Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia
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Imaging findings
Chest radiography shows faint nodular opacities. Chest CT shows widespread mosaic perfusion representing air trapping, and multiple well-defined, non-calcified pulmonary nodules up to one centimeter in size. A PET CT demonstrates that the larger nodules are non-avid, and biopsy reveals a WHO grade 1 typical carcinoid.
Key takeaways
DIPNECH is a rare, under-recognized neuroendocrine cell proliferation of the airways that typically affects non-smoking females. It presents with progressive wheeze or breathlessness mimicking asthma, and is characterized on imaging by a combination of multiple pulmonary nodules (representing tumorlets or carcinoid tumors) and mosaic perfusion/air trapping due to constrictive bronchiolitis.
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