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Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia▶ Watch on YouTube — age-restricted, cannot embed here

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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