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Imaging of Common Adult

and Pediatric Primary Brain Tumors


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

T he primary goals of brain tumor imaging are lesion de-


tection, localization, delineation of extent, and charac-
terization. This information is used to formulate an appropri-
that has a 90% 5-year survival after resection whereas glio-
blastoma multiforme (GBM) represents one of the most com-
mon WHO grade 4 tumors with a 4% or less survival at 5
ate differential diagnosis that is extremely helpful for the years.6
referring neuro-surgeons and the neuro-oncologists.1,2 In ad- Although the final diagnosis and grading of brain tumors is
dition, imaging studies play a vital role in therapy planning, determined by histologic analysis, imaging can be very help-
such as stereotactic location for surgery or radiotherapy, and ful in the initial assessment of neoplasms and can, in many
assessing response to therapy.3 cases, help direct surgical biopsy or treatment. Imaging fea-
This article discusses the imaging characteristics of several tures associated with increasing malignancy include mass
common pediatric and adult primary central nervous system effect, vasogenic edema, enhancement, necrosis, and hemor-
(CNS) tumors. The review will focus on the initial imaging rhage, particularly when astrocytic tumors are compared.7,8
diagnostic workup and will give a useful radiological ap- Cerebral blood volume (CBV), which can be calculated from
proach based on age, localization, imaging characteristics, MR perfusion studies and choline/creatine (Cho/Cr) ratios
and relative frequency of brain tumors. Advanced magnetic based on MR spectroscopy, are relatively elevated in malig-
resonance (MR) techniques including spectroscopy and per- nant tumors, secondary to increased vascularity and cell pro-
fusion can provide additional information of brain tumors. liferation, respectively.8,9 However it is important to note that
These techniques will be discussed separately in this supple- some low-grade tumors can demonstrate “aggressive fea-
ment. tures” on imaging. In children, PAs may have a malignant
appearance on conventional imaging, increased metabolism
on positron emission tomographic images, and increased
Classification rCBV and Cho/Cr ratios.10,11 In adults, oligodendrogliomas
The World Health Organization (WHO) classifies primary often have increased rCBV.12,13
brain tumors according to their cellular origin. The major
categories include neuroepithelial tumors, tumors of the me-
ninges, lymphoma and hematopoietic neoplasms, germ cell Diagnostic Approach to
tumors, tumors of the cranial and paraspinal nerves, tumors Intracranial Tumors Based
of the sellar region, metastatic tumors, and cysts and tumor-
like lesions.4,5 Tumors of neuroepithelial origin comprise a sig-
on Frequency, Age, Location,
nificant number of primary brain tumors, including astrocyto- and Imaging Characteristics
mas, oligodendrogliomas, ependymomas, choroid plexus The radiologist’s ultimate goal in the initial imaging evalu-
tumors, neuronal and mixed neuronal-glial tumors, pineal le- ation of brain tumors should be to provide an appropriate
sions, and embryonal tumors. differential diagnosis that will guide future intervention
Grading of brain tumors is according to the WHO classi- and treatment. Accurate diagnosis is based not only on the
fication that assigns a grade of 1-4 from benign to malignant, radiological appearance of the tumor but also on the pa-
taking into account the presence of nuclear changes, mitotic tient’s age and the location and relative incidence of the
activity, endothelial proliferation, and necrosis. The proto- lesion (Table 1).14 Each of these factors must be taken into
typic WHO grade 1 tumor is the pilocytic astrocytoma (PA) account.
The yearly incidence of primary brain tumors is 16.5 cases
per 100,000 population or approximately 30,000-35,000
Division of Neuroradiology, University Hospital, Cincinnati, OH.
Address reprint requests to Humberto Morales, MD, Division of Neuroradi- new cases per year. In comparison, intracranial metastases
ology, University Hospital, 231 Albert Sabin Way, Cincinnati, OH are much more common with as many as 170,000 cases
45267. E-mail: moralehc@ucmail.uc.edu reported each year. The overall incidence of primary tumors

92 0037-198X/10/$-see front matter © 2010 Elsevier Inc. All rights reserved.


