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Embryonal Tumors

  • Chapter
Pediatric CNS Tumors

Part of the book series: Pediatric Oncology ((PEDIATRICO))

Embryonal tumors comprise a large fraction of pediatric brain tumors. Their cell of origin, histo-pathologic classification, and treatment are all areas of controversy. The prognosis for these tumors was at one time exceedingly poor, but therapeutic advances have led to substantial improvement in survival. Nevertheless, current treatment protocols often lead to debilitating and severe late effects.

Historically, all embryonal tumors, regardless of their site of origin in the central nervous system (CNS), were grouped under the umbrella term primitive neuroectodermal tumor (PNET) (Rorke 1983). These tumors were distinguished by a relatively homogeneous histological appearance consisting of poorly cohesive, undifferentiated neuroepithelial cells, often with a high mitotic rate. These small, mon-omorphic, round cells sometimes demonstrate neu-roblastic differentiation. All embryonal tumors were conjectured to arise from a common precursor cell of the subependymal matrix in the CNS. The tendency for these neoplasms to disseminate through cerebro-spinal fluid (CSF) pathways was believed to contribute to their poor outcome. Medulloblastoma, thought by Bailey and Cushing to originate from “medullo-blasts,” was sometimes referred to as infratento-rial PNET. However, the distinct biologic nature of medulloblastoma now has become apparent.

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  1. Department of Neurology, Stanford University, Stanford Advanced Medicine Center, Blake Wilbur Drive, 875, Palo Alto, CA, 94305-5826, USA

    Sonia Partap & Paul Graham Fisher

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  1. Sonia Partap
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  1. Departments of Neurological Surgery and Pediatrics, Division of Pediatric Neurosurgery, University of California San Francisco, 505 Parnassus Avenue, Rm M779, San Francisco, CA, 94143-0112, USA

    Nalin Gupta

  2. Departments of Pediatrics and Neurological Surgery, Division of Hematology and Oncology, University of California San Francisco, 505 Parnassus Avenue, San Francisco, CA, 94143-0106, USA

    Anuradha Banerjee

  3. Departments of Radiation Oncology and Neurological Surgery, Helen Diller Family Comprehensive Cancer Center, University of California San Francisco, 1600 Divisadero Street, San Francisco, CA, 94115-1708, USA

    Daphne Haas-Kogan

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Partap, S., Fisher, P.G. (2010). Embryonal Tumors. In: Gupta, N., Banerjee, A., Haas-Kogan, D. (eds) Pediatric CNS Tumors. Pediatric Oncology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-87979-4_5

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