Eur Radiol (2005) 15: 453–467

DOI 10.1007/s00330-004-2557-0 NEURO

Antonios Drevelegas
Extra-axial brain tumors

Received: 14 July 2004

Abstract Meningiomas, schwanno- Keywords Extra-axial . Brain
Revised: 14 October 2004 mas, metastases, maldevelopmental tumors . MRI . Meningioma .
Accepted: 15 October 2004 cysts, epidermoids, dermoids and Schwannoma
Published online: 31 December 2004 bone tumors represent the vast ma-
# Springer-Verlag 2004 jority of extra-axial brain tumors. The
location of extra-axial brain tumors
A. Drevelegas affects treatment planning and pre-
Radiology Department, Aristotele dicts their prognosis. Computed
University of Thessaloniki, tomography and particularly magnetic
Thessaloniki, Greece
resonance imaging are used for diag-
A. Drevelegas (*) nosis and localization. In this article,
25, Nikis Street, the imaging findings of the extra-axial
55236 Thessaloniki, Greece brain tumors are discussed.
e-mail: [email protected]
Tel.: +30-2310-343921
Fax: +30-2310-471056

Extra-axial tumors are the tumors of extracerebral loca- [1, 3, 4] and 20% [1, 5, 6]. They are encountered in
tion. They are usually benign. The location of brain tumors middle-aged adults and are more common in women than
affects treatment planning and predicts their prognosis. in men. Childhood meningiomas are uncommon and rep-
The multiplanar capability of MR imaging makes it the resent 1–4.2% of central nervous system tumors and 1.5–
best technique in the evaluation of extra-axial brain tumors. 1.8% of all intracranial meningiomas [7–11].
Meningiomas are the most common exta-axial neoplasms The presenting signs and symptoms of meningiomas,
in the supratentorial compartment. Other extra-axial neo- when neurofibromatosis is excluded, are related to the tumor
plasms are: schwannomas, metastatic lesions, arachnoid location and size, they are often non-specific and vague,
cysts, epidermoids, dermoids, chordomas and eosinophilic primarily related to brain compression and edema from the
granulomas. adjacent neoplasm [1, 12]. Signs of increased cranial pressure
(nausea, headache, vomiting) are found in 50% of the pa-
tients. Confusion, focal weakness and seizures are the most
Meningiomas common symptoms while paresis is the most frequently
found physical sign [1, 13, 14]. Headache has been reported
Meningiomas are vascular, non-glial tumors of the central as the most common single presenting symptom, found in
nervous system arising from meningothelial (arachnoidal) 36%, while normal physical examination has been reported
cells. They generally receive their blood supply from in 26% of patients [12].
branches of the external carotid arteries, although large The most common locations of meningiomas in de-
tumors will also recruit branches from the internal carotid scending order of frequency are: convexity meningiomas
arteries [1, 2]. Meningiomas are the most common non-glial 20–34%, parasagittal 18–22%, sphenoid and middle cra-
primary CNS tumors and the most common extra-axial nial fossa 17–25%, frontobasal 10% and posterior fossa
intracranial tumors. The relative frequency of meningio- 9–15%.
mas regarding all intracranial tumors varies between 15%
454

Meningiomas are well-circumscribed globular or lobu- The morphologic characteristics of meningiomas on

