Brain Tumors of Childhood

Andrew D. Fine, M. D.

P di i Brain Pediatric B i Tumors T

Ad l and Adults d Children Child

Adults cerebellum

Children
medulloblastoma astrocytoma

Met astrocytoma medulloblastoma

4th ventricle
ependymoma choroid papilloma meningioma i i

4th ventricle
ependymoma choroid papilloma

Cli i l P Clinical Presentation i

Infants

increased head circumference nausea and vomiting

Children

as above and h d h headaches focal deficits vermian syndrome / hemicerebellar syndrome head tilt

E id i l Epidemiology
15 15-20% of all primary brain tumors 2nd most common pediatric tumor overall supratentorial = infratentorial

0-2 yrs 3-10 yrs over 10 yrs

Posterior Fossa Tumors

M d ll bl Medulloblastoma

M d ll bl Medulloblastoma
PNET of the posterior fossa 15 15-20% of all childhood brain tumors 1/3 of childhood posterior fossa tumors peak incidence 3 3-8 yrs slight male predominance chromosome 17q abnormality in 1/3 shunt related metastasis adjuvent therepy indicated

M d ll bl Medulloblastoma
Clinical features

midline midline syndrome syndrome cerebellar mutism - post post-op and transient in 13%
duration to diagnosis 66-7 weeks Atypical presentations

meningitis
medulloblastoma cells confused with lymphocytes

posterior fossa hemorrhage may go to bone - usually lytic

M d ll bl Medulloblastoma
Pathology

Blue tumor

rosettes
Homer Wright rosetterosette- 20% of cases perivascular pseudorosette

Medulloblastoma M d ll bl - Risk Ri k Factors F

Collins Law
(Age at diagnosis + 9 months)

extent of resection less significant significant-80% resection dissemination dissemination- most significant factor
2020-30% overall, overall 50% in infants at time of surgery pre pre-op MRI and LP

age ageg - older is better histology

desmoplastic +/+/ identifiable id tifi bl cell ll lines(glial, li ( li l neuronal, l ependymal) d l)

M d ll bl Medulloblastoma
CCSG data d surgery and craniospinal irradiation
5 yr disease free survival 5050-70% poor risk group group- 36% 5yr survival good risk groupgroup- 60 60-70% 5yr survival

M d ll bl Medulloblastoma
Cognitive effects of RT are ageage-dependant

Children older than 10 appear to suffer no measurable decline

Medulloblastoma

M d ll bl Medulloblastoma

M d ll bl Medulloblastoma
Radiographs

well delineated mass arises from vermis 75% fills 4th ventricle calcification 15% hydrocephalus 95% enhances subarrachnoid seeding

Cerebellar Astrocytoma

Cerebellar Astrocytoma
Midline solid vermian mass

22% of cases
lateral cystic mass

ddx ddx- hemangioblastoma

Usually does not enhance

may in areas
mural nodule areas of calcification

C b ll Astrocytoma Cerebellar A
Clinical presentation

Headache associated with N/V Treated for GI disease for a long time Papilledema in 90% Ataxia in 95% 6th nerve is the most common cranial neuropathy hydrocephalus common at presentation

C b ll Astrocytoma Cerebellar A
Epidemiology

7070 -80% are found in children 1/3 of pediatric posterior fossa brain tumors no sex predilection typical patient is 5 5-15 yrs old, may occur at any age

C b ll Astrocytoma Cerebellar A
Pathology cysts contain yellow proteinaceous fluid Brain stem invasion in 8% microscopic

biphasic p p pattern
pilocytic loose stellate astrocytes

Rosenthal bodies
cytoplasmic aggregates of glial filaments

Cerebellar Astrocytoma

Outcome
20 yr survival > 80% is reported low recurrence rate after total excision 2.5% in gross total excision 35% in subtotal removal Radiation
initial radiation did not affect the rate of recurrence radiation is used for malignant g histology gy or recurrence where surgery not feasible

Chemotherapy
reserved for malignancy not responsive to radiation

brain stem invasion and malignant histology indicate less favorable prognosis

E Ependymoma d

E Ependymoma d
10% of all childhood tumors In children, 70% occur in the posterior fossa present with headaches, vomiting, diplopia, and g gait ataxia arise from floor of 4th ventricle and extend through foramina subarrachnoid seeding g in 3%

