Pediatric Supratentorial Tumors

In Toddlers

Presentan : dr. Hadian Adhipratama & dr. Adhitya Rahadi Y.

Pembimbing : dr. Mirna Sobana, SpBS, M.Kes
Sumber Ilmiah : Youmans & Winn Neurological Surgery 7th edition
Principles and Practice of Pediatric Neurosurgery 3 rd edition
 Introduction
20-30% of cancers in children
2500-3000 new diagnoses/year
2nd most common neoplasm
Most occur before age 10 years
Tumors in infants - usually congenital.
Male/Female = 1.3/1.0
60-70% 5 year survival
 Analysis of a Potential Brain Tumor
Age of the patient
Localization
- Intra-axial or extra-axial
- Compartment
- Crossing of the midline
CT and MR characteristics
- Calcification, fat and cystic
- T1, T2, DWI
- Contrast enhancement
Effect on surrounding structures
- Mass effect, edema
Solitary or multiple
Psuedotumor
 Differentiating with Extra-axial
lesions
CSF cleft sign
Displaced subarachnoid vessels
Cortical gray matter between mass and white matter
Displace and expand subarachnoid space
Broad dural space
Bony reaction
 Location
Supratentorial 25-40%
Astrocytoma, low grade 8-20%
Astrocytoma, high grade 6-12%
Ependymoma 2-5%
Mixed glioma 1-5%
Ganglioglioma 1-5%
Oligodendroglioma 1-2%
PNET 1-2%
Choroid plexus tumor 1-2%
Meningioma 1-2%
Germ Cell Tumors 1-2%
Other 1-3%
 Infratentorial
Location (contd.)
45-60%
Medulloblastoma (PNET) 20-25%
Astrocytoma, low grade 12-18%
Ependymoma 4-8%
Brain stem glioma, high grade 3-9%
Brain stem glioma, low grade 3-6%
Other 2-5%
 Brain Tumors signs and symptoms
Increased intracranial pressure - symptoms
Headache
Nausea/vomiting
Double vision
Head tilt
Decreased alertness
Lethargy/irritability
Poor feeding
Endocrine dysfunction
Unexplained behavior changes
- affect, motivation, energy level
 Brain Tumors Signs/Symptoms
Increased ICP Signs
Papilledema, optic atrophy
Loss of vision
OFC (head circumference) increased
Bulging fontanelles, spreading sutures
Setting sun sign (Parinaud syndrome)
Increased blood pressure, low pulse
- herniation?
Posterior Fossa & Brainstem Tumors
- Clinical Features

Posterior Fossa primary Brainstem primary

Ataxia Extremity weakness
Tremors Cranial nerve signs
Dysarthria double vision
Stiff neck facial weakness
Papilledema swallowing dysfunction
 Hemispheric Tumors
Clinical Features

Hemiparesis
Hemianopia
Aphasia
Seizures
 Desmoplastic Infantile
Ganglioglioma
form of desmoplastic infantile tumours
tend to have good prognosis
vast majority occur in children less than 1 year of age
M:F ratio of approximately 2:1
rapidly increasing head circumference is the most common presentation
Seizure activity is uncommon
The frontal and parietal lobes are the most common sites.
 Desmoplastic Infantile Ganglioglioma
(contd.)
CT:
manifests as an exceptionally large cerebral hemispheric mass composed of
both cystic and solid portions.
solid portion of these large masses is typically slightly hyperattenuating and
typically located along the cortical margin of the mass
these masses usually enhance intensely, and may demonstrate a dural tail
No calcification
MRI:
The solid portions typically have the following signal intensity:
T1 - isointense to brain parenchyma
T2 - isointense to brain parenchyma
T1 C+ (Gd) - intense enhancement; dural tail may be seen
T1WI

