CTICasesFollicular Dendritic Cell Carcinoma with Paraneoplastic Autoimmune Multi-organ Syndrome (PAMS)
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Imaging findings
An anterior mediastinal mass with low attenuation components (initially thought to be teratoma) was resected. Pathology was initially read as thymoma but later reinterpreted as follicular dendritic cell carcinoma. The patient developed progressive shortness of breath, and a follow-up CT showed significant diffuse air trapping and bronchiectasis, consistent with obliterative/constrictive bronchiolitis. The patient also had lichen planus-like skin lesions.
Key takeaways
This rare case illustrates Paraneoplastic Autoimmune Multi-organ Syndrome (PAMS) associated with follicular dendritic cell carcinoma (or thymoma). PAMS can manifest with lichenoid skin reactions and progressive constrictive bronchiolitis, an irreversible airways disease. The lung disease can worsen over time but may be arrested with high-dose steroids.
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