doi:10.1053/j.ro.2009.09.006
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 Extraaxial
Rare in pediatric population: schwannoma and meningioma. Convexities
Meningioma, hemangioendothelioma, and
hemangioperycitoma.
Cerebello-pontine angle
Schwannoma, meningioma, and epidermoid.
Intraaxial Intraaxial
Supratentorial Supratentorial
Cortical Cortical
Neuronal and mixed neural-glial (DNET, ganglioglioma) Pleomorphic xanthoastrocytoma.
and pleomorphic xanthoastrocytoma. Corticomedullary
Corticomedullary Oligodendroglioma and fibrillary/anaplastic
Low-grade astrocytoma and S-PNET. astrocytoma
Deep white matter Deep white matter
Fibrillary/anaplastic astrocytoma, supratentorial Glioblastoma and fibrillary/anaplastic
ependymoma, and glioblastoma. astrocytoma
Corpus callosum/periventricular white matter
Glioblastoma, lymphoma, and gliomatosis
cerebri.
Infratentorial Infratentorial
Cerebellar hemisphere Cerebellar hemisphere
Pilocytic astrocytoma (PA) and ATRT. Hemangioblastoma and astrocytoma.
Intraventricular Intraventricular
Fourth ventricle Fourth ventricle
Medulloblastoma/PNET-MB and ependymoma. Subependymoma, choroid plexus papilloma, and
ependymoma.
Lateral ventricle Lateral ventricle
Choroid plexus papilloma, SEGA, meningioma, ependymoma, Meningioma, ependymoma, central neurocytoma,
and choroid plexus carcinoma. and subependymoma.
Third ventricle
Colloid cyst, astrocytoma, and chordoid glioma.
Midline Midline
Suprasellar-chiasmatic-optic Sellar/suprasellar
Craniopharyngioma, PA, germinoma, LCH, hypothalamic Pituitary adenoma, meningioma, and
hamartoma, lipoma, dermoid, and PMA. craniopharyngioma.
Pineal region Pineal region
Germinoma, teratoma, and pineoblastoma. Pineal cyst, pineocytoma, and
Brainstem epidermoid/dermoid.
Tectal glioma, focal/diffuse pontine, and medullary and
midbrain astrocytomas.
Multiple spaces Multiple spaces
Embryonal tumors (PNET, ependymoblastoma, and Malignant meningioma, hemangiopericytoma, and
neuroblastoma), ATRT. lymphoma.
Abbreviations: DNET, dysembryoplastic neuroepithelial tumor; S-PNET, supratentorial primitive neuroectodermal tumor; ATRT, atypical teratoid
rhabdoid tumor; SEGA, subependymal giant cell astrocytoma; LCH, Langerhan’s cell histiocytosis; PMA, pilomyxoid astrocytoma.

increases with age. The pediatric population (0-19 years) has the adult population, a solitary lesion in the posterior fossa is
a low incidence rate of approximately 4.5 cases per 100,000 most commonly a metastasis.
population/year while the highest occurrence rate is observed Differentiating between an intra- and extraaxial location of
in patients older than 50 years of age, accounting for over 30 a mass will significantly alter potential diagnoses. Although
cases per 100,000 population/year.6 extraaxial primary tumors, such as meningiomas, are com-
The location and histology of primary brain tumors vary mon in adults, they are very uncommon in the pediatric
with age. Although the most common location for primary population. Intraventricular tumors have a very specific dif-
neoplasms in both adults and children is the cerebral hemi- ferential diagnosis, which can vary depending on which ven-
spheres; brain tumors in children have a special predilection tricle is involved. If a tumor is intraaxial, determination of its
for the posterior fossa. In comparison, primary tumors lo- specific location such as cortex, corticomedullary junction,
cated in the cerebellum are unusual in adults. Therefore, in or white matter lesion will alter possible diagnoses.
94 H. Morales and M. Gaskill-Shipley

