PNET/Medulloblastoma: Toward A Tailored Treatment Paradigm

1
PNET/MedulloblastomaToward A Tailored Treatment
Paradigm
Anna J. Janss, MD, PhD
2
Overview

• Definitions
• Evolution of Therapy
• Success
• Shortcoming
• Biology
• Interface of Treatment/Biology
• The future is at hand!

3
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
4
Primitive Neuroectodermal Tumor
PNETs
PNETs
5
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