Kaposiform lymphangiomatosis is an uncommon generalized lymphatic anomaly with
distinctive clinical, radiologic, histopathologic, and molecular findings. Herein, we document
the pathology in 43 patients evaluated by the Boston Children's Hospital Vascular
Anomalies Center from 1999 to 2020. The most frequent presentations were respiratory
difficulty, hemostatic abnormalities, and a soft tissue mass. Imaging commonly revealed
involvement of some combination of mediastinal, pulmonary, pleural, and pericardial …