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 vari- common pediatric fourth ventricular tumors, medulloblas-
ous ages (Fig. 1). The most common tumor of childhood is toma (MB), and ependymoma (Fig. 2).15,16
the PA, followed by medulloblastoma. However, in adult- PA’s occurring in the optic nerve/chiasm/hypothalamic re-
hood, the most common tumor is meningioma, followed by gion have been separated into 3 subsets. In the pediatric
glioblastoma. Oligodendroglioma, which is common in population, tumors are divided into 2 types: those associated
adults, is very rare in children. Neuronal and mixed neural- with neurofibromatosis type 1 (NF1) and those not associ-
glial tumors are more common in children than in adults ated with NF1. PA’s in patients with NF1 are usually bilateral
(Table 1). Therefore, correlating the location and character- nonenhancing tumors involving the optic nerves and less
istics of a tumor with the age of the patient is crucial in commonly, the optic chiasm/hypothalamic region. They
determining an appropriate differential diagnosis. have an indolent course. PA’s in non-NF1 patients usually
involve the chiasm/hypothalamic region. They are typically
solid/cystic enhancing tumors, which are often larger than
Tumors More PA’s associated with NF1 and have a less indolent course. The
third subset of tumors, which is identified in adults, demon-
Common in Childhood strates more aggressive behavior.
Pilocytic Astrocytoma (WHO Grade 1) As mentioned earlier, PA may demonstrate a false “aggres-
PA is the most common primary brain tumor in children. sive appearance” on MR spectroscopy with significant eleva-
Overall, patients with PA’s have a good prognosis after resec- tion of Cho/Cr ratios. Recent studies have shown very low
tion with a 94% survival rate at 10 years. Common locations concentrations of Cr in PA’s compared with other pediatric
for PA’s include the cerebellum, optic nerve/chiasm/hypotha- tumors, as well as diminished quantities of total Cho. There-
lamic region, or brain stem. Involvement of the cerebral fore, the paradoxical increase in Cho/Cr ratios does not nec-
essarily indicate increased cell proliferation.17
hemispheres is less frequent.
Pilocytic astrocytomas are typically well-defined lesions
that classically present as a cystic mass with an enhancing Medulloblastoma/
mural nodule, however solid lesions do occur. Significant PNET-MB) (WHO Grade 4)
vasogenic edema is rare. These are slow growing tumors that MB or PNET-MB (primitive neuroectodermal tumor-medul-
often present due to localized mass effect. Lesions occurring loblastoma) is a highly cellular, rapidly growing malignant
in the cerebellum may significantly compress the fourth ven- embryonal tumor. It is the second most common tumor in
tricle, making it difficult to identify the tumor’s parenchymal children but the most common posterior fossa lesion. These
origin. The main differential diagnosis is between the 2 most 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) Medullo-
blastoma 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 Children’s Hospital).

from the superior medullary velum. Involvement of the cer- of these lesions are dense on computed tomography (CT),
ebellar hemispheres is rare, usually occurring in older chil- which can aid in the radiographic diagnosis (Fig. 2). Approx-
dren and adults and typically representing desmoplastic me- imately one-third of MB’s will have subarachnoid dissemina-
dulloblastomas.18 MBs have a variable prognosis after resection tion at the time of presentation; therefore, imaging the entire
with a 50% survival rate at 10 years. neuroaxis is extremely important to evaluate the extent of
MBs are typically isointense on T1 weighted images, disease.
mildly hypointense to cortex on T2 weighted images, and Supratentorial primitive neuroectodermal tumors (S-PNET)
homogeneously enhance. The desmoplastic variant that has a are a subset of tumors genetically distinct from infratento-
better prognosis often demonstrates a different imaging pat- rial PNETs (PNET-MB). Though only 6% of PNETs are
tern characterized by calcifications and subtotal heteroge- supratentorial, they are important hemispheric masses to
neous enhancement.19 Diffusion restriction is commonly ob- consider in newborns and infants. S-PNETs are large, com-
served due to the tumor’s dense cellularity.20 Ninety percent plex hemispheric masses with heterogeneous enhancement
96 H. Morales and M. Gaskill-Shipley

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 Children’s Hospital).

and minimal peritumoral edema.21 The differential diagnosis mal cells lining the ventricles and are most commonly located in
of these lesions includes teratoid or rhabdoid tumors and the fourth ventricle. Unlike medulloblastomas, which originate
ependymomas. Unlike PNET-MB, calcifications, hemor- superiorly from the medullary velum, ependymomas arise from
rhage, and necrosis are common (Fig. 3). Patients with the floor of the fourth ventricle (Fig. 2). One-third of ependy-
S-PNET have decreased survival compared to those with momas are located supratentorially where they have a
PNET-MB. characteristic deep parietal white matter location rather
than intraventricular. They are slow growing tumors with an
Ependymoma (WHO Grade 2) overall 60%-70% survival rate at 5 years after resection.
Ependymomas are the fourth most common posterior fossa Infratentorial ependymomas typically preset as heteroge-
tumor in the pediatric population.22 They arise from ependy- neous enhancing lesions that often extend out through the
Imaging of primary brain tumors 97

