CTICasesDiffuse Pulmonary Lymphangiomatosis with Skeletal Involvement
Imaging findings
Chest and abdominal CT in a 17-year-old male show marked, diffuse peribronchial wall thickening and cuffing extending from the hila, diffuse mediastinal soft-tissue infiltration, and bilateral pleural thickening with extrapleural fat proliferation. Skeletal windows demonstrate diffuse, bilateral expansion and sclerosis of the ribs and vertebral bodies, without focal lytic destruction.
Key takeaways
Diffuse pulmonary lymphangiomatosis (DPL) can rarely be associated with skeletal involvement (generalized lymphangiomatosis), where abnormal lymphatic proliferation involves the bone marrow. This causes diffuse marrow expansion and sclerosis of the ribs and spine, mimicking bone dysplasia or Gorham-Stout disease (which is characteristically osteolytic). Interferon alpha-2b is an anti-angiogenic therapeutic option.
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