PMC7198717_01
MRI. Axial (a,b) and coronal (d) T2w and axial GdT1 (c) images show two skull lesions located in the right parietal and temporal region, with intense contrast-enhancement. Pituitary gland was normal (e, white arrow) CT axial images (e,f) show infiltrative aspect of the lesion.
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F1
Brain MRI showed a small lesion in the right occipital region, without significant mass effect, and another lesion in the right temporal region; the pituitary gland appeared normal (Figure 1).
CT. Coronal (a, bone window) and 3D (b) reconstruction show a voluminous tumor involving the left ischio-and ileo-public branches with extensive bone destruction, speculated periosteal reaction, and partially mineralized soft tissue mass.
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F2
A bone scintigraphy confirmed multiple secondary bone localizations (vault and skull, skull-base, spine, several ribs, sternum, left scapula, pelvis, both femurs, and the right tibia) (Figure 2).
The histological picture is a monomorphic proliferation of small round cells with scanty cytoplasm containing glycogen (E&E 10X). The cells shows positive stain for CD99 (immunoperoxidase stain 10X).
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F3
A biopsy of the mass confirmed Ewing's Sarcoma with EWS/FLI-1 gene fusion diagnosis (Figure 3). The brain CT scan showed a progressive disease correlated by abnormal ACTH levels (50.9 pg/ml; Figure 3).