PMC6338001_01
Anteroposterior and lateral radiograph of the right leg showing lytic lesion with large soft-tissue component and hair-on-end periosteal reaction in mid-diaphysis of tibia
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F1
A 13-year-old female with a history of below the knee amputation 3 months prior for Ewing's sarcoma [Figure 1], presented to her primary care physician, with left hemicranial headache and rapidly progressive diminution of vision in the left eye along with drooping of eyelid, binocular diplopia, and difficulty in moving the eye for 2 weeks.
Left eye. (a) Total ptosis, (b) total ophthalmoplegia
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F2
In the left eye, there was no light perception, and there was axial proptosis (2 mm), total ptosis, total ophthalmoplegia [Figure 2a and b], total afferent pupillary defect with total optic atrophy, and loss of corneal reflex.
(a) Magnetic resonance imaging brain showing large mass based on sellar/suprasellar region extending to the left orbital apex, ethmoidal air cells, and cavernous sinus. (b) Chest X-ray showing well-defined opacities in bilateral mid-zones. (c) Computed tomography chest showing irregular soft-tissue lesions in lungs and mediastinum. (d) Computed tomography pelvis showing lytic lesion in the left femoral head
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F3
Magnetic resonance imaging brain at this time [Figure 3a] demonstrated an extensive hyperintense mass lesion centered over sella, engulfing the pituitary gland, compressing the optic chiasma and completely occupying the left cavernous sinus, attenuating the left internal carotid artery (ICA), and abutting the right cavernous sinus and right ICA. Whole-body imaging revealed multifocal irregular heterogeneously enhancing soft-tissue lesions of variable size suggestive of lung and mediastinal metastases [Figure 3b and c]. A few enhancing lymph nodes in both axillae and in the mesentery and a focal lytic defect of the left femoral head was also found [Figure 3d].
(a) H and E-stained section of nasopharyngeal punch biopsy specimen showing nests of small round blue cells, (b) Strong CD99 immunoreactivity
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F4
Strong CD99 positivity, borderline synaptophysin immunoreactivity, and chromogranin nonreactivity supported the diagnosis of Ewing's Sarcoma [Figure 4a and b].