lated dural-based tumors, clearly demarcated from the MRI are similar to those seen on CT studies. Typically, they
brain. Histologically, meningiomas are characterized by are peripheral unilobular masses with broad-based dural
increased inter- and intratumoral heterogeneity. Although attachments and smooth, well-defined borders. On T1-W
by recent classifications a number of types are recognized images, meningiomas are usually isointense or mildly hypo-
(and new variants are described), only a minority are found intense to normal gray matter [19–22]. This finding con-
in pure form. Meningothelial, fibrous and transitional me- trasts with those from other intracranial tumors, which are
ningiomas are the most commonly found forms. usually moderately hypointense on T1W images, due to the
Although psammoma bodies are often found in menin- increased water content of the neoplastic tissues. The rel-
giomas, when abundant, they characterize the tumor as atively high cellularity and low water content of most
psammomatous meningioma (Fig. 1). Less commonly meningiomas may account for the generally isointense
found subtypes are angiomatous, microcystic [15] and appearance on T1W images. Although the signal intensity
secretory meningiomas. The above-described subtypes are on T2W images varies, most tumors are reported to be
grade I by WHO classification, exposing a low risk of re- isointense to mildly hyperintense compared with gray mat-
currence and aggressive growth. On the contrary, the ter. Nearly all meningiomas enhance rapidly and intensely
WHO grade II (chordoid, clear cell and atypical) menin- following contrast administration [23] (Fig. 5).
giomas, and especially the WHO grade III (papillary, Determination of the compartment in which the lesion is
rhabdoid and anaplastic,) meningiomas are more aggres- located is very important to the establishment of a correct
sive with a higher risk for recurrence [16]. On non-contrast differential diagnosis. The extra-axial location of the me-
CT, meningiomas appear as unilobular, homogeneous hy- ningiomas is better detected on MRI than on CT.
perdense masses in relation to brain parenchyma. After the A useful feature in confirming the extra-axial location of
administration of contrast medium, they show intense and the suspected meningioma is the inward bowing of the
usually homogeneous enhancement (Fig. 2). Calcification gray-white junction of the adjacent brain parenchyma, often
is seen on CT in 20–27% of meningiomas [17, 18]. It is called white matter buckling [24]. White matter buckling is
usually microscopic or punctuated, but may be large, especially well visualized on MRI studies because of the
conglomerate, peripheral or central (Fig. 3). Hyperostosis superior recognition of the gray and white matter of the
of the adjacent calvarium or skull base may be seen in 18– superficial brain on MRI examinations (Fig. 6). Other use-
50%. Bone destruction is an uncommon feature found in ful MR characteristics, confirming the extra-axial location,
approximately 3% of cases (Fig. 4.) are the presence of a signal void pseudocapsule and CSF

Fig. 1 Different histological

types of meningiomas (haema-
toxylin–eosin, original magnifi-
cation ×200). a Meningothelial
meningioma, composed of nests
of cells with rounded nuclei and
indistinct cytoplasmic borders.
b Fibrous meningioma consist-
ing of parallel spindle cells and
bands of collagen. c Transitional
meningioma characterized by
the formation of whorls.
d Psammomatous meningioma
with multiple psammoma bodies
 455

Fig. 2 Typical meningioma.

NCCT shows a well-marginated,
high density mass with punctu-
ate calcifications (a), which en-
hances homogeneously after the
administration of contrast mate-
rial (b)

Fig. 3 Calcification patterns of

meningiomas in NCCT. a Ring-
like peripheral calcification.
b Dense confluent calcifica-
tions. c Multiple psammomatous
calcifications (arrowheads) with
partial rim-like calcification
(arrow). d Almost completely
calcified meningioma
456

Fig. 4 Axial post-contrast

(a) and coronal (b) CT images
show lobulated bilaretaral fal-
cine mass with intense homo-
geneous enhancement. Note the
destruction of the calvarium and
the extracranial extension of the
mass

cleft. The pseudocapsule consists of linear signal void rep- [30] and aneurysm [33]. The precise pathologic explana-
resenting the dura itself, interposed between the tumor and tion for the “dural tail” sign is questionable as to whether
the brain parenchyma, as well as of punctuate foci of signal there is correlation with dural invasion of intracranial me-
void owing to the displaced vessels (Fig. 7) [20, 21]. ningiomas or only reactive thickening of the dura mater.
The CSF cleft trapped between the cortex and the Some reports have found reactive vascular changes [34,
meningioma demonstrates low-signal intensity on T1W 35], whereas other describe neoplastic invasion into the
images and high-signal intensity on T2W images (Fig. 8). subarachnoid or subdural space [36, 37].
About 60% of meningiomas show a linear enhancement En plaque meningiomas are a variant of primary neur-
along the dura matter on either side of the tumor called the axial meningiomas, which may infiltrate both the dura and
“dural-tail sign” (Fig. 9). the bone cloaking the inner table of the skull. Other im-
This sign is not specific to meningioma, and is also aging features of meningiomas include peritumoral edema,
observed in several conditions including glioma, [25, 26] cystic changes, lipomatous transformation and intracranial
brain metastasis [27], acoustic neuroma [28, 29], lympho- hemorrhage [38].
ma [30, 31], adenoid cystic carcinoma [32], sarcoidosis

Fig. 5 Typical MR findings in a parasagittal meningioma. Axial T1WI (a) and coronal T2WI (b) show a well delineated, isointense mass on
both sequences. After administration of contrast medium (c), the mass shows intense and homogeneous enhancement
 457

