Fig. 1.

Clinical appearance and examinations of the patient with KLA (P1). a Cutaneous manifestation of left chest wall. b Chest radiography shows pleural effusion and mediastinal enlargement. c, d Chest contrast-enhanced MRI demonstrates diffuse thickening of the left chest wall, pleural effusion in the left lung, and retroperitoneal soft-tissue mass. e, f Specimen shows proliferation of thin-walled, anastomosing lymphatic vessels lined by a single layer of endothelial cells with a focus of spindle cells (bar 100 μm, H&E). Endothelial cells were identified as lymphatics using D2–40 (bar 100 μm)