CTICasesCongenital Lymphangiomatosis
Imaging findings
CT of the chest shows a large, infiltrative, soft-tissue mediastinal mass that encases major vessels and airways without causing hemodynamically significant stenosis or airway pinch. There is pronounced thickening of the peribronchovascular interstitium with prominent interlobular septal thickening, mimicking a severe lymphatic congestion or interstitial process. There are no associated bony lesions, pleural effusions, pleural thickening, or splenic lesions.
Key takeaways
Congenital lymphangiomatosis is a rare, benign, but progressive infiltrative disease of the lymphatic system characterized by diffuse proliferation of lymphatic vessels, commonly involving the mediastinum, lungs, and bone. Unlike pulmonary lymphangiectasia (which is characterized by ectasia of normally situated lymphatics and is often fatal in infancy), lymphangiomatosis presents as diffuse infiltrative masses and septal thickening that can progress slowly into early adulthood. Biopsies typically reveal benign lymphatic spaces and normal lymphoid tissue, with treatment options remaining highly limited and prognosis variable.
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