Imaging of Common Adult

and Pediatric Primary Brain Tumors

Humberto Morales, MD, and Mary Gaskill-Shipley, MD

he primary goals of brain tumor imaging are lesion detection, localization, delineation of extent, and characterization. This information is used to formulate an appropriate differential diagnosis that is extremely helpful for the
referring neuro-surgeons and the neuro-oncologists.1,2 In addition, imaging studies play a vital role in therapy planning,
such as stereotactic location for surgery or radiotherapy, and
assessing response to therapy.3
This article discusses the imaging characteristics of several
common pediatric and adult primary central nervous system
(CNS) tumors. The review will focus on the initial imaging
diagnostic workup and will give a useful radiological approach based on age, localization, imaging characteristics,
and relative frequency of brain tumors. Advanced magnetic
resonance (MR) techniques including spectroscopy and perfusion can provide additional information of brain tumors.
These techniques will be discussed separately in this supplement.

Classification
The World Health Organization (WHO) classifies primary
brain tumors according to their cellular origin. The major
categories include neuroepithelial tumors, tumors of the meninges, lymphoma and hematopoietic neoplasms, germ cell
tumors, tumors of the cranial and paraspinal nerves, tumors
of the sellar region, metastatic tumors, and cysts and tumorlike lesions.4,5 Tumors of neuroepithelial origin comprise a significant number of primary brain tumors, including astrocytomas, oligodendrogliomas, ependymomas, choroid plexus
tumors, neuronal and mixed neuronal-glial tumors, pineal lesions, and embryonal tumors.
Grading of brain tumors is according to the WHO classification that assigns a grade of 1-4 from benign to malignant,
taking into account the presence of nuclear changes, mitotic
activity, endothelial proliferation, and necrosis. The prototypic WHO grade 1 tumor is the pilocytic astrocytoma (PA)

Division of Neuroradiology, University Hospital, Cincinnati, OH.

Address reprint requests to Humberto Morales, MD, Division of Neuroradiology, University Hospital, 231 Albert Sabin Way, Cincinnati, OH
45267. E-mail: moralehc@ucmail.uc.edu

92

0037-198X/10/$-see front matter 2010 Elsevier Inc. All rights reserved.

doi:10.1053/j.ro.2009.09.006

that has a 90% 5-year survival after resection whereas glioblastoma multiforme (GBM) represents one of the most common WHO grade 4 tumors with a 4% or less survival at 5
years.6
Although the final diagnosis and grading of brain tumors is
determined by histologic analysis, imaging can be very helpful in the initial assessment of neoplasms and can, in many
cases, help direct surgical biopsy or treatment. Imaging features associated with increasing malignancy include mass
effect, vasogenic edema, enhancement, necrosis, and hemorrhage, particularly when astrocytic tumors are compared.7,8
Cerebral blood volume (CBV), which can be calculated from
MR perfusion studies and choline/creatine (Cho/Cr) ratios
based on MR spectroscopy, are relatively elevated in malignant tumors, secondary to increased vascularity and cell proliferation, respectively.8,9 However it is important to note that
some low-grade tumors can demonstrate aggressive features on imaging. In children, PAs may have a malignant
appearance on conventional imaging, increased metabolism
on positron emission tomographic images, and increased
rCBV and Cho/Cr ratios.10,11 In adults, oligodendrogliomas
often have increased rCBV.12,13

Diagnostic Approach to
Intracranial Tumors Based
on Frequency, Age, Location,
and Imaging Characteristics
The radiologists ultimate goal in the initial imaging evaluation of brain tumors should be to provide an appropriate
differential diagnosis that will guide future intervention
and treatment. Accurate diagnosis is based not only on the
radiological appearance of the tumor but also on the patients age and the location and relative incidence of the
lesion (Table 1).14 Each of these factors must be taken into
account.
The yearly incidence of primary brain tumors is 16.5 cases
per 100,000 population or approximately 30,000-35,000
new cases per year. In comparison, intracranial metastases
are much more common with as many as 170,000 cases
reported each year. The overall incidence of primary tumors

Imaging of primary brain tumors

93

Table 1 Diagnostic Approach of Primary Brain Tumors by Age, Location, and Relative Frequency
Pediatric Brain Tumors

Adult Brain Tumors

Extraaxial
Rare in pediatric population: schwannoma and meningioma.

Intraaxial
Supratentorial
Cortical
Neuronal and mixed neural-glial (DNET, ganglioglioma)
and pleomorphic xanthoastrocytoma.
Corticomedullary
Low-grade astrocytoma and S-PNET.
Deep white matter
Fibrillary/anaplastic astrocytoma, supratentorial
ependymoma, and glioblastoma.

Infratentorial
Cerebellar hemisphere
Pilocytic astrocytoma (PA) and ATRT.
Intraventricular
Fourth ventricle
Medulloblastoma/PNET-MB and ependymoma.
Lateral ventricle
Choroid plexus papilloma, SEGA, meningioma, ependymoma,
and choroid plexus carcinoma.

Midline
Suprasellar-chiasmatic-optic
Craniopharyngioma, PA, germinoma, LCH, hypothalamic
hamartoma, lipoma, dermoid, and PMA.
Pineal region
Germinoma, teratoma, and pineoblastoma.
Brainstem
Tectal glioma, focal/diffuse pontine, and medullary and
midbrain astrocytomas.
Multiple spaces
Embryonal tumors (PNET, ependymoblastoma, and
neuroblastoma), ATRT.

