Embryonal brain tumor with unknown primary lesion and massive cerebrospinal fluid dissemination: A case report

Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia
Hirotomo TanakaEiji Kohmura

Abstract

The 2007 World Health Organization Classification of Tumors of the Central Nervous System (CNS) categorized embryonal tumors of the CNS into three classes: medulloblastoma, CNS primitive neuroectodermal tumor, and atypical teratoid/rhabdoid tumor. Due to the lack of specific histological features, it was sometimes difficult to accurately differentiate CNS embryonal tumors pathologically. Here, we report a case of a young man, who presented with headache. Gadolinium-enhanced magnetic resonance imaging demonstrated massive lesions in the cerebrospinal fluid space, which strongly suggested leptomeningeal dissemination of a brain tumor. The histology showed the tumor comprised densely packed, small cells with scant cytoplasm. Immunoreactivities were positive for synaptophysin and chromogranin A, and negative for glial fibrillary acidic protein, S-100, EMA, and CD20. Because the tumors were located in multiple sites and most of them were within the cerebrospinal fluid space, the primary lesion could not be determined. We diagnosed this case as 'CNS primitive neuroectodermal tumor' by the patient age and predominantly supratentorial distribution of the lesions. After the induction therapy, WHO published its updated classification in ...Continue Reading

Citations

Sep 15, 2018·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Sofiene BoualiHafedh Jemel
Feb 9, 2021·Clinical Neurology and Neurosurgery·N KumarC K Ahuja

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