Schwannomas

The schwannoma is a benign tumor, composed entirely of

Schwann cells. The neurofibroma is a well-differentiated
nerve sheath tumor composed predominantly of Schwann
cells and, to a lesser extent, fibroblasts and perineural
cells. Neurofibromas of cranial nerves are extremely rare
[2, 40]. Schwannomas are the second most common extra-
axial intracranial tumors, preceded only by meningiomas.
They constitute 5–10% of all intracranial neoplasms. The
peak incidence is between the third and sixth decade.
Macroscopically, schwannomas are typically well cir-
cumscribed and more often globular than fusiform in con-
figuration. In small lesions, the parent nerve can be detected
within the tumor, but in larger tumors the relationship
between the nerve and the tumor becomes obscured. The
Fig. 6 Large parietal meningioma. Sagittal T1WI shows an extra- schwannoma is surrounded by a thick, completely collag-
axial isointense mass compressing the adjacent cortical convolutions enous capsule.
(arrows) Microscopically, two patterns can be distinguished, ac-
cording to the morphology of the tumor cells and their
spatial arrangements: the Antoni A and B types as de-
Diffusion-weighted MRI, perfusion MRI and proton MR scribed in 1920 by Antoni [6, 41]. In the Antoni A type,
spectroscopy may be used in the diagnosis of meningio- tumor texture is compact and composed of interwoven
mas. On diffusion-weighted MR images the atypical and bundles of long bipolar spindle cells. The type B Antoni
malignant meningiomas tend to be markedly hyperintense architecture is often intermingled with type A, and has a
and exhibit marked decreases in the diffusion constant loose texture and polymorphism. Mucinous and cystic
(DAV) or ADC values when compared with normal brain changes occur and when confluent, large cysts develop.
parenchyma, while the benign meningiomas have a var- Like their intraspinal counterparts, the intracranial schwan-
iable appearance on diffusion-weighted images and tend to nomas show a predilection for the sensory nerves, and
have higher (DAV) values compared with normal brain. On most often involve the vestibular division of the eight
perfusion MRI the meningiomas are hyperperfused. In MR nerve. The fifth cranial nerve is the second most common
spectroscopy, the meningiomas are characterized by the site of origin. Schwannomas of the jugular foramen usually
presence of alanine, low creatine and N-acetyl-aspartate, originate from the ninth nerve. The facial nerve schwan-
high choline and glutamine and absence or low quantities noma can be located in the internal auditory canal (IAC) or
of lipids [39] (Fig. 10). in the facial canal.

Fig. 7 Frontal meningioma.

a Axial T2WI shows a rim-like
low signal intensity (arrows)
representing the dural pseudo-
capsule. b T1WI at a lower level
shows the signal voids (arrows)
at the periphery of the mass due
to the displaced vessels
458

Fig. 8 Parasagittal meningio-

ma. a Axial T1- and b T2-
weighted images show a CSF
cleft surrounding the tumor that
exhibits low signal on T1 and
high signal intensity on T2WI
(arrows)

The intracanalicular vestibular schwannoma is char- Moreover, the tumor enhances after intravenous gado-
acterized on MR imaging by the absence of the normal linium injection. Therefore, it is important to perform thin
CSF signal in the IAC, and by the distorted anatomy of the section T1-W images both before and after gadolinium
neural bundle complex. Instead, an intracanalicular mass is injection. The T1W images without gadolinium can used
seen, not always associated with an enlargement of the for the differential diagnosis with intralabyrinthine haem-
IAC, and with variable extension into the cerebellopontine orrhage [42, 43] (Fig. 12).
angle cistern. The tumor enhances after gadolinium. With The large vestibular schwannoma is located in the IAC
conventional CT these lesions cannot be reliably diag- with extension the cerebellopontine angle, centered around
nosed (Fig. 11). the IAC. The resulting appearance is that of a scoop of ice
The intralabyrinthine vestibular or cochlear schwanno- cream on the top of an ice cream cone. The signal intensity
ma is characterized on T2-W MR images by the absence (SI) depends on the histological composition of the tumor.
of the normal fluid signal in the involved part of the Antoni type A lesions tend to present a homogeneous SI,
labyrinth. The diagnosis relies on the use of high res- are hypointense on T2-W images and enhance homoge-
olution, ultra-thin section heavily T2-W images. These can neously. Antoni type B lesions contain more extracellular
be obtained by using ultra-thin 3D FT FSE T2W images or fluid and therefore present a higher SI in the T2W image;
by using a 3D gradient echo sequence such as constructive they often contain intratumoral cysts or necrotic foci. A
interference in steady (CISS) sequence. nearly complete cystic schwannoma is rare [44]. Associ-