Extraaxial
Convexities
Meningioma, hemangioendothelioma, and
hemangioperycitoma.
Cerebello-pontine angle
Schwannoma, meningioma, and epidermoid.
Intraaxial
Supratentorial
Cortical
Pleomorphic xanthoastrocytoma.
Corticomedullary
Oligodendroglioma and fibrillary/anaplastic
astrocytoma
Deep white matter
Glioblastoma and fibrillary/anaplastic
astrocytoma
Corpus callosum/periventricular white matter
Glioblastoma, lymphoma, and gliomatosis
cerebri.
Infratentorial
Cerebellar hemisphere
Hemangioblastoma and astrocytoma.
Intraventricular
Fourth ventricle
Subependymoma, choroid plexus papilloma, and
ependymoma.
Lateral ventricle
Meningioma, ependymoma, central neurocytoma,
and subependymoma.
Third ventricle
Colloid cyst, astrocytoma, and chordoid glioma.
Midline
Sellar/suprasellar
Pituitary adenoma, meningioma, and
craniopharyngioma.
Pineal region
Pineal cyst, pineocytoma, and
epidermoid/dermoid.

Multiple spaces
Malignant meningioma, hemangiopericytoma, and
lymphoma.

Abbreviations: DNET, dysembryoplastic neuroepithelial tumor; S-PNET, supratentorial primitive neuroectodermal tumor; ATRT, atypical teratoid
rhabdoid tumor; SEGA, subependymal giant cell astrocytoma; LCH, Langerhans cell histiocytosis; PMA, pilomyxoid astrocytoma.

increases with age. The pediatric population (0-19 years) has

a low incidence rate of approximately 4.5 cases per 100,000
population/year while the highest occurrence rate is observed
in patients older than 50 years of age, accounting for over 30
cases per 100,000 population/year.6
The location and histology of primary brain tumors vary
with age. Although the most common location for primary
neoplasms in both adults and children is the cerebral hemispheres; brain tumors in children have a special predilection
for the posterior fossa. In comparison, primary tumors located in the cerebellum are unusual in adults. Therefore, in

the adult population, a solitary lesion in the posterior fossa is

most commonly a metastasis.
Differentiating between an intra- and extraaxial location of
a mass will significantly alter potential diagnoses. Although
extraaxial primary tumors, such as meningiomas, are common in adults, they are very uncommon in the pediatric
population. Intraventricular tumors have a very specific differential diagnosis, which can vary depending on which ventricle is involved. If a tumor is intraaxial, determination of its
specific location such as cortex, corticomedullary junction,
or white matter lesion will alter possible diagnoses.

H. Morales and M. Gaskill-Shipley

94

Figure 1 Distribution of primary brain and central nervous system tumors by histology and age (Modified from CBTRUS
2000-2004). (Color version of figure is available online.)

Different tumor types occur at very different rates at various ages (Fig. 1). The most common tumor of childhood is
the PA, followed by medulloblastoma. However, in adulthood, the most common tumor is meningioma, followed by
glioblastoma. Oligodendroglioma, which is common in
adults, is very rare in children. Neuronal and mixed neuralglial tumors are more common in children than in adults
(Table 1). Therefore, correlating the location and characteristics of a tumor with the age of the patient is crucial in
determining an appropriate differential diagnosis.

Tumors More
Common in Childhood
Pilocytic Astrocytoma (WHO Grade 1)
PA is the most common primary brain tumor in children.
Overall, patients with PAs have a good prognosis after resection with a 94% survival rate at 10 years. Common locations
for PAs include the cerebellum, optic nerve/chiasm/hypothalamic region, or brain stem. Involvement of the cerebral
hemispheres is less frequent.
Pilocytic astrocytomas are typically well-defined lesions
that classically present as a cystic mass with an enhancing
mural nodule, however solid lesions do occur. Significant
vasogenic edema is rare. These are slow growing tumors that
often present due to localized mass effect. Lesions occurring
in the cerebellum may significantly compress the fourth ventricle, making it difficult to identify the tumors parenchymal
origin. The main differential diagnosis is between the 2 most

common pediatric fourth ventricular tumors, medulloblastoma (MB), and ependymoma (Fig. 2).15,16
PAs occurring in the optic nerve/chiasm/hypothalamic region have been separated into 3 subsets. In the pediatric
population, tumors are divided into 2 types: those associated
with neurofibromatosis type 1 (NF1) and those not associated with NF1. PAs in patients with NF1 are usually bilateral
nonenhancing tumors involving the optic nerves and less
commonly, the optic chiasm/hypothalamic region. They
have an indolent course. PAs in non-NF1 patients usually
involve the chiasm/hypothalamic region. They are typically
solid/cystic enhancing tumors, which are often larger than
PAs associated with NF1 and have a less indolent course. The
third subset of tumors, which is identified in adults, demonstrates more aggressive behavior.
As mentioned earlier, PA may demonstrate a false aggressive appearance on MR spectroscopy with significant elevation of Cho/Cr ratios. Recent studies have shown very low
concentrations of Cr in PAs compared with other pediatric
tumors, as well as diminished quantities of total Cho. Therefore, the paradoxical increase in Cho/Cr ratios does not necessarily indicate increased cell proliferation.17

Medulloblastoma/
PNET-MB) (WHO Grade 4)
MB or PNET-MB (primitive neuroectodermal tumor-medulloblastoma) is a highly cellular, rapidly growing malignant
embryonal tumor. It is the second most common tumor in
children but the most common posterior fossa lesion. These
masses are typically located in the fourth ventricle arising

Imaging of primary brain tumors

95

Figure 2 (A, D) Pilocytic astrocytoma in a 7-year-old girl with headaches, vomiting, and papilledema. (B, E) Medulloblastoma in a 3-year-old boy with headaches and vomiting. (C, F) Ependymoma in a 2-year-old girl with vomiting.
(A, B, C) Axial computed tomography (CT) noncontrast images. (A) Isodense complex cystic-solid mass centered in the
mid cerebellum slightly right to midline. Note the mass effect upon the fourth ventricle. (B) Hyperdense predominantly
solid mass centered in the fourth ventricle with small cystic/necrotic areas. An additional small hyperdense lesion is
noted in the suprasellar region. (C) Isodense solid mass in the fourth ventricle with speckled calcifications. (D, E, F)
Axial and sagittal T1 with gadolinium images. (D) Complex mass with peripheral enhancement of the cystic/necrotic
portions causing significant effacement of the fourth ventricle. (E) Solid enhancing mass within the fourth ventricle,
associated with small enhancing lesion in the suprasellar region most consistent with cerebrospinal fluid dissemination.
(F) Lobulated heterogeneous enhancing mass extending out through the floor of the fourth ventricle. Note ventricular
enlargement in all 3 patients secondary to obstructive hydrocephalus (Images courtesy of Dr. Blaise Jones, MD,
Cincinnati Childrens Hospital).

