CTICasesDiffuse Pulmonary Lymphangiomatosis
Imaging findings
Initial chest radiographs and CT in a young woman show shaggy interstitial thickening, mediastinal and pleural infiltration (dilated lymphatic channels), and large abdominal soft-tissue masses. A lymphangiogram shows dilated, retroperitoneal lymphatic channels with pooling. Over a 10-year follow-up, CT shows substantial regression and clearing of the interstitial and mediastinal abnormalities following progesterone therapy and a lymphangiogram.
Key takeaways
Diffuse pulmonary lymphangiomatosis (DPL) is a rare, benign but progressive lymphatic disorder characterized by proliferation of lymphatic channels in the lungs, mediastinum, and pleura. While historically carrying a poor prognosis, some cases can show remarkable long-term regression or stabilization, possibly mediated by progesterone therapy or the embolizing/sclerosing effect of lipiodol used during lymphangiography.
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