The authors report a case of a primary intracranial yolk sac tumor which is a quite rare eventuality. The patient, an 18 months old girl was referred for severe intracranial hypertension. Neurological examination revealed a right hemiparesis, a left abducens nerve palsy and a bilateral papilledema. The serum and C.S.F. levels of alpha fetoprotein were at 2100 Ugr/l and 2500 Ugr/l, respectively. The computerized tomography showed a mass with a low density area in the left temporoparietal lobe and the carotid angiogram a highly hyper-vascular tumor. The child underwent a left temporo-parietal craniotomy and the friable vascular tumor was totally removed. A radiotherapy was associated to the surgical treatment. Histologically, glomerular like structures (Shiller Duval body), intra and extra cellular hyaline globules PAS positive were frequently seen. The tumor marker was demonstrated immunohistochemically.
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