from the superior medullary velum. Involvement of the cerebellar hemispheres is rare, usually occurring in older children and adults and typically representing desmoplastic medulloblastomas.18 MBs have a variable prognosis after resection
with a 50% survival rate at 10 years.
MBs are typically isointense on T1 weighted images,
mildly hypointense to cortex on T2 weighted images, and
homogeneously enhance. The desmoplastic variant that has a
better prognosis often demonstrates a different imaging pattern characterized by calcifications and subtotal heterogeneous enhancement.19 Diffusion restriction is commonly observed due to the tumors dense cellularity.20 Ninety percent

of these lesions are dense on computed tomography (CT),

which can aid in the radiographic diagnosis (Fig. 2). Approximately one-third of MBs will have subarachnoid dissemination at the time of presentation; therefore, imaging the entire
neuroaxis is extremely important to evaluate the extent of
disease.
Supratentorial primitive neuroectodermal tumors (S-PNET)
are a subset of tumors genetically distinct from infratentorial PNETs (PNET-MB). Though only 6% of PNETs are
supratentorial, they are important hemispheric masses to
consider in newborns and infants. S-PNETs are large, complex hemispheric masses with heterogeneous enhancement

H. Morales and M. Gaskill-Shipley

96

Figure 3 Supratentorial primitive neuroectodermal tumor in a 1-year-old boy. (A) Axial noncontrast CT image shows a
large mass centered in the trigone of the left lateral ventricle extending into the parietal and occipital brain parenchyma.
There are multiple calcifications and enlargement of the left lateral ventricle. Note ventricular drainage catheter tip in
the right frontal horn. (B) Axial T2 image shows a large mass with significant heterogeneous signal. Areas of dark signal
predominantly in the periphery and cortical regions are consistent with calcifications/hemorrhagic staining.
(C) Coronal T1 with gadolinium image shows heterogeneously enhancing mass with solid/cystic components involving
the ventricle and brain parenchyma as well as extending into the dural/extracranial spaces. Involvement of multiple
spaces is an important characteristic of supratentorial primitive neuroectodermal tumor (Images courtesy of Dr. Blaise
Jones, MD, Cincinnati Childrens Hospital).

and minimal peritumoral edema.21 The differential diagnosis

of these lesions includes teratoid or rhabdoid tumors and
ependymomas. Unlike PNET-MB, calcifications, hemorrhage, and necrosis are common (Fig. 3). Patients with
S-PNET have decreased survival compared to those with
PNET-MB.

Ependymoma (WHO Grade 2)

Ependymomas are the fourth most common posterior fossa
tumor in the pediatric population.22 They arise from ependy-

mal cells lining the ventricles and are most commonly located in
the fourth ventricle. Unlike medulloblastomas, which originate
superiorly from the medullary velum, ependymomas arise from
the floor of the fourth ventricle (Fig. 2). One-third of ependymomas are located supratentorially where they have a
characteristic deep parietal white matter location rather
than intraventricular. They are slow growing tumors with an
overall 60%-70% survival rate at 5 years after resection.
Infratentorial ependymomas typically preset as heterogeneous enhancing lesions that often extend out through the

Imaging of primary brain tumors

foramina of Luschka and Magendie into the cisterns. Punctate calcifications occur in approximately 50% of cases,
therefore CT may be helpful in differentiating this tumor
from MB and PA (Fig. 2).23 Ependymomas may also demonstrate cystic transformation, hemorrhage, necrosis, and
edema.

Choroid Plexus Papilloma/

Carcinoma (WHO Grade 1 and 4)
Choroid plexus papillomas (CPP) are intraventricular papillary neoplasms that are derived from the choroid plexus epithelium. CPP is one of the most common brain tumors in
children below the age of 2 years.24 In the pediatric population they are located in the atrium of the lateral ventricle. In
contrast, tumors in adults are typically located in the fourth
ventricle and cerebellopontine angles. CPP is a slowly growing tumor with a 5-year survival rate close to 100%.
Imaging features include a frond-like lobulated mass with
intense enhancement. Calcifications and hemorrhage are frequently present. Hydrocephalus is very commonly associated
with CPPs and can be due to either overproduction or obstruction of cerebrospinal fluid (CSF).
Choroid plexus carcinomas (CPC) are malignant neoplasms that account for approximately 20%-30% of all choroid plexus masses. Most cases occur in children and also
typically arise in the lateral ventricles. CPCs have a poor
prognosis with a 50% survival rate at 5 years. Imaging findings that can help differentiate CPP from carcinomas include
extension of the lesion beyond the ventricle wall, prominent
vasogenic edema, and mass effect.23 Both CPPs and CPCs
can seed the CSF, however metastatic spread is more commonly observed with carcinomas.

