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Gliosarcoma, a variant of glioblastoma, is a rare and aggressive tumor of the central nervous system (CNS) composed of glial and sarcomatous tissues. Up to now, there are only 2 reported cases of gliosarcoma of the optical pathway. We report a case from March 2018 of a 53-year-old male patient presented with 6 months’ of right fronto-orbital pulsatile headache, behavior changes, and visual loss. The MRI study showed an expansile optic pathway lesion involving the chiasm and right optic nerve. The diagnosis of gliosarcoma was obtained by open brain biopsy and immunohistochemical analysis. Although gliosarcoma is rare, it should be considered a differential diagnosis even in optic pathway tumors in older patients. The experience of the neuropathologist with a trained eye can be the differential in the accurate diagnostic process. Optic pathway, Gliosarcoma, Glioblastoma, Magnetic resonance imaging

Spinale Dysrhaphien sind angeborene Fehlbildungen des Rückenmarks, die durch eine Störung in der komplexen Kaskade embryologischer Ereignisse bei der Entwicklung der Wirbelsäule bedingt sind. Die Kenntnis der normalen embryologischen Entwicklung des Rückenmarks ist entscheidend für das Verständnis der Pathogenese, der neuroradiologischen Szenarien und der klinisch-radiologischen Klassifizierung angeborener Fehlbildungen des Rückenmarks.