foramina of Luschka and Magendie into the cisterns. Punc- Neuronal and Mixed
tate calcifications occur in approximately 50% of cases, Neural-Glial Tumors (WHO Grade 1-2)
therefore CT may be helpful in differentiating this tumor
Dysembryoplastic neuroepithelial tumors (DNET) and gan-
from MB and PA (Fig. 2).23 Ependymomas may also dem-
gliogliomas/gangliocytomas are the most common mixed
onstrate cystic transformation, hemorrhage, necrosis, and
neural-glial tumors in children and young adults. These le-
edema.
sions are cortical tumors often associated with refractory sei-
Choroid Plexus Papilloma/ zures.
DNET is a benign mixed neural-glial tumor frequently
Carcinoma (WHO Grade 1 and 4) associated with a background of cortical dysplasia.25 They are
Choroid plexus papillomas (CPP) are intraventricular papil- most commonly found in the temporal lobes; however, they
lary neoplasms that are derived from the choroid plexus ep- can arise in other areas of the cerebral hemispheres and pos-
ithelium. CPP is one of the most common brain tumors in
terior fossa. On magnetic resonance imaging (MRI), DNETs
children below the age of 2 years.24 In the pediatric popula-
typically have a “bubbly” or multicystic appearance. Most
tion they are located in the atrium of the lateral ventricle. In
lesions are well defined with no edema and little mass effect.
contrast, tumors in adults are typically located in the fourth
Enhancement is present in up to 50% of cases and is usually
ventricle and cerebellopontine angles. CPP is a slowly grow-
nodular or ringlike in appearance. Calcification may be
ing tumor with a 5-year survival rate close to 100%.
present. A “FLAIR (fluid attenuated inversion recovery)
Imaging features include a frond-like lobulated mass with
intense enhancement. Calcifications and hemorrhage are fre- bright ring” sign has been described with these tumors,
quently present. Hydrocephalus is very commonly associated which is a complete or incomplete hyperintense rim that may
with CPP’s and can be due to either overproduction or ob- be caused by the presence of loose peripheral neuroglial ele-
struction of cerebrospinal fluid (CSF). ments (Fig. 4). This sign may help differentiate these tumors
Choroid plexus carcinomas (CPC) are malignant neo- from other cortical lesions.26
plasms that account for approximately 20%-30% of all cho- Gangliogliomas (WHO grade 1 or 2) and gangliocytomas
roid plexus masses. Most cases occur in children and also (WHO grade 1) are also cortically based neoplasms. Ganglio-
typically arise in the lateral ventricles. CPC’s have a poor gliomas are composed of both benign mixed atypical gan-
prognosis with a 50% survival rate at 5 years. Imaging find- glion cells and neoplastic glial cells, whereas gangliocytomas
ings that can help differentiate CPP from carcinomas include are primarily composed of dysplastic or neoplastic neurons.
extension of the lesion beyond the ventricle wall, prominent Although both lesions are usually benign, rare cases of ma-
vasogenic edema, and mass effect.23 Both CPP’s and CPC’s lignant degeneration or metastatic spread have been repor-
can seed the CSF, however metastatic spread is more com- ted.27 These lesions can be either solid, cystic, or mixed.15
monly observed with carcinomas. 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 inver-
sion 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 Children’s Hospital).
98 H. Morales and M. Gaskill-Shipley