T1 + C
T2WI FLAIR
 Choroid Plexus Papilloma
uncommon, benign neuroepithelial intraventricular tumour (WHO grade 1)
account for approximately 1% of all brain tumours, 2-6% of all paediatric brain
tumours
Approximately 85% of all choroid plexus papillomas occur in children under the age
of 5 years.
Most common brain tumors in children under 2 years
Fourth ventricular tumors cause obstructive hydrocephalus with headache & ataxia
CPPs that arise in the foramen of Luschka or CP angle may cause cranial nerve
palsies
The most common location is the lateral ventricle trigone ( atrium ) 50 %
- fourth ventricle 40 %
- third ventricle & cerebellopontine cistern 10 %
The primary neoplasm sheds tumor & seeds the CSF pathways ( drop metastases )
 Choroid Plexus Papilloma (contd.)
CT:
The tumours are usually well-defined lobulated masses
iso- or somewhat hyperdense compared to the adjacent brain. There is associated
hydrocephalus.
They usually homogeneously enhance, demonstrating with an irregular frond-like pattern,
resulting in a cauliflower-like appearance.
Fine, speckled calcification is seen within the tumour in approximately 25% of cases
MRI:
T1 - typically isointense c.f. to adjacent brain. May be somewhat hypointense
T2 - iso to hyperintense, small flow-voids may be seen within the tumour
T1 C+ (Gd) - marked enhancement, tends to be homogenous
MRS - decreased NAA, increased Cho
Angiography: demonstrate intense vascular blush on angiography. Enlarged choroidal
arteries may be seen feeding the tumour, with shunting
 T1 + C
T1WI T2WI

Differential diagnosis:
Choroid plexus carcinoma
Choroid plexus metastases
Medulloblastoma
ATRT
Papillary anaplastic
ependymoma
Central neurocytoma
 Choroid Plexus Carcinoma
malignant neoplasm arising from the choroid plexus (WHO grade III)
significantly poorer prognosis than choroid plexus papilloma (CPP)
10 20 % of all choroid plexus neoplasms
Almost all occur in infants & children 2 4 years of age
Symptoms are due to hydrocephalus & less commonly due to parenchymal invasion
There is an association with Li-Fraumeni syndrome
They almost always arise in the lateral ventricles & infiltrate the adjacent brain
parenchyma
 Choroid Plexus Carcinoma (contd.)
CT:
choroid plexus carcinomas are heterogeneous and typically iso to hyperdense to grey
matter
Calcification may be seen in 20-25% of cases.
Contrast enhanced is usually prominent but heterogeneous with areas of necrosis and
cyst formation evident.
MRI:
T1: iso- to hypointense Differential diagnosis:
T2: iso- to hypointense with hyperintense necrotic areas Choroid Plexus papilloma
Central neurocytoma
T2* GRE: blooming from calcifications/haemorrhage
Choroid plexus meningioma
T1 C+ (Gd): can show marked, heterogeneous enhancement.PNET
The tumours may have CSF seeding GBM
NCCT

T1 + C T2WI

T1WI
 Pineoblastoma
primitive neuroectodermal tumours (PNET) located in the pineal region
most agressive and highest grade tumour among pineal parenchymal tumours
typically found in young children, with both sexes being equally affected
well established association with hereditary retinoblastomas
Patients with bilateral retinoblastoma 5-15% develop midline (suprasellar or pineal)
neuroblastic tumours, referred to as trilateral retinoblastoma.
always associated with obstructive hydrocephalus
highly malignant tumours prone to CSF seeding
 Pineoblastoma (contd.)
CT:
Large poorly defined masses (>4 cm)
Tendency to directly involve adjacent brain structures
The solid component tends to be slightly hyperdense compared to adjacent brain.
Classically, they are described as having peripherally disperse or "exploded"
calcification
MRI:
T1- isointense to hypointense to adjacent brain
T2 - isointense to adjacent brain; areas of cyst formation or necrosis may be present
T1 C+ (Gd) - vivid heterogenous enhancement
DWI - restricted diffusion due to dense cellular packing
 Differential diagnosis:
Pineocytoma
Pineal papillary tumor
Germinoma
Astrocytoma of pineal
gland
Pineal cyst

T1WI T1 + C

T2WI
 DNET
Dysembryoplastic neuroepithelial tumor- benign, focal, intracortical mass
superimposed on background of cortical dysplasia
vast majority are centered in cortical grey matter, arise from secondary germinal
layers
C/F- longstanding partial complex seizures
Age- < 20 years
Site - temporal lobe ( amygdala/ hippocampus) 60%, frontal lobe 30%, caudate
nucleus, cerebellum and pons
 DNET (contd.)
DNETs are typically predominantly cortical and well circumscribed tumours.
CT:
if cortical may scallop the inner table of the skull vault (44-60%), but no erosion
the cranial fossa can be minimally enlarged at times
calcification in ~30% (more common histologically)
low density; no enhancement
MRI:
T1 - generally hypointense c.f adjacent brain
T1 C+ (Gd) - may show enhancement in ~20-30% of cases; enhancement may be
heterogeneous or a mural nodule; focal punctate or ring enhancement- 20%
T2 - generally high signal; high signal 'bubbly appearance
FLAIR- hypo/ isointense with bright rim; no peritumoral edema
DWI- lacks restricted diffusion
T1WI DWI