E Ependymoma d
Pathology

true rosettes perivascular rosettes

E Ependymoma d
Treatment

surgical resection is the primary treatment radiation for residual tumor if old enough
Prognosis

young age at t di diagnosis i brain stem involvement incomplete resection

B i Stem Brain S Gli Glioma

B i Stem Brain S Gli Glioma

4 categories:

diffuse, focal, exophytic, and cervicomedullary diffuse diffuse- malignant astrocytoma

6060-70% of brain stem tumors rapid progression of gait dysfunction and multiple cranial nerve nuclei involved hypointense on CT and T1 MRI, variable enhancement surgery of no benefit on survival

Brain Stem Glioma

Focal tumors

<2 <2.5 5 cm intrinsic may erupt at any surface history spans mo.mo -yrs mo.
N/V, poor wt gain, diplopia, hoarseness tectal glioma - subgroup

macrocephaly hydrocephalus

B i Stem Brain S Gli Glioma

Exophytic tumors

arise from subependymal glial tissue and fungate into the 4th ventricle clinical Hx long
may obstruct CSF ataxia and nystagmus common intractable vomiting homogeneous enhancement

Brain Stem Glioma

Cervicomedullary Tumors

Clinical
long standing neck pain, Lhermittes sign, torticollis slowly l l evolving l i weakness k of f the h arms and dl legs cranial nerves often not involved

variable enhancement

Teratoma Teratoma

Teratoma Teratoma
Infancy I f

arise in the midline in the region of the 3rd ventricle posterior fossa teratomas in the 4th ventricle Present with hydrocephalus

Teratoma Teratoma
Pathology

contain more than one germ layer well differentiated or immature cysts and calcification occur with differentiation

S Supratentorial i lT Tumors
Tumors of the cerebral hemispheres Sellar and suprasellar tumors Pineal region masses Extraparenchymal tumors

Astrocytomas Astrocytomas

Astrocytomas Astrocytomas
1/3 of childhood hemisphere tumors no sex predilection peak incidence 8 8-12 yrs
low grade astrocytoma is most common

60 60-70% long term survival

JPA account for 1010-20% 20%--- 72% survival anaplastic and GBM 2020-30%

20 20-40% and 5 5-15% 5 yr survival respectively leptomeningeal spread in 10%

Astrocytoma Astrocytoma
Tumor cyst

seen in 40 40-50% lined with neoplastic cells in 70%

CCSG data suggests greatest benefit when

>90% resection

Astrocytomas Astrocytomas
Cytoreduction

Laws Laws-Gross total resection and radical subtotal resection VS. biopsy and partial resection CCSG CCSG- radical resection of malignant gliomas ( over 90% resection )
survival advantage independent of location and adjuvant therapy

Astrocytoma Astrocytoma

Ch Chemotherapy h
Malignant astrocytoma Improved survival at 5 yrs vs radiation alone (13 to 43%) nitrosourea-based regimen nitrosourea

Irradiation low grade glioma

question of efficacy

Malignant glioma
5400 5400-6000 cGy offer longer survival than 5000 cGy focal field more common than whole brain

E Ependymomas d

2020 -40% of childhood ependymomas

m>f peak k incidence i id 1-5 yrs old, 1ld half h lf < 2yrs 2

most commonly associated with the frontal horn, but uncommonly intraventricular identical histology as those of the posterior fossa overall 5 yr disease free survival is 4040-60%
if gross total resection, then 85%

risk of recurrence: subtotal resection, age <3, leptomeningeal dissemination

Gangliogliomas
Mixed tumor 4-8% of pediatric brain tumors medial temporal lobe male children Present with seizures well demarcated, cystic, without edema total resection

10 yr disease disease-free survival 75 75-90%

PNET
< 5% of supratentorial p tumors in children Radical surgical resection
gross total improved survival

CSF dissemination common j treatment Adjuvant

craniospinal irradiation chemotherapy

survival
<30% at 5 yrs

Oli d d li Oligodendrogliomas
2-3% of hemispheric pediatric tumors 30% of hemispheric gliomas have a mixed population containing oligodendroglioma peak incidence in childhood 66-12 y yrs p frontal lobes neuraxis dissemination is rare fried egg , calcification, microhemorrhages 75-85% for pure oligo 5 yr survival 75 anaplastic tumors, add PCV chemo

DNET
Benign present as partial complex seizures in

childhood or young adults 60% temporal lobe, lobe 30% frontal may erode the inner table of the skull associated with cortical dysplasia

Metastases Metastases
2-8% of pediatric brain tumors 6% of solid pediatric brain tumors will have a cerebral met >50% will be multiple p

Frequent primary neoplasms

neuroblastoma rhabdomyosarcoma Wilms tumor