CTICasesLangerhans Cell Histiocytosis
Imaging findings
A man around 25 has ill-defined upper and mid lung blobs that increased markedly over three years on radiographs. CT shows peribronchial, sarcoid-like nodular opacities with some low-attenuation and cavitary components tapering toward the lower lungs, with dramatic worsening on the most recent scan that suggested malignant transformation. Lung biopsy confirmed Langerhans cell histiocytosis, and he has bone lesions (mastoid, skull/parietal, pelvic, rib, scapular). The lung findings improved after chemotherapy.
Key takeaways
Langerhans cell histiocytosis can present in a young adult with a systemic, non-smoking-related form featuring aggressive peribronchial nodular lung disease with cavitation plus multifocal bone lesions, and can worsen dramatically enough to mimic malignant transformation to lymphoma. It is treated with chemotherapy (with targeted therapy possible if a BRAF or MEK-pathway mutation is found, though responses may be less durable), and the lung disease can regress with treatment.
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