Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia

Dr Henry Knipe and Dr Yuranga Weerakkody et al.

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 characterised by several nodules and extensive mosaic attenuation. It is, however, recognised 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 dyspnoea 7.

It is characterised by a generalised 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 tumours.

Various features have described, each of which is non-specific on their own. These include:

The rarity of the condition poses some clinical challenge in 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.

The first clinical series of six cases describing DIPNECH was published by Aguayo et al. in 1992.


Article information

rID: 20656
System: Oncology, Chest
Synonyms or Alternate Spellings:
  • Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH)

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Cases and figures

  • Case 1
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  • Case 2: with probable carcinoid
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  • Case 3
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