97

Neuronal and Mixed

Neural-Glial Tumors (WHO Grade 1-2)
Dysembryoplastic neuroepithelial tumors (DNET) and gangliogliomas/gangliocytomas are the most common mixed
neural-glial tumors in children and young adults. These lesions are cortical tumors often associated with refractory seizures.
DNET is a benign mixed neural-glial tumor frequently
associated with a background of cortical dysplasia.25 They are
most commonly found in the temporal lobes; however, they
can arise in other areas of the cerebral hemispheres and posterior fossa. On magnetic resonance imaging (MRI), DNETs
typically have a bubbly or multicystic appearance. Most
lesions are well defined with no edema and little mass effect.
Enhancement is present in up to 50% of cases and is usually
nodular or ringlike in appearance. Calcification may be
present. A FLAIR (fluid attenuated inversion recovery)
bright ring sign has been described with these tumors,
which is a complete or incomplete hyperintense rim that may
be caused by the presence of loose peripheral neuroglial elements (Fig. 4). This sign may help differentiate these tumors
from other cortical lesions.26
Gangliogliomas (WHO grade 1 or 2) and gangliocytomas
(WHO grade 1) are also cortically based neoplasms. Gangliogliomas are composed of both benign mixed atypical ganglion cells and neoplastic glial cells, whereas gangliocytomas
are primarily composed of dysplastic or neoplastic neurons.
Although both lesions are usually benign, rare cases of malignant degeneration or metastatic spread have been reported.27 These lesions can be either solid, cystic, or mixed.15
Approximately 50% will present as a cystic mass with a mural

Figure 4 Dysembryoplastic neuroepithelial tumor in a 10-year-old girl with seizures. (A) Axial fluid attenuated inversion recovery (FLAIR) image shows a cortical lesion in the left temporal lobe, with an incomplete medial rim of
hyperintense signal. (B) Coronal T1 image with gadolinium shows subtle enhancement of the cortical lesion. Note the
bubbly appearance with small rounded areas of hypointensity on FLAIR and T1 near the cortex (Images courtesy of
Dr. Blaise Jones, MD, Cincinnati Childrens Hospital).

H. Morales and M. Gaskill-Shipley

98
nodule. Most lesions demonstrate either solid or heterogeneous enhancement. Mass effect and edema are uncommon.

Brainstem Tumors (WHO Grade 1-4)

Most brainstem tumors are gliomas, including fibrillary astrocytomas, PAs, and rarely glioblastomas. Eighty percent of
all brainstem gliomas occur in the pediatric population. They
are categorized according to where they develop (pontine,
medullary, and midbrain) and whether they are diffuse or
focal. Prognosis varies greatly depending on the location and
grade of the lesion.
Pontine gliomas are more often diffuse lesions that may
present with cranial nerve palsies and ataxia.28 These lesions
are often high-grade and have a poor long-term prognosis.
Imaging features include expansion of the brain stem with
abnormal T2 prolongation. Enhancement is usually absent.
Tectal gliomas typically have a distinctly benign behavior
compared to other brainstem tumors. Some authors believe
that these tumors are hamartomas; however, other series
have histologically shown these tumors to be low-grade PAs.
Due to their location adjacent to the sylvian aqueduct, these
lesions often present with obstructive hydrocephalus. They
usually do not require treatment beyond CSF diversion such
as third ventriculostomy or shunting.24

Tumors More
Common in Adulthood
Astrocytomas
Gliomas are the most common primary intraaxial mass in
adult population. Astrocytomas account for over half of all
gliomas and can be divided into 2 major categories, infiltrative and noninfiltrative. Infiltrative astrocytomas are much
more common, representing 75% of all lesions. The nonin-

filtrative subset includes PAs, pleomorphic xanthoastrocytomas, subependymal giant cell astrocytomas, and desmoplastic cerebral astrocytomas of childhood. This section will
primarily focus on the infiltrative type.
Infiltrative astrocytomas range from low-grade lesions to
highly aggressive malignant neoplasms. Grading these tumors is based on several histopathologic features including
cellularity, nuclear atypia, mitotic activity, endothelial proliferation, and necrosis. As described previously several imaging features including mass effect, edema, enhancement, and
necrosis can help predict tumor grade.
Low grade astrocytomas (WHO grade 2) comprise approximately 25% of all gliomas.29 They tend to occur in younger
adults, often in the third and fourth decades of life. Low grade
gliomas are best visualized on MRI and typically present as
nonenhancing well demarcated or ill-defined lesions in the
cerebral hemispheres (Fig. 5). Edema is usually absent. Typically there is absent or minimal mass effect, a finding which
can be striking given the size of some lesions.
Anaplastic astrocytomas (AA) (WHO grade 3) occur in a
slightly older population and confer a worse prognosis. The
median survival rate for these patients is 2-3 years. Most AAs
result from dedifferentiation of low grade gliomas, however
some arise de novo.30 Histologically, AA contains gemistocytes and protoplasmic elements but no necrosis. Dissemination occurs through the white matter tracts. AAs typically
demonstrate edema and mass effect that helps to differentiate
them from low grade gliomas (Fig. 6). Approximately twothirds of AAs will partially enhance.
GBM (WHO grade 4) is the most aggressive and malignant
form of astrocytoma and is characterized histologically by
necrosis and neovascularity. It is the second most common
tumor in adults after meningioma and usually occurs in patients older than 50 years of age.6,31 Involvement of patients

Figure 5 Low-grade astrocytoma in a 35-year-old woman who presented with seizures. (A) Axial T2 weighted image
demonstrates a moderate sized area of hyperintense signal involving the white matter and cortex of the left frontal lobe.
(B) Axial T1-weighted post gadolinium image demonstrates subtle hypointensity within the lesion. No abnormal
enhancement is observed. Note the relative lack of mass effect despite the size of the lesion.

Imaging of primary brain tumors

99

Figure 6 Anaplastic astrocytoma in a 48-year-old man with visual abnormalities and headaches. (A) Axial T2-weighted
image shows a large lesion in the right medial occipitoparietal lobes. Surrounding abnormal T2 signal extends into the
right parietal white matter and across the splenium of the corpus callosum. (B) Axial T1-weighted post gadolinium
image demonstrates a nonenhancing, hypointense mass. Note the mass effect and midline shift that helps to differentiate this lesion from a low-grade astrocytoma. One-third of anaplastic astrocytomas will not enhance.

less than 30 years is rare. These tumors have the worst prognosis among primary brain tumors. The median postoperative survival time is 8 months, and the overall 10-year survival rate in patients older than 45 of age is less than 1.8%.
GBMs are most commonly located in the supratentorial
white matter. Involvement of the brainstem and cerebellum
is rare; however, these locations are more common in children than adults (Fig. 7). On MRI, GBMs are usually identified as large heterogeneously enhancing masses with significant necrosis, mass effect, and vasogenic edema (Fig. 8).

