Pediatric Supratentorial Tumors / Tumores Supratentoriais Pediátrico

Erion Junior de Andrade
R3 NEUROCIRUGIA
HC UNICAMP
PEDIATRIC
SUPRATENTORIAL TUMORS

PEDIATRIC SUPRETENTORIAL
TUMORS

INTRODUCTION
• 15-20% of all CNS tumors.
• They are the most common solid tumors in children
between 0 to 14 years of age, and their incidence is
highest during the first year of life.
• These tumors account for the most cancer- related
deaths in the 0 to 14 age group according to the
Central Brain Tumor Registry of the United States
(CBTRUS).

• > are gliomas, with roughly half of them consisting
of pilocytic astrocytomas or other low-grade
neoplasms, followed by embryonal tumors.
• Approximately 21% of all gliomas have a high-grade
histology and are associated with an aggressive
clinical behavior and a dismal prognosis.
• When brain stem tumors are excluded, high-grade
gliomas are most commonly supratentorial,
occurring in the cerebral hemispheres, followed by
central gray matter structures.
INTRODUCTION

• 15% of all CNS neoplasms are embryonal tumors, a
heterogeneous group of lesions that arise from
undifferentiated small round cells, tend to occur in
small children, and are associated with a poor
prognosis and tendency to disseminate throughout
the neuraxis.
• With the exception of medulloblastomas,
embryonal tumors are predominantly supraten-
torial.
INTRODUCTION

• Finally, although neuronal and mixed neuronal-glial
tumors are not as common, accounting for less than
5% of all neoplasms, they may nonetheless lead to
significant morbidity in many patients due to
intractable seizures.
• Many of these lesions share similar clinical and
imaging presentations making their prospective
diagnosis challenging. T
INTRODUCTION

Surgical GTR is the most important prognostic
factor in most of these tumors.
INTRODUCTION

Clinical Presentation
In children:
• Seizures – often first symptom
• Neurological Deficit
• Elevated ICD: headache, vomiting, drowsiness,
papilledema.
In infants:
• Developmental delay and failure to thrive
• Elevated ICP: vomiting, buldging fontaneles, sunset
sign.
• Macrocephaly

When admit the patient to the PICU?
• Signs of elevated ICP
• Moderate to severe hydrocephalus
• Intratumoral Hemorrhage
• First Seizure
• GCS < 14

1) GLIAL TUMORS
• World Health Organization (WHO) grade 1 and 2
gliomas roughly account for 60% of all gliomas in
children.

1.A) PILOCYTIC ASTROCYTOMA
The adjective pilocytic refers to the presence of bipolar astrocytes with long
extensions, which form Rosenthal fibers. This type of cell is not very
prominent in the normal CNS, but its existence is demonstrated by areas of
long duration gliosis associated with chronic lesions such as
craniopharyngiomas and pineal cysts.

• 1/3 all gliomas in children from 0 to 14 years of age and
constitute the most common primary brain tumor in this
population.
• Their incidence is relatively evenly distributed across this age
group after the first year of life.
• They are histologically benign (WHO grade I) and
demonstrate slow growth over time.
• Pilocytic astrocytomas have an excellent prognosis, with
survival rates as high as 95% at 10 years.

• They most commonly occur in the cerebellar hemispheres (about two-
thirds of lesions in pediatric patients), followed by optic chiasm and
nerves and hypothalamus, but they can rarely develop in the cerebral
hemispheres (particularly in older children and adults, accounting for half
of all tumors in the latter group).6,7
• Most pilocytic astrocytomas are sporadic, but there is a higher incidence
in neurofibromatosis type 1, where they occur in up to 20% of patients.
• Notably, approximately one-third of patients with an optic pathway glioma
(the majority of which are pilocytic) have neurofibromatosis type 1.

• Most pilocytic astrocytomas harbor a BRAF-KIAA1549 fusion
gene mutation, which may be associated with improved
clinical outcomes.
• Classical association of two histological patterns (biphasic
pattern). These are:

1) the pilocytic pattern, which is densely fibrillar, formed by bipolar astrocytes, with
long extensions and frequent Rosenthal fibers;

b) the protoplasmic pattern with microcystic degeneration. In this, the
astrocytes are starched or multipolar, but with short extensions, loosely
arranged, leaving between them spaces (microcysts) filled with slightly
basophilic amorphous material produced by the neoplastic cells.

