to be non-smokers.
Most are asymptomatic, where discovery is made incidentally on thoracic imaging for an unrelated reason. Some, however, may present with symptoms such as an insidious cough or a slowly progressive dyspnea 7.
It is characterized by a generalized proliferation of scattered single cells, small nodules or linear proliferations of pulmonary neuroendocrine cells (enterochromaffin cells, "Kulchitsky cells") 9. It is sometimes considered to be a precursor to pulmonary carcinoid tumors.
Various features have described, each of which is non-specific on their own. These include:
- small lung nodules: considered a generally frequent feature 3-4
- ground-glass changes 3
- mosaic perfusion pattern 6-7: due to air trapping
- bronchial wall thickening 7
- mucus plugging
- bronchiectasis 3,7
Treatment and prognosis
The rarity of the condition poses some clinical challenge on establishing treatment and assessing prognosis. Limited studies suggest that the condition can have a variable prognosis ranging from clinical improvement to being stable to rare clinical deterioration 5. Various treatment strategies have been described which include systemic and inhaled corticosteroids, bronchodilators, and lung resection.
History and etymology
The first clinical series of six cases describing DIPNECH was published by Aguayo et al. in 1992.
- 1. Warth A, Herpel E, SchmäHl A et-al. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) in association with an adenocarcinoma: a case report. J Med Case Rep. 2008;2 (1): 21. doi:10.1186/1752-1947-2-21 - Free text at pubmed - Pubmed citation
- 2. Ge Y, Eltorky MA, Ernst RD et-al. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. Ann Diagn Pathol. 2007;11 (2): 122-6. doi:10.1016/j.anndiagpath.2005.12.008 - Pubmed citation
- 3. Nassar AA, Jaroszewski DE, Helmers RA et-al. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: a systematic overview. Am. J. Respir. Crit. Care Med. 2011;184 (1): 8-16. Am. J. Respir. Crit. Care Med. (full text) - doi:10.1164/rccm.201010-1685PP - Pubmed citation
- 4. Gorshtein A, Gross DJ, Barak D et-al. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia and the associated lung neuroendocrine tumors: clinical experience with a rare entity. Cancer. 2012;118 (3): 612-9. Cancer (full text) - doi:10.1002/cncr.26200 - Pubmed citation
- 5. Davies SJ, Gosney JR, Hansell DM et-al. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognised spectrum of disease. Thorax. 2007;62 (3): 248-252. Thorax (full text) - doi:10.1136/thx.2006.063065 - Free text at pubmed - Pubmed citation
- 6. Johney EC, Pfannschmidt J, Rieker RJ et-al. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia and a typical carcinoid tumor. J. Thorac. Cardiovasc. Surg. 2006;131 (5): 1207-8. doi:10.1016/j.jtcvs.2005.12.064 - Pubmed citation
- 7. Koo CW, Baliff JP, Torigian DA et-al. Spectrum of pulmonary neuroendocrine cell proliferation: diffuse idiopathic pulmonary neuroendocrine cell hyperplasia, tumorlet, and carcinoids. AJR Am J Roentgenol. 2010;195 (3): 661-8. AJR Am J Roentgenol (full text) - doi:10.2214/AJR.09.3811 - Pubmed citation
- 8. Patel C, Tirukonda P, Bishop R et-al. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) masquerading as metastatic carcinoma with multiple pulmonary deposits. Clin Imaging. 2012;36 (6): 833-6. doi:10.1016/j.clinimag.2012.01.022 - Pubmed citation
- 9. Aubry MC, Thomas CF, Jett JR et-al. Significance of multiple carcinoid tumors and tumorlets in surgical lung specimens: analysis of 28 patients. Chest. 2007;131 (6): 1635-43. doi:10.1378/chest.06-2788 - Pubmed citation
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