Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is an extremely rare pulmonary disorder where there is a proliferation of neuroendocrine cells within the lung. 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 for pulmonary carcinoid tumours.

CT/HRCT chest

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

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 clinical deterioration (rare 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.

 

Share article

Article information

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

Support Radiopaedia and see fewer ads

Cases and figures

  • Drag
    Case 1
    Drag here to reorder.
  • Drag
    Case 2: with probable carcinoid
    Drag here to reorder.
  • Updating… Please wait.
    Loadinganimation

    Alert accept

    Error Unable to process the form. Check for errors and try again.

    Alert accept Thank you for updating your details.