T2WI
Differential diagnosis:
Ganglioglioma
Pleomorphic xanthoastrocytoma
Pilocytic astrocytoma
T1 + C Desmoplastic infantile FLAI
ganglioglioma R
Oligodendroglioma
Choroid fissural cyst
HSE and limbic encephalitis
Mesial temporal sclerosis
 Intracranial Teratoma
account for the largest proportion of fetal intracranial neoplasms
divided into two broad categories:
- intra- and extra-axial
Intra-axial teratomas present antenatally due to increasing head circumference; tend
to occur supratentorially
Extra axial teratomas usually present in childhood or early adulthood; commonly
arise in the pineal or suprasellar regions; obstructive hydrocephalus, Parinaud
syndrome
 Intracranial Teratoma (contd.)
CT:
Intracranial teratomas are often seen as large lesions at presentation
tumours typically demonstrating a mixture of tissue densities and signal intensity
demonstrate at least some fat and some calcification, which is usually solid / "clump-
like"
They usually have cystic and solid components, contributing to an irregular outline.
Solid components demonstrate variable enhancement
MRI:
T1 - hyperintense components due to fat and proteinaceous/lipid rich fluid;
intermediate components of soft tissue; hypointense components due to calcification
and blood products
T1 C+ (Gd) - solid soft tissue components show enhancement
T2 - again mixed signal from differing components
T1WI

FLAIR T1 + C

Differential diagnosis:
sPNET
ATRT
T2WI Choroid plexus carcinoma
Intracranial lipoma
Intracranial dermoid
Craniopharyngioma
 Craniopharyngioma
Arise from squamous epithelial rests along the involuted hypophyseal- Rathkes duct
3% of intracranial neoplasms.
15% of supratentorial and 50% suprasellar tumors in children
M>F
Bimodal age distribution- 1st- 5-15 yrs and 2nd peak- 4th-6th decade
Types- Adamantinomatous and papillary
 Craniopharyngioma (contd.)
Radiography: Lateral skull- Amorphous sellar & suprasellar Ca++, sellar
enlargement, dorsum sellae & clinoid erosion
NECT:
- Admantinomatous : 90% mixed (solid & cystic)
90% calcify
- Papillary : Often solid , isodense, rarely calcifies
CECT: 90% enhance (solid + capsule)
CTA: Displacement & encasement of circle of Willis
 Craniopharyngioma (contd.)
MRI:
Multilobulated, multicystic suprasellar masses.
T1WI - cystics areas may be isointense or have high or low SI as compared to brain
T2WI - both solid and cystic components tend to be hyperintense but cystic
component tend to have higher SI. Solid part has granular appearance on pre-
contrast T1WI and may show heterogenecity as a result of small cysts and
calcification.
Post-contrast- solid part enhance heterogenously. Thin walls of cysts nearly
always enhance
Papillary type - entirely solid. Heterogenous appearance and enhancement.
MRS - to differentiate from suprasellar astrocytoma which shows large Choline
peak and reduced but present NAA peak
17 year old
male with
headache
and impaired
vision
 Atypical Teratoid Rhabdoid Tumor
uncommon malignant intracranial tumors, representing only 1.3% of primary CNS
tumors in the pediatric population (WHO Grade IV tumour)
vast majority of cases occurs in young children less than two years of age
can occur anywhere in the central nervous system (CNS) including the spinal cord.
infratentorial: ~50%
- cerebellum (most common), brainstem
supratentorial
- cerebral hemispheres, pineal gland region, septum pellucidum and hypothalamus
 ATRT (contd.)
CT : Differential diagnosis for ATRT:
often isodense to gray matter Supratentorial PNET
may demonstrate heterogeneous enhancement Intracranial teratoma
Medulloblastoma
calcification is common Choroid plexus carcinoma
may show associated obstructive hydrocephalus Malignant glioma

MRI:
Can show necrosis, multiple foci of cyst formation and sometimes haemorrhage:
T1: iso- to slightly hyperintense to grey matter (haemorrhagic areas can be more
hyperintense)
T2: generally hyperintense (haemorrhagic areas can be hypointense)
T1 C+ (Gd): heterogeneous enhancement
MRS
- Cho: elevated
- NAA: decreased
Pre-contrast Post-contrast
T1WI

T2WI T1 + C

DWI
Terima Kasih