IMAGE:
• A prior study has shown that approximately 36% of all
cerebral astrocytomas present with cystic changes
• Pilocytic astrocytomas may also appear as solid enhancing
masses.
• An additional characteristic feature is the lack of significant
vasogenic edema in the surrounding parenchyma. When
edema does occur, it tends to be limited in relation to the
size of the tumor.

Treatment
GTR should be the goal when it can be achieved with
an acceptable functional outcome.
Postoperative MRI should be obtained in the first 24
hours. Be aware that systematic and substantial
overestimation of residual nonenhancing volume on
MRI within 48 hours of resection compared with
months postoperatively, in particular for FLAIR imaging,
has been observed.t9

Adjuvant Therapy
There is no role fur adjuvant chemo- or radiotherapy if GTR was
achieved in supratentorial hemispheric LGGs.
Radiotherapy and particularly chemotherapy may be indicated
for unresectable or partially resected tumors. Stereotactic
radiotherapy or intensity-modulated radiotherapy can be
applied; both provide local control for children with small,
localized, low-grade glial tumors.

Outcome
According to the data of the Children's Oncology Group, the
overall 5-year progression-free survival (PFS) is 78% and 8-year
PFS is 75% in hemispheric tumors. If GTR is achieved, 5-year PFS
is 94%.
The 5- and 8-year overall survival (OS) rate is 96%.
Follow-Up
Regular follow-up with sequential MRis every 6 months for the
first 2 years, and yearly thereafter.

1.B) DIFFUSE ASTROCYTOMA
• Diffuse astrocytomas are low-grade tumors (WHO grade II)
that are several times less common in children than pilocytic
astrocytomas.
• They can occur anywhere in the CNS, but one-third arise in
the frontal or parietal lobes, which represent the most
common location.
• Interestingly, while in adults most diffuse low-grade
astrocytomas eventually undergo anaplastic transformation,
progression to a higher-grade tumor is a rare event in
children and accounts for only about 10% of cases.

• High-grade gliomas are significantly less common in children
than in adults, yet they constitute 11% of all CNS neoplasms
in the pediatric population, with an estimated incidence of
0.59 per 100,000 person–years.
• Supratentorial high-grade gliomas comprise one-third of all
pediatric high-grade gliomas and occur most commonly in
adolescents.
• They may be related to prior radiation exposure or occur in
the setting of rare syndromes such as Li Fraumeni.
• Notably, evidence shows that pediatric high-grade gliomas are
genetically and molecularly distinct from their adult
counterparts.24
1.C) HIGH GRADE GLIOMA

• Glioblastomas are rare in children, in whom they constitute
about 3% of primary brain tumors.
• Survival is poor but better than that of adults
1.D) GBM

1.D) GBM
Treatment
• GTR should be achieved since the extent of tumor resection is also the
strongest predictor of survival in pediatric glioblastoma.
• GTR is significantly associated with OS in pediatric patients with glioblastoma
excluding tumors located in the brain- stem.
• 5-AIA has an off-label use for recurrent HGG is reported to be useful and
appears to be safe.
Chemotherapy
Chemotherapeutic agents such as temozolomide, bevacizumab, dsplatin.
etoposide, vincristine, and ifosfamide are used, but there is no international
standard protocol for children.

1.D) GBM
Radiation
• Radiation therapy is the standard ofcare after surgical resection for
children older than 3 years. Treatment of children with
glioblastoma with con- formal radiation, temozolomide, and
bevacizumab as adjuncts to surgical resection .

1.D) GBM
Outcome
• Glioblastoma survival is somewhat higher for children than adults;
in children, the 5-year survival rate is 19%. The higher survival rates
may be due to more intensive chemotherapy protocols or due to
the fact that pediatric HGGs differ biologically from HGGs in adults.
Follow-Up
• Follow-up should always be interdisciplinary and the children
should be under the surveillance of the pediatric neuro-oncologists.
• If recurrence is suspected, there is a role for FDG-PET and MRS to
distinguish between radiation necrosis and tumor recurrence.

1.E) SUBEPENDIMAL GIANT CELL TUMOR
• Subependymal giant cell tumors (SGCTs) are slow-growing
neoplasms characterized as WHO grade I.
• SGCTs are most commonly seen in children and adolescents
with tuberous sclerosis complex (TSC), in whom they
constitute the most common CNS neoplasm. (5%–20% of
patients).
• It is unusual to develop an SGCT after age 21 years if not
already present, although tumors that have been diagnosed
in childhood can become symptomatic later.
• They are supratentorial and virtually always located in a
lateral ventricle near the foramen of Monro, although they
may rarely occur in other locations.

