CTICasesMultifocal Cystic Fibrohistiocytic Tumor of the Lung
Imaging findings
Chest CT demonstrates multiple thin-walled, spherical, and perivascular pulmonary cysts distributed throughout both lungs, which have remained remarkably stable over a 10-to-12-year follow-up period. A stable, small soft-tissue nodule is also present in the right lower lobe. Pathology showed features of multifocal cystic fibrohistiocytic tumor, including metaplastic squamous epithelium lining the cysts.
Key takeaways
Multifocal cystic fibrohistiocytic tumor of the lung is an extremely rare, low-grade malignant neoplasm that can mimic other cystic lung diseases such as Birt-Hogg-Dubé syndrome or light chain deposition disease. Biopsy often reveals characteristic fibrohistiocytic proliferation and squamous metaplasia.
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