CTICasesDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNE) with Cystic Lung Disease
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Imaging findings
A 72-year-old woman presented with progressive shortness of breath and dry cough. CT showed scattered nodules, predominant small cystic lesions, and scattered areas of lobular air trapping. Biopsies from the right middle and lower lobes confirmed DIPNE with tumorlets ranging from 1 to 8 mm, including the resected cystic lesions. Abdominal imaging revealed simple renal cysts. Lungs remained stable, but she developed chronic pulmonary hypertension.
Key takeaways
This is an unusual presentation of DIPNE, characterized by prominent cystic lung disease in addition to the typical nodules and air trapping. The pathogenesis of the cysts is postulated to be related to ball-valve obstruction of small airways by the tumorlets, highlighting a rare morphologic variant of DIPNE and the challenge of differentiating it from other cystic lung diseases.
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