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]).

Clinical:[4]

  • Typical patient in 60s.
  • Cough.

Treatment:

  • Somatostatin analogs[4] - improves cough.

Criteria

Marchevsky and Walts criteria - both 1 & 2 are required:[5]

  1. Three or more separate small airways pulmonary neuroendocrine cell clusters.
    • Each cluster should have five or more pulmonary neuroendocrine cells.
  2. Three or more carcinoid tumourlets.

Notes:

  • The Marchevsky and Walts definition is not universally accepted.
  • WHO definition is vague.

Gross

  • Multiple small pulmonary nodules.[1]

DDx:

Microscopic

See pulmonary neuroendocrine cell hyperplasia.
See typical lung carcinoid tumour.

See also

References

  1. ↑ 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.
  2. ↑ 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. ↑ 3.0 3.1 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. ↑ 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.
  5. ↑ 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.
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