Hemorrhage of differing stages is often observed. Involvement of the bilateral hemispheres through the corpus callosum (butterfly glioma) is a classic presentation.
Surgical resection of GBMs is nearly always incomplete
due to the highly infiltrative nature of the tumor. Biopsies
taken from around the margins of resection cavities have
demonstrated tumor cells in the surrounding edema and
even in normal appearing white matter.32 Surgical resection and radiation are frequently targeted to the enhancing
(higher grade) portion of the tumor, however the presence

Figure 7 Glioblastoma multiforme in a 5-year-old boy with abnormal gait and vomiting. (A) Axial T2 image shows an
intraaxial mass centered in the right brachium pontis with a central area of necrosis and thick peripheral solid
component. (B) Axial T1 postgadolinium image shows minimal enhancement of the thick peripheral component.
Cerebellar and brainstem glioblastomas are rare, however they are more common in children than adults.

H. Morales and M. Gaskill-Shipley

100

Figure 8 Glioblastoma multiforme in a 59-year-old man who presented with seizures and neurologic deficit. (A) Axial
T2-weighted image demonstrates a mildly hyperintense mass in the lateral right frontal lobe. Note the prominent
surrounding abnormal T2 signal in the adjacent white matter that represents edema and infiltrative tumor. (B) Axial
T1-weighted post gadolinium image shows an irregularly enhancing mass with areas of necrosis.

of tumor cells in the surrounding tissue limits curative

therapy.

Oligodendroglioma (WHO Grade 2)

Oligodendrogliomas are infiltrative gliomas that originate from
oligodendroglial cells. They occur in middle-aged adults and
often present with seizures. Pediatric involvement is rare. Oligodendrogliomas may be low grade (WHO grade 2) or anaplastic (WHO grade 3). Similar to astrocytomas, higher grade
oligodendrogliomas demonstrate increased cellularity,
mitotic activity, endothelial hyperplasia, and necrosis. Mixed
oligodendrogliomas contain both features of astrocytomas and
oligodendrogliomas, and can be classified as grade 2 or 3.

Many oligodendrogliomas demonstrate 1p and/or 19q

chromosomal deletions.33 The presence of these chromosome losses increases the tumors response to radiation and
chemotherapy and improves prognosis. Patients with both
1p and 19q deletions have a median survival time of 8-10
years whereas patient without the deletions have a survival
time of 3-4 years.
Differentiating oligodendrogliomas from astrocytomas on
conventional imaging is often not possible. However, several
imaging features favor the diagnosis of oligodendroglioma,
including cortical involvement, heterogeneous signal, intratumoral cysts, patchy enhancement, and the presence of calcifications34,35 (Fig. 9). Calcifications, which can be seen in

Figure 9 Oligodendroglioma in a 30-year-old man who presents with seizures. (A) Axial T2 FLAIR image shows a large,
mildly heterogeneous left frontal mass involving cortex. Focal areas of hypointense signal represent calcifications that
were confirmed on CT. The lesion is well-defined with no edema and mild mass effect. (B) Axial T1-weighted post
gadolinium image shows patchy enhancement within the lesion.

Imaging of primary brain tumors

80% of all oligodendrogliomas, are best demonstrated on CT;
however, gradient imaging can increase MRs sensitivity. Oligodendrogliomas tend to have higher relative CBVs on MR
perfusion studies compared to other low-grade glial tumors
that is related to a dense capillary network rather than a
higher grade of tumor.13

Pleomorphic
Xanthroastrocytoma (WHO Grade II)
Pleomorphic xanthroastrocytoma (PXA) is a distinct type of
circumscribed astrocytic tumor noted for cellular pleomorphism and xanthomatous change. Although usually classified
as WHO grade 2 tumors, they can undergo malignant transformation. PXAs are slow growing lesions that typically
present in the first 2 decades of life. They are usually located

101
in the cerebral hemispheres with a higher incidence in the
temporal lobes. Recurrence after resection is uncommon and
the survival rate at 10 years is 70%.
Imaging features of PXAs typically include a supratentorial
cortical cystic mass with an enhancing mural node. PXAs are
superficial lesions that involve the leptomeninges as well as
the brain parenchyma. As a result, a dural tail may be
present that can help differentiate these neoplasms from
other cortical tumors, such as DNET or ganglioglioma.36

Primary CNS Lymphoma

The vast majority of intracranial lymphomas are primary lesions that involve the brain parenchyma. More than 90% are
non-Hodgkin B-cell type. Secondary CNS lymphoma is rare

Figure 10 Primary central nervous system lymphoma in a 62-years-old immunocompetent man. (A) Axial T2-weighted,
(B) T2 FLAIR, and (C) post gadolinium images demonstrate an irregular, homogeneously enhancing mass in the
anterior genu of the corpus callosum extending into the frontal lobes. An additional lesion is noted in the splenium of
the corpus callosum. The lesions are mildly hyperintense on T2 weighted images and are associated with moderate
edema. The multiplicity of lesions, periventricular location, and homogeneous enhancement support the diagnosis of
lymphoma.