nodule. Most lesions demonstrate either solid or heteroge- filtrative subset includes PAs, pleomorphic xanthoastrocyto-
neous enhancement. Mass effect and edema are uncommon. mas, subependymal giant cell astrocytomas, and desmoplas-
tic cerebral astrocytomas of childhood. This section will
Brainstem Tumors (WHO Grade 1-4) primarily focus on the infiltrative type.
Most brainstem tumors are gliomas, including fibrillary as- Infiltrative astrocytomas range from low-grade lesions to
trocytomas, PAs, and rarely glioblastomas. Eighty percent of highly aggressive malignant neoplasms. Grading these tu-
all brainstem gliomas occur in the pediatric population. They mors is based on several histopathologic features including
are categorized according to where they develop (pontine, cellularity, nuclear atypia, mitotic activity, endothelial prolif-
medullary, and midbrain) and whether they are diffuse or eration, and necrosis. As described previously several imag-
focal. Prognosis varies greatly depending on the location and ing features including mass effect, edema, enhancement, and
grade of the lesion. necrosis can help predict tumor grade.
Pontine gliomas are more often diffuse lesions that may Low grade astrocytomas (WHO grade 2) comprise approx-
present with cranial nerve palsies and ataxia.28 These lesions imately 25% of all gliomas.29 They tend to occur in younger
are often high-grade and have a poor long-term prognosis. adults, often in the third and fourth decades of life. Low grade
Imaging features include expansion of the brain stem with gliomas are best visualized on MRI and typically present as
abnormal T2 prolongation. Enhancement is usually absent. nonenhancing well demarcated or ill-defined lesions in the
Tectal gliomas typically have a distinctly benign behavior cerebral hemispheres (Fig. 5). Edema is usually absent. Typ-
compared to other brainstem tumors. Some authors believe ically there is absent or minimal mass effect, a finding which
that these tumors are hamartomas; however, other series can be striking given the size of some lesions.
have histologically shown these tumors to be low-grade PAs. Anaplastic astrocytomas (AA) (WHO grade 3) occur in a
Due to their location adjacent to the sylvian aqueduct, these slightly older population and confer a worse prognosis. The
lesions often present with obstructive hydrocephalus. They median survival rate for these patients is 2-3 years. Most AA’s
usually do not require treatment beyond CSF diversion such result from dedifferentiation of low grade gliomas, however
as third ventriculostomy or shunting.24
some arise de novo.30 Histologically, AA contains gemisto-
cytes and protoplasmic elements but no necrosis. Dissemina-
Tumors More tion occurs through the white matter tracts. AA’s typically
Common in Adulthood demonstrate edema and mass effect that helps to differentiate
them from low grade gliomas (Fig. 6). Approximately two-
Astrocytomas thirds of AA’s will partially enhance.
Gliomas are the most common primary intraaxial mass in GBM (WHO grade 4) is the most aggressive and malignant
adult population. Astrocytomas account for over half of all form of astrocytoma and is characterized histologically by
gliomas and can be divided into 2 major categories, infiltra- necrosis and neovascularity. It is the second most common
tive and noninfiltrative. Infiltrative astrocytomas are much tumor in adults after meningioma and usually occurs in pa-
more common, representing 75% of all lesions. The nonin- tients 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 differen-
tiate 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 prog- Hemorrhage of differing stages is often observed. Involve-
nosis among primary brain tumors. The median postopera- ment of the bilateral hemispheres through the corpus callo-
tive survival time is 8 months, and the overall 10-year sur- sum (“butterfly glioma”) is a classic presentation.
vival rate in patients older than 45 of age is less than 1.8%. Surgical resection of GBM’s is nearly always incomplete
GBM’s are most commonly located in the supratentorial due to the highly infiltrative nature of the tumor. Biopsies
white matter. Involvement of the brainstem and cerebellum taken from around the margins of resection cavities have
is rare; however, these locations are more common in chil- demonstrated tumor cells in the surrounding “edema” and
dren than adults (Fig. 7). On MRI, GBM’s are usually identi- even in normal appearing white matter.32 Surgical resec-
fied as large heterogeneously enhancing masses with signifi- tion and radiation are frequently targeted to the enhancing
cant necrosis, mass effect, and vasogenic edema (Fig. 8). (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.
100 H. Morales and M. Gaskill-Shipley