• SGCTs appear to arise from neoplastic transformation of
existing subependymal nod-ules, but the reason why some
nodules grow and others do not is not clear.
• Enhancement is variable but usually avid and heterogeneous.
However, in and of itself, contrast enhancement is not
sufficient for diagnosis, as many subependymal nodules have
also been shown to enhance.
• Both subependymal nodules and SGCTs can calcify and
hemorrhage.
• From a clinical standpoint, the most important factor in the
evaluation of a subependymal nodule or SGCT is the
development of intracranial hypertension with new
papilledema or obstructive hydrocephalus, or growth over
serial imaging.38

1.F) PLEOMORPHIC XANTOASTROCYTOMA
PLEOMORPHIC - refers to the variable histological appearance in which spindle
elements are mixed with mono- or multinucleated giant astrocytes. The nuclei show
great variation in size and chromatism, with frequent pseudoinclusions.
XANTOASTROCYTOMA - Xanthomatous GFAP positive cells with cytoplasmic
accumulation of lipid droplets.

• Pleomorphic xanthoastrocytomas (PXAs) are rare tumors that account for
less than 1% of all astrocytic neoplasms.
• They have a wide range of age at presentation, from early infancy to the
ninth decade of life, with a median of 20 years at the time of diagnosis.4
• Most are classified as WHO grade II and have a relatively favorable
prognosis, with 5- and 10-year survival rates of 75% and 67%,
respectively.40 However, between 10% and 23% display a more agg-
ressive behavior with histologically malignant features, and prognosis
seems to be worse in males and with increasing age.40–42 Anaplastic
pleomorphic xanthoastrocytoma, WHO grade III, has been added to the
2016 CNS WHO as a distinct entity. Patients with such tumors have shorter
survival times when compared to those with WHO grade II PXAs.
• Seventy percent to 80% of patients present with seizures.

Image
The imaging features of PXAs are variable.45 PXAs occur most
commonly in the temporal (39%), followed by the frontal (19%)
and parietal (14%) lobes.40 They are overwhelmingly supraten-
torial, with only 2 cerebellar tumors out of 213 PXAs in the
largest single series published to date.40 These tumors favor a
peripheral location and may scallop the inner table of the
calvarium,
eflecting their slow growth.45 Most are heterogeneous, and
the solid components show avid enhancement and may
characteristically abut the meninges.

Treatment
GTR is the primary goal as it is a strong predictor of PFS. The role
of adjuvant treatment is not well established. Radiation has
should be spared for tumors with anaplastic features.
Outcome
PXAs have very variable outcome. Five-year PFS is reported to be
40 to 68% and 5-year OS rate is 76 to 87%. Ten-year OS rate is
reported to be about 43%.38.

1.G) EPENDYMOMA
• Ependymomas constitute 10% of all primary CNS neoplasms
in children.
• Most occur in the posterior fossa, and 40% are
supratentorial, half of which are situated within the brain
parenchyma.
• A rare subset of supratentorial ependymomas may selectively
involve the cortex and is more commonly associated with
seizures.
• It is believed that parenchymal ependymomas may arise from
em bryonic ependymal rests trapped during development of
the cerebral hemispheres.Due to their parenchymal location,
extraventricular ependymomas tend to be larger at
presentation than intraventricular ones, which more
commonly result in obstructive hydrocephalus.

1.G) EPENDYMOMA
• In most of the cases, they present with elevated ICP.
Imaging
• On imaging, ependymomas are usually well circumscribed
but heterogeneous tumors that show variable degrees of
inhomogeneous contrast enhancement.
• They have a higher incidence of cysts compared with
infratentorial ependymomas; about 50% show areas of
calcification, and hemorrhage may occur.
• MRI of the neuroaxis and CSF analysis for cytology for staging
are indicated.

1.G) EPENDYMOMA
Pathology
The WHO classification system separates ependymomas into
three groups based on histopathological criteria:
• Grade I (myxopapillary);
• Grade II, which is further subdivided into four subtypes
(cellular, papillary, clear- cell, and tanycytic);
• Grade III (anaplastic).

