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Malignant Histiocytosis Presenting with Pneumothorax

Imaging findings

CT chest and PET-CT show rapidly progressive, lower-lobe-predominant pulmonary nodules and thick-walled cysts, associated with a spontaneous left pneumothorax. The patient had extrathoracic disease in the thyroid, and biopsy confirmed malignant histiocytosis (histiocytic sarcoma).

Key takeaways

Malignant histiocytosis (histiocytic sarcoma) is a rare malignant neoplasm of histiocytic origin. When involving the lungs, it can radiographically mimic pulmonary Langerhans cell histiocytosis (PLCH) with nodules and cysts, but it displays a lower-lobe predominance and a highly aggressive clinical course.

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