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 Many oligodendrogliomas demonstrate 1p and/or 19q
therapy. chromosomal deletions.33 The presence of these chromo-
some losses increases the tumor’s response to radiation and
Oligodendroglioma (WHO Grade 2) chemotherapy and improves prognosis. Patients with both
Oligodendrogliomas are infiltrative gliomas that originate from 1p and 19q deletions have a median survival time of 8-10
oligodendroglial cells. They occur in middle-aged adults and years whereas patient without the deletions have a survival
often present with seizures. Pediatric involvement is rare. Oli- time of 3-4 years.
godendrogliomas may be low grade (WHO grade 2) or anaplas- Differentiating oligodendrogliomas from astrocytomas on
tic (WHO grade 3). Similar to astrocytomas, higher grade conventional imaging is often not possible. However, several
oligodendrogliomas demonstrate increased cellularity, imaging features favor the diagnosis of oligodendroglioma,
mitotic activity, endothelial hyperplasia, and necrosis. Mixed including cortical involvement, heterogeneous signal, intra-
oligodendrogliomas contain both features of astrocytomas and tumoral cysts, patchy enhancement, and the presence of cal-
oligodendrogliomas, and can be classified as grade 2 or 3. cifications34,35 (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 101

80% of all oligodendrogliomas, are best demonstrated on CT; in the cerebral hemispheres with a higher incidence in the
however, gradient imaging can increase MR’s sensitivity. Ol- temporal lobes. Recurrence after resection is uncommon and
igodendrogliomas tend to have higher relative CBVs on MR the survival rate at 10 years is 70%.
perfusion studies compared to other low-grade glial tumors Imaging features of PXAs typically include a supratentorial
that is related to a dense capillary network rather than a cortical cystic mass with an enhancing mural node. PXAs are
higher grade of tumor.13 superficial lesions that involve the leptomeninges as well as
the brain parenchyma. As a result, a “dural tail” may be
Pleomorphic present that can help differentiate these neoplasms from
Xanthroastrocytoma (WHO Grade II) other cortical tumors, such as DNET or ganglioglioma.36
Pleomorphic xanthroastrocytoma (PXA) is a distinct type of
circumscribed astrocytic tumor noted for cellular pleomor-
phism and xanthomatous change. Although usually classified Primary CNS Lymphoma
as WHO grade 2 tumors, they can undergo malignant trans- The vast majority of intracranial lymphomas are primary le-
formation. PXAs are slow growing lesions that typically sions that involve the brain parenchyma. More than 90% are
present in the first 2 decades of life. They are usually located 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.
102 H. Morales and M. Gaskill-Shipley

and often presents with dural or leptomeningeal involve- plasms. Although rare, hemanagioblastomas are the most
ment. common primary adult intraaxial posterior fossa tumor.42
Imaging features and clinical prognosis of primary CNS Over 85% of hemangioblastomas occur in the cerebellar
lymphoma vary with the patient’s immune status. In immu- hemispheres. Less commonly they involve the vermis, me-
nocompetent patients primary lymphoma typically presents dulla, and spinal cord; rare supratentorial lesions have been
as solitary or multiple predominantly solid masses in the reported. Males are more commonly affected than women,
basal ganglia and white matter, often periventricular in loca- and most patients present within the third to fifth decades of
tion.37 These are highly cellular tumors that give them a char- life. Pediatric involvement is rare.
acteristic hyperdensity on CT and homogeneous hypointen- Most hemangioblastomas occur sporadically, however ap-
sity on T2 with strong homogeneous enhancement38 (Fig. proximately 25% are associated with von Hippel-Lindau syn-
10). Diffusion restriction may be present due to the dense drome, an autosomal dominant disease which is also associ-
cellularity of the lesion.39 The administration of corticoste- ated with retinal angiomatosis and visceral tumors involving
roids can modify or annul tumor enhancement, and therefore the kidneys and adrenal glands.43 Surgical resection is usually
should not be administered before initial CT and MR imaging curative, however patients with von Hippel-Lindau syn-
unless clinically necessary. drome often have multiple lesions.
In immunodeficient patients, CNS lymphoma tends to The most common imaging presentation of hemangioblas-
present as multifocal heterogeneous/peripheral enhancing le- tomas is a well-defined cyst of variable size with an intensely
sions with central necrosis.40 These lesions may be indistin- enhancing mural nodule (Fig. 11). Approximately 25% of
guishable from infectious etiologies including toxoplasmosis. lesions will present as a solid mass without a defined cyst.
However, as in immunocompetent patients, lymphoma in Prominent flow voids may be present within the solid por-
patients with acquired immunodeficiency syndrome (AIDS) tions of the tumor due to the vascular nature of the tumor.
has a characteristic tendency to involve the ependymal sur- Uncommonly, hemangioblastomas can present as a cyst
faces and periventricular white matter. without evidence of an enhancing nodule or wall.
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 Meningioma (WHO Grade 1-3)
the immunocompetent population.38 No environmental or Meningiomas arise from arachnoid meningothelial cells and
behavioral factors have been identified to account for this rate are the most common benign intracranial neoplasm in adults.
increase.41 In general the prognosis for CNS lymphoma is In general, these lesions are slow growing tumors (WHO
poor due to recurrent disease, however it is moderately better grade 1) with a good prognosis, however atypical and ana-
in immunocompetent patients. The median survival time for plastic meningiomas (WHO grade 2–3) do occur.44 Aggres-
AIDS patients with intracranial lymphoma is only 2-6 sive meningiomas can invade the brain parenchyma and will
months. rarely metastasize to distal sites.
The incidence of meningiomas is highest in middle-aged
Hemangioblastoma (WHO Grade 1) women. Meningiomas may occur in multiples, particularly
Hemangioblastomas are benign tumors of vascular etiology when associated with neurofibromatosis type 2, and have
that represent approximately 1%-2% of all primary CNS neo- 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 cerebel-
lum. Marked compression of the fourth ventricle is present.
Imaging of primary brain tumors 103