H. Morales and M. Gaskill-Shipley

102
and often presents with dural or leptomeningeal involvement.
Imaging features and clinical prognosis of primary CNS
lymphoma vary with the patients immune status. In immunocompetent patients primary lymphoma typically presents
as solitary or multiple predominantly solid masses in the
basal ganglia and white matter, often periventricular in location.37 These are highly cellular tumors that give them a characteristic hyperdensity on CT and homogeneous hypointensity on T2 with strong homogeneous enhancement38 (Fig.
10). Diffusion restriction may be present due to the dense
cellularity of the lesion.39 The administration of corticosteroids can modify or annul tumor enhancement, and therefore
should not be administered before initial CT and MR imaging
unless clinically necessary.
In immunodeficient patients, CNS lymphoma tends to
present as multifocal heterogeneous/peripheral enhancing lesions with central necrosis.40 These lesions may be indistinguishable from infectious etiologies including toxoplasmosis.
However, as in immunocompetent patients, lymphoma in
patients with acquired immunodeficiency syndrome (AIDS)
has a characteristic tendency to involve the ependymal surfaces and periventricular white matter.
The incidence of primary CNS lymphoma has tripled over
the past 2 decades, largely due to the increase in patients with
AIDS. However, the incidence of lymphoma is also rising in
the immunocompetent population.38 No environmental or
behavioral factors have been identified to account for this rate
increase.41 In general the prognosis for CNS lymphoma is
poor due to recurrent disease, however it is moderately better
in immunocompetent patients. The median survival time for
AIDS patients with intracranial lymphoma is only 2-6
months.

Hemangioblastoma (WHO Grade 1)

Hemangioblastomas are benign tumors of vascular etiology
that represent approximately 1%-2% of all primary CNS neo-

plasms. Although rare, hemanagioblastomas are the most

common primary adult intraaxial posterior fossa tumor.42
Over 85% of hemangioblastomas occur in the cerebellar
hemispheres. Less commonly they involve the vermis, medulla, and spinal cord; rare supratentorial lesions have been
reported. Males are more commonly affected than women,
and most patients present within the third to fifth decades of
life. Pediatric involvement is rare.
Most hemangioblastomas occur sporadically, however approximately 25% are associated with von Hippel-Lindau syndrome, an autosomal dominant disease which is also associated with retinal angiomatosis and visceral tumors involving
the kidneys and adrenal glands.43 Surgical resection is usually
curative, however patients with von Hippel-Lindau syndrome often have multiple lesions.
The most common imaging presentation of hemangioblastomas is a well-defined cyst of variable size with an intensely
enhancing mural nodule (Fig. 11). Approximately 25% of
lesions will present as a solid mass without a defined cyst.
Prominent flow voids may be present within the solid portions of the tumor due to the vascular nature of the tumor.
Uncommonly, hemangioblastomas can present as a cyst
without evidence of an enhancing nodule or wall.

Meningioma (WHO Grade 1-3)

Meningiomas arise from arachnoid meningothelial cells and
are the most common benign intracranial neoplasm in adults.
In general, these lesions are slow growing tumors (WHO
grade 1) with a good prognosis, however atypical and anaplastic meningiomas (WHO grade 23) do occur.44 Aggressive meningiomas can invade the brain parenchyma and will
rarely metastasize to distal sites.
The incidence of meningiomas is highest in middle-aged
women. Meningiomas may occur in multiples, particularly
when associated with neurofibromatosis type 2, and have
been associated with a history of radiation treatment. The

Figure 11 Hemangioblastoma in a 43-year-old man who presented with headaches. (A, B) Axial T2-weighted and post
gadolinium T1-weighted images demonstrate a large cystic mass with an enhancing mural nodule in the right cerebellum. Marked compression of the fourth ventricle is present.

Imaging of primary brain tumors

most common location for meningiomas is in the cerebral
convexities. Less common locations include the sphenoid
ridge, olfactory groove, parasellar region, cerebellopontine
angle, and optic nerves. Intraventricular lesions in the adult
population are rare but have a typical presentation in the atria
of the lateral ventricles (Fig. 12).
Meningiomas are most commonly present as extraaxial
masses with prominent homogeneous enhancement and
broad dural attachments. Although the MR signal characteristics of meningiomas on precontrast sequences can
vary, typically they are isointense to brain on T1 weighted
images and isotense to mildly hyperintense on T2
weighted images. This is due to the homogeneous cellularity of the tumor. Tumoral calcifications and hyperosto-

103
sis of the underlying skull bone are frequent. Edema is
commonly observed in the adjacent brain parenchyma
particularly with masses over the cerebral hemispheres.
The cause of the edema is not fully understood and may be
related to mechanical compression or secretions from the
tumor, however there is no direct correlation between the
presence of edema and the aggressiveness of the lesion or
brain invasion.45 Lesions that can mimic meningiomas include dural metastases, lymphoma, and hemangiopericytomas.
Meningiomas in the pediatric population are rare, however
when they occur they often have distinct characteristics.
Childhood meningiomas have a male predilection, are more
commonly intraventricular in location compared to adults

Figure 12 Intraventricular meningioma in a 46-year-old woman with headaches. (A) Precontrast axial T2 and (B) T1
weighted images demonstrate an intraventricular mass within the atrium of the right lateral ventricle. The lesion is
hyperintense on the T2 weighted image and isointense on T1. (C) Post gadolinium T1-weighted image shows marked,
homogeneous enhancement of the mass. The signal characteristics, location, and enhancement pattern are typical for
an intraventricular meningioma.

H. Morales and M. Gaskill-Shipley

104
and usually do not have a dural tail.46 These tumors also have
a tendency to malignant transformation.

(Fig. 13). Calcifications and cystic changes are common.48

Solid portions of the tumors demonstrate variable and irregular enhancement.

Central Neurocytoma (WHO Grade 2)

Central neurocytoma is an intraventricular neuronal cell tumor
that typically arises from the septum pellucidum or ventricular
wall. Most lesions are present in the lateral ventricles, however
third ventricular involvement does occur. Histologically, central
neurocytomas resemble oligodendrogliomas, and until recently
were considered intraventricular oligodendrogliomas. Neuronal characteristics observed on electron microscopy led to the
reclassification of this lesion in 1982.47
Central neurocytomas occur in young adults and often
present with signs of increased intracranial pressure or hydrocephalus due to their intraventricular location. The lesion
typically has a benign course, however more aggressive behavior including invasion of the brain and seeding of the CSF
can occur. The survival rate is approximately 81% at 5 years.
On imaging, central neurocytomas are present as heterogeneous masses often attached to the septum pellucidum

Subependymoma (WHO Grade 1)

Subependymoma is an uncommon, benign intraventricular tumor that typically occurs in middle-aged to older
adults. The most common location for this tumor is the
inferior fourth ventricle followed by the frontal horns of
the lateral ventricles attached to the septum pellucidum.
Subependymomas are usually asymptomatic, however obstructive hydrocephalus can occur due to the lesions intraventricular location.49 Patients with subependymomas
have an excellent prognosis; surgical resection is curative
in most cases.
Imaging features of subependymomas on CT include a
hypodense lobular mass within the fourth or lateral ventricles. On MR these lesions are usually hypo-to isointense on
T1 weighted images and hyperintense on T2 weighted sequences. Enhancement is typically absent (Fig. 14).

