Central Nervous System Xanthoma Disseminatum: Response to 2CdA in an Adolescent

Figure 1

Disease status prior to intervention with 2CdA. (a) Brain MRI: multiple focal lesions are seen at the subcortical white matter and brainstem (yellow arrows), cerebellum (not shown), and left mesial temporal lobe/amygdala () on T2 FLAIR imaging. Enhancing soft tissue is also seen at the pituitary stalk extending into the sella (big red arrow). Additional enhancing nodules (small red arrows) are seen in the brain. Corresponding abnormal FDG uptake (teal arrow) is also demonstrated from a brain image in the whole-body PET-MRI. (b) Whole-body PET MRI: multiple foci of abnormal FDG activity are seen in the neck, including the right thyroid (long teal arrow) and in the mediastinum along the thymic tissues (double teal arrows). Diffuse mucosal thickening of the gastric wall () and gallbladder wall (arrowhead) with FDG-avidity also suggested possible XD involvement and/or inflammation. Enlarged and FDG avid portocaval lymph node (yellow arrow) and involvement of the vertebral body (red arrow) were also present.

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