Fig. 2

Treatment response in patient with KLA (Patient #1). a At the initial presentation of respiratory distress, coronal enhanced CT scan revealed massive pleural effusion almost entirely filling the left hemithorax with complete atelectasis of the left lung and mediastinal shift to the right. The lesions involved the vertebral bodies of T8-T12 and multiple ribs (b). Horizontal T2-weighted MRI revealed pleural thickening and effusion, heterogeneously increased T2 signal intensity along the right tracheobronchial tree, and infiltrative, hyperintense, posterior mediastinal soft tissue masses (c). The patient was treated with vincristine for 12 months. However, the response was suboptimal. The lesion became progressively static in size (d). Treatment with sirolimus and prednisolone was then initiated. After 8 weeks of combination treatment with sirolimus and prednisolone, followed by 10 months of sirolimus monotherapy, the lesions became more prominent (e)