Figure 13 Central neurocytoma in a 39-year-old man with signs of increased intracranial pressure. (A) Noncontrast CT
scan demonstrates a large heterogeneous, mildly hyperdense intraventricular mass with involvement of the septum
pellucidum. (B) T2-weighted sagittal image shows prominent heterogeneity of the lesion with multiple small cysts.
(C) T1-weighted post gadolinium coronal image demonstrates patchy enhancement. Note the lateral ventricular
dilatation caused by obstruction at the foramen of Monro.

Imaging of primary brain tumors

105

Figure 14 Subependymoma in an asymptomatic 33-year-old woman. (A) Axial T2 FLAIR image shows a small, welldefined homogeneous intraventricular mass arising from the septum pellucidum. (B) T1-weighted post gadolinium
coronal image demonstrates no enhancement. The location and lack of enhancement are typical for this benign lesion.

Conclusion
Imaging is a vital step in the initial work-up of brain tumors.
An appropriate differential diagnosis should be based not
only on the radiographic characteristics of the tumor but also
on the age of the patient, location of the lesion, and the
tumors relative frequency. Advanced imaging techniques often can provide complementary information; however, conventional CT and MRI remain the primary tools for neurooncological diagnosis and early detection.

References
1. Thoman WJ, Ammirati M, Caragine LP Jr, et al: Brain tumor imaging
and surgical management: the neurosurgeons perspective. Top Magn
Reson Imaging 17:121-126, 2006
2. Newton HB, Ray-Chaudhury A, Cavaliere R: Brain tumor imaging and
cancer management: the neuro-oncologists perspective. Top Magn Reson Imaging 17:127-136, 2006
3. Jacobs AH, Kracht LW, Gossmann A, et al: Imaging in neurooncology.
NeuroRx 2:333-347, 2005
4. Louis DN, Ohgaki H, Wiestler OD, et al (eds): WHO Classification of
Tumours of the Central Nervous System. Lyon, France, IARC, 2007
5. Louis DN, Ohgaki H, Wiestler OD, et al: The 2007 WHO classification
of tumours of the central nervous system. Acta Neuropathol 114:97109, 2007
6. CBTRUS: Statistical report: primary brain tumors in the United States,
2000-2004. Hinsdale, IL, Central Brain Tumor Registry of the United
States2008. Available at: http://www.cbtrus.org/reports/reports.html
7. Dean BL, Drayer BP, Bird CR, et al: Gliomas: classification with MR
imaging. Radiology 174:411-415, 1990
8. Fayed N, Morales H, Modrego PJ, et al: Contrast/noise ratio on conventional MRI and choline/creatine ratio on proton MRI spectroscopy accurately discriminate low-grade from high-grade cerebral gliomas.
Acad Radiol 13:728-737, 2006
9. Law M, Yang S, Wang H, et al: Glioma grading: sensitivity, specificity,
and predictive values of perfusion MR imaging and proton MR spectroscopic imaging compared with conventional MR imaging. Am J Neuroradiol 24:1989-1998, 2003
10. Ball WS Jr, Holland SK: Perfusion imaging in the pediatric patient.
Magn Reson Imaging Clin N Am 9:207-230:ix, 2001

11. Hwang JH, Egnaczyk GF, Ballard E, et al: Proton MR spectroscopic

characteristics of pediatric pilocytic astrocytomas. Am J Neuroradiol
19:535-540, 1998
12. Cha S, Tihan T, Crawford F, et al: Differentiation of low-grade oligodendrogliomas from low-grade astrocytomas by using quantitative
blood-volume measurements derived from dynamic susceptibility contrast-enhanced MR imaging. Am J Neuroradiol 26:266-273, 2005
13. Lev MH, Ozsunar Y, Henson JW, et al: Glial tumor grading and outcome prediction using dynamic spin-echo MR susceptibility mapping
compared with conventional contrast-enhanced MR: confounding effect of elevated rCBV of oligodendrogliomas [corrected]. Am J Neuroradiol 25:214-221, 2004
14. Poussaint TY: Diagnostic imaging of primary pediatric brain tumors, in
Hodler DJ, et al (eds): Diseases of the Brain, Head and Neck, Spine
Diagnostic Imaging and Interventional Techniques 40th International
Diagnostic Course in Davos (IDKD). Italy, Springer, 2008
15. Koeller KK, Sandberg GD: From the archives of the AFIP. Cerebral
intraventricular neoplasms: radiologic-pathologic correlation. Radiographics 22:1473-1505, 2002
16. Barkovich AJ: Diagnostic Imaging (1st ed). Salt Lake City, Utah, Amirsys, 2007
17. Panigrahy A, Krieger MD, Gonzalez-Gomez I, et al: Quantitative short
echo time 1H-MR spectroscopy of untreated pediatric brain tumors:
preoperative diagnosis and characterization. Am J Neuroradiol 27:560572, 2006
18. Maleci A, Cervoni L, Delfini R: Medulloblastoma in children and in
adults: a comparative study. Acta Neurochir 119:62-67, 1992
19. Pramanik P, Sharma MC, Mukhopadhyay P, et al: A comparative study of
classical vs. desmoplastic medulloblastomas. Neurol India 51:27-34, 2003
20. Koetsenas AL, Roth TC, Manness WK, et al: Abnormal diffusionweighted MRI in medulloblastoma: does it reflect small cell histology?
Pediatr Radiol 29:524-526, 1999
21. Figeroa RE, el Gammal T, Brooks BS, et al: MR findings in primitive
neuroectodermal tumors. J Comput Assist Tomogr 13:773-778, 1989
22. Kleihues P, Cavenee WK: World Health Organization Classification of
Tumours: Pathology and Genetics of Tumours of the Nervous System.
Lyon, France, IARC Press, 2000
23. Chen CJ, Tseng YC, Hsu HL, et al: Imaging predictors of intracranial
ependymomas. J Comput Assist Tomogr 28:407-413, 2004
24. Poissaint TY: Pediatric brain tumors, in Newton HB and Jolsz FA (eds):
Handbook of Neuro-Oncology Neuroimaging. Canada, Elsevier, 2008
25. Dumas-Duport C, Scheithauer BW, Chodkiewicz JP, et al: Dysembryoplastic neuroepithelial tumor: a surgically curable tumor of young