1.G) EPENDYMOMA
PSEUDOROSETAS PERI-VASCULARES

1.G) EPENDYMOMA
Recent molecular classification of ependymomas identified nine different
subgroups in the CNS, with three subgroups in the supratentorial location.
1vo supratentorial subgroups are in the pediatric population and are
characterized by prototypic fusion genes involving REI.A and YAPl,
respectively.74

1.G) EPENDYMOMA
Treatment
• Ependymoma remains a "surgical disease.”
• Surgical considerations:
- aim for GTR:the most important prognostic factor.
- GTR and GTR combined with external beam
radiation therapy results in the longest time to
recurrence/progression.
- Subtotal resection is correlated with inferior
outcome.

1.G) EPENDYMOMA
Outcome
• Favorable outcome is reported with a 5-year OS rate of 72
to 85%.
• There are case reports of grade II ependymomas with GTR
without adjuvant therapy and long-term survival.
• In a report of 40 pediatric patients, the Children's Cancer
Group found no differences in PFS across WHO grades.

1.H) ANGIOCENTRIC GLIOMA
• Angiocentric gliomas are now recognized as a distinct subset of glial
tumors with uncertain histogenesis but with some degree of astrocytic
and ependymal differentiation.
• Two independent case series were first described in 2005, and these
lesions were listed as a new entity in the WHO classification of tumors of
the CNS in 2007.
• Angiocentric gliomas are by far tumors of children and less commonly
young adults, although a few cases in older patients have also been
described.
• They are relatively indolent and slow growing (WHO grade I), and most
come to attention due to longstanding or intractable seizures.
Angiocentric gliomas are superficial nonenhancing cortical lesions, although
a few cases showing subtle to mild enhancement have been reported.67
Some of them may be intrinsically hyperintense on T1-weighted sequences
and have a stalk-like extension to the adjacent ventricle on T2- weighted
sequences, features thought to be characteristic but inconsistently present
(Fig. 12).64,68 A recent study using MRS has found a myoinositol and/or

1.H) ANGIOCENTRIC GLIOMA
• Angiocentric gliomas are superficial nonenhancing cortical lesions,
although a few cases showing subtle to mild enhancement have been
reported.
• Some of them may be intrinsically hyperintense on T1-weighted
sequences and have a stalk-like extension to the adjacent ventricle on T2-
weighted sequences, features thought to be characteristic but
inconsistently present (Fig. 12).
• A recent study using MRS has found a myoinositol and/or glycine peak in
an angiocentric glioma, but for the most part their spectral
characteristics overlap with those of other low- grade neoplasms.

2) MIXED NEURONAL
AND
GLIAL TUMORS

2) MIXED NEURONAL AND GLIAL TUMORS
• Neuronal and mixed neuronal–glial cell tumors are rare,
representing nearly 1% of all primary brain tumors in
children, with a median age of 9 years at presentation
• In the pediatric population, their incidence is highest in the
10 to 14 years age group, among whom they constitute
6.5% of brain tumors.
• The more relevant neuronal–glial tumors will be discussed,
while recognizing that various other entities may be
included under the same classification.

2.A) GANGLIOGLIOMA
Epidemiology
The majority of gangliogliomas are localized in the temporal
lobe, but can occur throughout the CNS. Most of the
supratentorial cases are temporal and frontal.
Seizures are the most frequent presentation, and gangliogliomas
are the most common tumor to cause intractable, chronic
pediatric epilepsy.

2.A) GANGLIOGLIOMA
Imaging
• MRI shows a circumscribed mass and enhancement varies; a
mural nodule with a cyst is also described. In cr. calcifications
may be visible.
• The conventional imaging characteristics of gangliogliomas
are nonspecific.
• Most of them will present as mixed cystic and solid masses
with avidly enhancing tumoral components, and calcifications
are a common feature.
• As with other lesions, ADC values tend to decrease with
higher tumor grades, which may aid in their preoperative
evaluation.74

2.A) GANGLIOGLIOMA
Pathology
• Well-differentiated, slow-growing neuroepithelial tumor
composed of neoplastic, mature ganglion cells alone
(gangliocytoma) or in combination with neoplastic glial cells
(ganglioglioma). Polymophisms ofthe 7SC1 and 7SC2genes
have been common in patients with
gangliogliomas.Anaplastic gangliogliomas are rare.
• Interestingly, tumors previously diagnosed as other low-grade
histologies have been reclassified as gangliogliomas after the
2000 WHO classification system was introduced, and newer
and more specific immunohistochemical profiles became
available (expression of CD34 and lack of MAP2 expression)

2.A) GANGLIOGLIOMA
Treatment
• It is very important to identify pre- and intraoperatively the
epilepsy focus and to perform a complete lesionectomy. The
use of intraoperative neuromonitoring is indicated.
• GTR should be performed safely whenever possible and does
not require postoperative irradiation.
• If only a subtotal resection is achieved, then radio therapy
may improve long- term tumor control of both low-grade and
high- grade tumors and, thus, should be considered.

