GANGLIOGLIOMA (WHO I) GANGLIOGLIOMA (WHO I)

GANGLIOGLIOMA (WHO I) GANGLIOGLIOMA (WHO I)

NON--GLIAL PEDIATRIC BRAIN
NON
TUMORS
Arie Perry, M.D.
GANGLIOGLIOMA (WHO I)
 Children/young
adults
 Chronic seizure disorder
 Temporal lobe
 Benign/surgically curable
 Anaplastic variant rare: ?definition
– WHO grade III (grade II eliminated in 2007)
 Genetics
inconsistent
GANGLIOGLIOMA (WHO I)
EMBRYONAL/NEURONAL
NEOPLASMS
Ganglio = large mature neuron
Neurocyte = small mature neuron
 Neuroblast = small primitive neuron
 Paraganglio
P
li = autonomic
t
i
neuroendocrine cell
 Esthesio = olfactory neuronal cell
 Pineo = pineal neuronal cell
 Retino = retinal neuronal cell


GANGLIOGLIOMA (WHO I)
PASD
CG
SYN
DESMOPLASTIC INFANTILE
GANGLIOGLIOMA (DIG) (WHO I)
Age <2 years
Massive cystic/solid hemispheric mass
 Dural attachment common
 Small glioneuronal cells in a fibrous
histiocytoma-like background
 PNET-like foci
 Favorable prognosis


CD34
DIG, WHO I
CENTRAL NEUROCYTOMA (WHO II)
Older children/Young adults
Lat. v./3rd v./F. of Monro/S. pellucidum
 Obstructive hydrocephalus
 Globular/calcified
 Usually benign, but may recur (MIB-1>2%)
 DDx: oligodendroglioma, ependymoma
 Genetics inconsistent, but no 1p/19q losses


GFAP
SYN
Neu-N
Reticulin
CENTRAL NEUROCYTOMA, WHO II
SYN
Neu-N
GFAP
MIB-1
EXTRAVENTRICULAR NEUROCYTOMAS
PINEAL PARENCHYMAL TUMORS

Pineocytoma, WHO grade I

PPT, Int. Differentiation, WHO grades II or III
PINEOCYTOMA, WHO GRADE I
– Identical to neurocytomas (pineocytic rosettes)
– No rosettes, mitotic activity, NF+ = gr. II
– High MI (Ki-67) and no NF+ = gr. III

Pineoblastoma, WHO grade IV
PPT, INT. DIFF., WHO GRADE II or III
PINEOBLASTOMA, WHO GRADE IV
GERM CELL TUMORS
GERMINOMA
Children/young adults
Pineal gland (boys)/suprasellar (both sexes)
 Parinaud’s syndrome (upward gaze paralysis)
 Precocious
P
i
puberty
b
 Germinoma radiosensitive
 Teratoma resectable
 Other germ cell elements = worse prognosis


CD117
MEDULLOBLASTOMA/PNET (WHO IV)
EMBRYONAL CNS TUMORS

Medulloblastoma
–
–
–
–
–
Classic
Desmoplastic
Extensively Nodular
L
Large
cell/Anaplastic
ll/A
l ti
Medullo with myogenic
differentiation
– Medullo with melanotic
differentiation


Pineoblastoma
CNS PNET
–
–
–
–

Neuroblastoma
Ganglioneuroblastoma
Ependymoblastoma
Medulloepithelioma
Atypical teratoid /
Rhabdoid tumor
OCT 3/4







Children/young adults
Aggressive natural history
CSF seeding (“icing” and drop mets)
55-year
year survival: 60
60-80%
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