H. Morales and M. Gaskill-Shipley

106

26.

27.
28.
29.

30.

31.
32.

33.
34.

35.

36.

37.

patients with intractable seizures. Report of thirty-nine cases.

Neurosurgery 23:545-556, 1988
Parmar HA, Hawkins C, Ozelame R, et al: Fluid-attenuated inversion
recovery ring sign as a marker of dysembryoplastic neuroepithelial
tumors. J Comput Assist Tomogr 31:348-353, 2007
Kurian NI, Nair S, Radhakrishnan VV: Anaplastic ganglioglioma: case
report and review of the literature. Br J Neurosurg 12:277-280, 1998
Littman P, Jarret P, Bilaniuk L, et al: Pediatric brainstem gliomas. Cancer 45:2787-2792, 1980
Kleihues P, Davis RL, Ohgaki H, et al: Diffuse astrocytoma; in Kleihues
P and Cavanee (eds): Pathology and Genetics of Tumours of the Nervous System. Lyon, France, International Agency for Research on Cancer, 2000, pp 22-26
Paris JE, Scheithauer BW: Glial tumors, in Nelson JS, Parisi JE,
Scheithauer BW (eds): Principles and Practice of Neuropathology. St.
Louis, MO, Mosby, 1993, pp 123-183
Patel MR, Tse V: Diagnosis and staging of brain tumors. Semin Roentgenol 39:347-360, 2004
Tovi M, Lilja A, Erickson A: MR imaging in cerebral gliomas: tissue
compoent analysis in correlation with histopathology of whole-brain
specimens. Acta Radiol 35:495-505, 2002
Engelhard HH, Stelea A, Cochran EJ: Oligodendroglioma: pathology
and molecular biology. Surg Neurol 58:111-117, 2002
Daumas-Duport C, Varlet P, Tucker ML, et al: Oligodendrogliomas.
part 1: pattern of growth, histological diagnosis, clinical and imaging
correlations: a study of 153 cases. J Neuro Oncol 34:37-59, 1997
Ankenbrandt W, Paleologos N: Imaging of oligodendrogliomas, in
Newton HB and Jolsz FA (eds): Handbook of Neuro-Oncology Neuroimaging. Canada, Elsevier, 2008
Pierallini A, Bonamini M, Di Stefano D, et al: Pleomorphic xanthoastrocytoma with CT and MRI appearance of meningioma. Neuroradiology 41:30-34, 1999
Coulon A, Lafitte F, Hoang-Xuan K, et al: Radiographic findings in 37
cases of primary CNS lymphoma in immunocompetent patients. Eur
Radiol 12:329-340, 2002

38. Koeller KK, Smirniotopoulos JG, Jones RV: Primary central nervous
system lymphoma: radiologic-pathologic correlation. Radiographics
17:1497-1526, 1997
39. Cotton F, Ongolo-Zogo P, Louis-Tisserand G, et al: Diffusion and perfusion MR imaging in cerebral lymphomas. J Neuroradiol 3:220-228,
2006
40. Johnson BA, Fram EK, Johnson PC, et al: The variable MR appearance
of primary lymphoma of the central nervous system: comparison with
histopathologic features. Am J Neuroradiol 18:563-572, 1997
41. Knopp E, Montanera W: Brain tumors, in Hodler DJ, et al (eds): Diseases of the Brain, Head and Neck, SpineDiagnostic Imaging and
Interventional Techniques 40th International Diagnostic Course in Davos (IDKD). Italy, Springer, 2008
42. Constans JP, Meder F, Maiuri F, et al: Posterior fossa hemangioblastomas. Surg Neurol 25:269-275, 1986
43. Bradley S, Dumas N, Ludman M, et al: Hereditary renal cell carcinomas
associated with von HippelLindau disease: a description of a Nova
Scotia cohort. Can Urol Assoc J 3:32-36, 2009
44. Louis DN, Budka H, von Deimling A: Meningiomas, in Kleihues P,
Cavenee WK (eds): Pathology and Genetics of Tumours of the Nervous
System. Lyon, France, International Agency for Research on Cancer, pp
134-141
45. Buetow MP, Buetow PC, Smirniotopoulos JG: Typical, atypical, and
misleading features in meningioma. Radiographics 11:1087-1106,
1991
46. Caroli E, Russillo M, Ferrante L: Intracranial meningiomas in children:
report of 27 new cases and critical analysis of 440 cases reported in the
literature. J Child Neurol 21:31-36, 2006
47. Hassoun J, Gambarelli D, Grisoli F, et al: Central neurocytoma. An
electron-microscopic study of two cases. Acta Neuropathol 56:151156, 1999
48. Goergen SK, Gonzales MF, McLean CA: Interventricular neurocytoma:
radiologic features and review of the literature. Radiology 182:787792, 1992
49. Scheithauer BW: Symptomatic subependymoma: report of 21 cases
with review of the literature. J Neurosurg 49:689-696, 1978