2.A) GANGLIOGLIOMA
Outcome
• Overall, outcome is favorable. Five-year PFS rate is 81.2% and
the OS rate is 97.4%.
• The 15-year OS rate is reported to be 94%.
• PFS is affected by the extent of initial resection, so GTR should
be achieved.

3) Dysembryoplastic
Neuroepithelial
Tumor
(DNETs)

3) Dysembryoplastic Neuroepithelial Tumor
• Dysembryoplastic neuroepithelial tumors (DNETs) are benign
glioneuronal neoplasms (WHO grade I) most commonly seen
in children and adolescents who present with intractable
seizures.
• They have a peak incidence during the second decade of life
and are more common in males.
• The great majority of DNETs are cortically based lesions and,
while they can occur anywhere in the supratentorial brain,
they preferentially arise in the temporal lobes.
• Less common locations include the brain stem, cerebellum,
and striatum

Epidemiology
DNET belongs to the neuronal-glial tumors, which together with
the neuronal tumors comprise 6.5 to 7.9% of CNS tumors in
children.
Pathology
DNET is a grade I glioneuronal tumor in the WHO classification
and shows no IDH1 mutations. The genetic basis of DNET is not
well known or well defined. Somatic FGFRI alterations and MAP
kinase pathway activation are events that both cause DNETs to
develop. Gains of chromosomes 5 and 7 and loss of
chromosomes 1p, 1Oq, and 19q have been observed and are
associated with DNETs.

Clinical Evaluation
• Preoperative evaluation must always include
consultation with the epilepsy service. Children with
epilepsy and AED may have coagulation
abnormalities, so preoperative hematology
consultation may be indicated.

Imaging
• MRI appearance: DNETs are characterized as having
a "bubbly" appearance, are multilobulated, are
hypointense on T1 in a wedge-shaped configuration,
and hyperintense on T2-weighted images.
• FLAIR imaging shows a characteristic bright rim.
DNETs have one of the highest apparent diffusion
coefficient values among benign tumors. MRS is
nonspecific.

Treatment
• Surgery is curative if the tumor can be completely resected.
DNETs in the temporal lobe are often resected by temporal
lobectomy (with or without hippocampectomy).
Outcome
• DNETs have a very good outcome from an oncologic
standpoint, similar to LGGs. Long-term seizure freedom can
be achieved in 86%.

4) EMBRYONAL TUMORS –
NOS
(PNET)

4) EMBRYONAL TUMORS – NOS (PNET)
• One of the major changes in the 2016 CNS WHO classification
has been the removal of primitive neuroectodermal tumors
(PNETs), which accounted for 15% of all embryonal neoplasms
and which are now included within the category of embryonal
tumors not otherwise specified (NOS).
• They are usually seen in children less than 5 years.

• Embryonal tumors NOS comprise a heterogeneous group of
aggressive malignancies (WHO grade IV) that are biologically
distinct from medulloblastomas and histologically include:
- Neuroblastomas
- Ganglioneuroblastomas
- Ependymoblastomas
- Medulloepitheliomas.

Epidemiology
• Supratentorial primitive neuroectodermal tumors (SPNETs)
account for approximately 2.5% of all pediatric brain tumors.c
• As it is a rapidly growing tumor, it often presents with
increased ICP.
• Focal neurologic deficits and seizures are described as well.

Clinical Evaluation
• Patients should have a staging workup to assess the
extent of disease.
• Preoperative MRI of the brain, MRI of the spinal
axis, and lumbar CSF sampling for cytological
examination (lumbar and intraoperatively) is
mandatory.

Pathology
Definition of the WHO Working Group:
Heterogeneous embryonal tumor composed of undifferentiated
or poorly differentiated neuroepithelial cells which have the
capacity for or display divergent differentiation along neuronal,
astrocytic, and ependymal lines.
• Cerebral neuroblastornas- only neuronal differentiation.
• Ganglioneuroblastomas-neuronal differentiation and ganglion
cells.
• Medulloepitheliomas-features of neural tube formation.
• Ependymoblastomas- ependymoblastic rosettes.

