CNS UPDATE 1: EMBRYONAL TUMORS

Arie Perry, M.D. Director, Neuropathology

Distribution of Childhood Primary Brain and CNS Tumors by Histology and Age (Ages 0-14) (N = 15,398), CBTRUS Statistical Report: NPCR and SEER, 2006-2010.

Ostrom Q T et al. Neuro Oncol 2013;15:ii1-ii56

© The Centers for Disease Control and Prevention. Published by Oxford University Press on behalf of the Society for Neuro-Oncology in cooperation with the Central Brain Tumor Registry 2013.

“WHO’s Next?”

A Colloquium to Guide Next Steps in

Brain Tumor Classification and

Grading

Sponsored by the

International Society of Neuropathology

Made possible through generous support

from the STOPbraintumors

Foundation

Organizers:

David Louis

Pieter Wesseling

Arie Perry

Program Committee:

Peter Burger

David Ellison

Guido Reifenberger Andreas von Deimling

WHO’s Next meeting, Haarlem/St. Bavo Church, May 1st 2014

School of Medicine

EMBRYONAL CNS TUMORS WHO 2007 SCHEME

• Medulloblastoma

– Classic

– Desmoplastic

– Extensively Nodular

– Large cell/Anaplastic

– Medullo with myogenic differentiation

– Medullo with melanotic differentiation

• Pineoblastoma

• CNS PNET

– Neuroblastoma

– Ganglioneuroblastoma

– Ependymoblastoma

– Medulloepithelioma

• Atypical teratoid / Rhabdoid tumor

School of Medicine

MEDULLOBLASTOMA (WHO IV)

• Children/young adults

• Aggressive natural history

• CSF seeding (“icing” and drop mets)

• 5-year survival: 60-80% with therapy

• Radiation may save patient’s life, but is harmful to the developing CNS

• Favorable and unfavorable variants

• Histogenesis: EGL or SEGM of 4th ventricle

School of Medicine

Taylor et al.,

Acta Neuropathol

2012;123:465-472

Medulloblastoma

Integrated diagnosis:

Medulloblastoma, histological subtype and molecular subgroup (e.g., Wnt, SHH, non-WNT/non-SHH*), WHO grade IV

Histological classification:

Classic, anaplastic, large cell, desmoplastic/nodular, or medulloblastoma with extensive nodularity

WHO grade:

IV

Molecular information:

MYC amp, NMYC amp, TP53 status, CTNNB1 status, SMO status, PTCH status, i17q, monosomy 6 (list illustrative and not meant to be exhaustive)

School of Medicine

CLASSIC SUBTYPE

School of Medicine

CLASSIC SUBTYPE

WNT MOL SUBTYPE

β-catenin+

WNT MOL SUBTYPE

(β-catenin-)

School of Medicine

Taylor et al.,

Acta Neuropathol

2012;123:465-472

DESMOPLASTIC SUBTYPE

Reticulin

MIB-1 SYN

GFAP Neu-N

GAB-1

SHH MOL SUBTYPE

GAB-1

SHH MOL SUBTYPE

GAB-1

SHH MOL SUBTYPE

School of Medicine

EXTENSIVELY NODULAR SUBTYPE

Yeh-Nayre et al.,

Case Rep Radiol

2012: 310359