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PNET/Medulloblastoma: Toward A Tailored Treatment Paradigm. Anna J. Janss, MD, PhD. Overview. Definitions Evolution of Therapy Success Shortcoming Biology Interface of Treatment/Biology The future is at hand!. PNET and medulloblastoma: Definition of Terms.
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PNET/Medulloblastoma:Toward A Tailored Treatment Paradigm Anna J. Janss, MD, PhD
Overview • Definitions • Evolution of Therapy • Success • Shortcoming • Biology • Interface of Treatment/Biology • The future is at hand!
PNET and medulloblastoma: Definition of Terms • Bailey , Cushing (1925) “medulloblastoma cerebelli” • Hart, Earl (1973) coined “Primitive Neuroectodermal Tumor, PNET” • Rorke (1983) proposed WHO standard - PNETs “CNS tumors with small, blue cell pathology” regardless of location
Primitive Neuroectodermal Tumor PNETs = PNETs =
Differentiation in PNETs PNETs may, or may not demonstrate differentiation by expression of lineage specific proteins • neuronal • retinal • glial • melanotic Melanin Retinal S protein NFP Melanin