Image
• Hemorrhage may be present but is relatively rare, and there is
typically little surrounding edema.
• As is the case with other high-grade malignancies, embryonal
tumors also show restricted diffusion with low ADC values
and are hyperdense on CT due to high cellularity.
• Contrast enhancement is generally intense and
heterogeneous, and these tumors have a propensity for
leptomeningeal spread.

Treatment
This tumor is often highly vascularized, so hypervolemia due to
blood loss is a common complication.
Be aware of brain collapse; occasionally, a shunt is needed due
to hydrocephalus. Surgical resection of tumor should be done
only if additional permanent neurologic deficits can be spared.
The role of GTR is controversial.
Chemotherapy
All patients are treated with chemotherapy. The specific
protocol should be discussed in tumor board. Chemotherapy
combined with radiation therapy has been associated with a
significant increase in survival.

Radiation Therapy
Radiation of neuroaxis with focal beam on tumor location is
included in most protocols. Whole-spine radiation is indicated
for spinal metastasis.
There are promising data about favorable local control and low
rates of acute radiation-induced toxicity for proton radiation
therapy.
Outcome
Despite adjuvant chemo- and radiotherapy, the outcome is
relatively poor. One-year survival rate is 76.4% and 5-year
survival is 49.5%.
Children younger than 2 years have a worse prognosis.

5) ATYPICAL RHABDOID
TERADOID
TUMORS

5) ATYPICAL RHABDOID TERADOID TUMORS
The term rhabdoid comes from the Greek rhabdos, which means
stick or rod. It is used as root in words that refer to striated
muscle, such as rhabdomyolysis, rhabdomyosarcoma.

Epidemiology
Around 80% of the patients diagnosed with ATRT are children
younger than 3 years. There is a slight male predominance of
58%. About 50% are supra-tentorial and 50% infratentorial.
As most of the tumors arise in infants, symptoms of elevated ICP
such as macrocephaly, bulging fontanels, and vomiting are the
presenting symptoms.

Risk Factors
In older children, ATRT can occur as secondary tumor after
whole-brain irradiation.
Pathology
The main histologic characteristics of rhabdoid tumor cells are
abundant cytoplasm with juxtanuclear eosinophilic inclusions
and nuclei that display a single, prominent nucleolus in clear,
uncondensed chromatin.
A defect in the INI 1 gene (SMARCB1) leading to loss of
expression is associated with rhabdoid tumors and is used for
diagnosis. SMARCBt gene abnormalities are found in 76 to 95% o
f ATRT patients.

Image
• MRI: A supratentorial tumor with a thick, irregular,
heterogeneously enhancing wall surrounding a central cystic
region is suggestive of ATRT, as it is a distinctive and unusual
pattern. It is present in approximately 40% of ATRT.
• MRI spectroscopy may be helpful to distinguish them from
other brain tumors. The combination of prominent choline
and lactate and lipid peaks and generally absent NAA and
myoinositol peaks provide a metabolite profile typically
distinct from other malignant pediatric brain tumors.
• MRI of the neuroaxis for staging reasons is mandatory,
espedally in young children, as 13% present with metastatic
disease.

Treatment
• Improved OS and PFS have been reported with maximal
primary tumor resection, but an association between the
extent of resection and OS is still controversiaJ.
Chemotherapy
• High-dose chemotherapy regimens seem to provide good
results and increase survival.
• Intrathecal therapy is widely used as it has shown significantly
higher survival rates.

Radiotherapy
• In children younger than 3 years, the strategy is usually to
avoid or delay radiation.
• Given the poor prognosis of ATRT, delayed risks of
neurocognitive developmental problems should be balanced
with potential survival benefit derived from adjuvant
radiotherapy.
• The advantages of proton therapy are particularly suited to
the treatment of ATRT, since the disease often requires
radiation treatment at an early age.

Outcome
• One-year survival is 48% and 5-year survival is 28%.
• The median OS is about 10 months and metastatic disease
correlates with a worse prognosis.
• Age less than 2 years, metastasis at diagnosis, and strong
daudin-6 positivity appear to be independent prognostic
factors for outcome.

Pediatric Supratentorial Tumors / Tumores Supratentoriais Pediátrico

Pediatric Supratentorial Tumors / Tumores Supratentoriais Pediátrico

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