most common location for meningiomas is in the cerebral sis of the underlying skull bone are frequent. Edema is
convexities. Less common locations include the sphenoid commonly observed in the adjacent brain parenchyma
ridge, olfactory groove, parasellar region, cerebellopontine particularly with masses over the cerebral hemispheres.
angle, and optic nerves. Intraventricular lesions in the adult The cause of the edema is not fully understood and may be
population are rare but have a typical presentation in the atria related to mechanical compression or secretions from the
of the lateral ventricles (Fig. 12). tumor, however there is no direct correlation between the
Meningiomas are most commonly present as extraaxial presence of edema and the aggressiveness of the lesion or
masses with prominent homogeneous enhancement and brain invasion.45 Lesions that can mimic meningiomas in-
broad dural attachments. Although the MR signal charac- clude dural metastases, lymphoma, and hemangiopericy-
teristics of meningiomas on precontrast sequences can tomas.
vary, typically they are isointense to brain on T1 weighted Meningiomas in the pediatric population are rare, however
images and isotense to mildly hyperintense on T2 when they occur they often have distinct characteristics.
weighted images. This is due to the homogeneous cellu- Childhood meningiomas have a male predilection, are more
larity of the tumor. Tumoral calcifications and hyperosto- 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.
104 H. Morales and M. Gaskill-Shipley

and usually do not have a dural tail.46 These tumors also have (Fig. 13). Calcifications and cystic changes are common.48
a tendency to malignant transformation. Solid portions of the tumors demonstrate variable and irreg-
ular enhancement.
Central Neurocytoma (WHO Grade 2)
Central neurocytoma is an intraventricular neuronal cell tumor Subependymoma (WHO Grade 1)
that typically arises from the septum pellucidum or ventricular Subependymoma is an uncommon, benign intraventricu-
wall. Most lesions are present in the lateral ventricles, however lar tumor that typically occurs in middle-aged to older
third ventricular involvement does occur. Histologically, central adults. The most common location for this tumor is the
neurocytomas resemble oligodendrogliomas, and until recently inferior fourth ventricle followed by the frontal horns of
were considered “intraventricular oligodendrogliomas.” Neuro- the lateral ventricles attached to the septum pellucidum.
nal characteristics observed on electron microscopy led to the Subependymomas are usually asymptomatic, however ob-
reclassification of this lesion in 1982.47 structive hydrocephalus can occur due to the lesion’s in-
Central neurocytomas occur in young adults and often traventricular location.49 Patients with subependymomas
present with signs of increased intracranial pressure or hy- have an excellent prognosis; surgical resection is curative
drocephalus due to their intraventricular location. The lesion in most cases.
typically has a benign course, however more aggressive be- Imaging features of subependymomas on CT include a
havior including invasion of the brain and seeding of the CSF hypodense lobular mass within the fourth or lateral ventri-
can occur. The survival rate is approximately 81% at 5 years. cles. On MR these lesions are usually hypo-to isointense on
On imaging, central neurocytomas are present as hetero- T1 weighted images and hyperintense on T2 weighted se-
geneous masses often attached to the septum pellucidum quences. 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, well-
defined 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.

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