Exceptional localization of Primary Ewing's sarcoma in the greater wing of the sphenoid bone
O.Hmamouche*, M.Hammoud, F.Lakhdar, M.Benzagmout, K.Chakour, MF.Chaoui
Department of Neurosurgery, Hassan II University Hospital of Fez, Morocco
*Oualid Mohammed Hmamouche, Department of Neurosurgery, Hassan II University Hospital of Fez, Morocco, University Sidi Mohammed Ben Abdellah, Fez, Morocco
Figure 1: Cerebral CT scan showing an osteolytic tumoral process centered on the large wing of the right sphenoid invading the orbital apex
Figure 2: MRI showing the poorly limited expansive tumoral process centered on the right orbital apex described as T2 heterosignal, T1 hyposignal, and heterogeneously enhanced after injection of gadolinium.
A stereotactic biopsy of the tumor was performed objectifying a tumoral proliferation arranged in diffused sheet with areas of necrosis. It is made up of small rounded cells, with fine chromatin, and sparse basophilic cytoplasm. Figures of mitosis are noted. On immunohistochemical study, tumor cells express CD99 (intense and diffuse membrane labeling) and synaptophysin. They do not express the neurofilament, CD45 (lymphoid marker), desmin (muscle marker), or GFAP (glial marker). Based on these findings, the diagnosis of Ewing’s sarcoma was made.
The tumor was judged inoperable because its anatomical location and its relationship with the vasculo-nervous elements.
Chemotherapy and radiotherapy were started and gave excellent results. The patient underwent a CT-scan after three months and the results were very encouraging.
Figure 3: Cerebral CT scan objectifying a clear regression in size of the tumor
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