Fig. 9 Post-contrast T1WI of different meningiomas. Axial (a), coronal (b), and sagittal (c) images show different types of “dural-tail”
enhancement (arrows)
 459

Fig. 10 Right parietal meningioma. a Gd-enhanced image. b On marked increase in choline (cho) and a significant decrese of creatine
ADC, the tumor is partially hyperintense and the diffusion constant is (Cr) and N-acetyl-aspartate (NAA) levels. The peaks to the right of
higher than normal brain values. c Relative cerebral volume map NAA correspond to lipids, lactate and alanine
shows typical high perfusion (red color). d Spectrum demonstrates

Fig. 11 a Axial 1 mm over-

lapping sections, 3D TSE T2W.
b Coronal 1.5 mm 3D FT GE
T1W after Gd. Intracanalicular
vestibular schwannoma. Very
small nodular tumor, located
inferoposterior in the IAC,
clearly related to the inferior
vestibular part of the nerve
(arrows)
460

Table 1 Radiological differential diagnosis between schwannoma

and meningioma in the cerebellopontine angle
Schwannoma Meningioma

Intracanalicular com- Almost always Rare

ponent (95%)
Centered around the Yes No, eccentric
IAC
Calcifications No Possible
Necrotic/cystic parts Frequent Rare
Dural tail sign Possible Possible
Secondary arachnoid Possible in large No
cyst tumors
Influence on the bone Enlargement of Hyperostosis, enostosis,
the IAC invasion
Supratentorial exten- No Possible
Fig. 12 Coronal 1.5 mm overlapping sections, 3D FT GE T1W sion
after Gd. Intralabyrinthine cochlear schwannoma. Pathological en- Contact with facies Sharp angle Broad based contact
hancement of the left cochlea (arrow)
posterior

ated arachnoidal cysts or loculations are encountered foramen is possible. Frequently, the tumor presents a dumb-
around the tumor in 5–10% of cases [44, 45]; they are bell configuration, with part of the lesion located in Meckel’s
especially observed in large schwannomas (Fig. 13). Intra- cave (supratentorial) and part of the tumor extending into
tumoral calcifications are rare and, when found, should the medial cerebellopontine angle cistern (infratentorial)
favor the diagnosis of meningioma. The main differential (Fig. 14). The imaging characteristics are the same as for the
diagnosis of acoustic schwannoma is CPA meningioma vestibular schwannoma, but intratumoral cysts are more fre-
and is described in Table 1. quent [46].
The trigeminal nerve schwannoma can be located in The jugular foramen schwannomas are usually large at
every segment of the nerve: the cisternal segment in the presentation with an extension in the posterior cranial fossa
prepontine and cerebellopontine cistern, Meckel’s cave,
cavernous sinus, the superior or inferior orbital fissure. From
Meckel’s cave extension below the skull base through oval

Fig. 14 Trigeminal nerve schwannoma. Axial SE T1W after Gd.

Trigeminal nerve schwannoma. The tumor has a solid, enhancing
part and a cystic part. There is a dumbbell shape with a component
Fig. 13 Axial 1.5 mm overlapping sections, 3D FT GE T1W after in Meckel’s cave and in the medial cerebellopontine angle cistern.
Gd. Large vestibular schwannoma with an intratumoral cyst and a Notice the cystic extension through the oval foramen, following the
large secondary arachnoidal cyst (non-tumoral) maxillary division of the nerve
 461

and below the skull base in the carotid loge (Fig. 15). The
most common clinical presentation is hearing loss or
symptoms relating to a posterior fossa mass. Glossopha-
ryngeal deficit points to a large extension below the skull
base. The tumor causes an enlargement of the jugular fo-
ramen, with rounded, sharp and sclerotic rims, without
bony invasion or osteolysis on CT. In the differential di-
agnosis, large vestibular schwannomas should be consid-
ered: they can grow into the jugular foramen, but are
associated with erosion and enlargement of the IAC.
Conversely, a jugular foramen schwannoma may have a
significant extension in the cerebellopontine angle but the
IAC remains normal. Jugular foramen schwannomas tra-
ditionally present a low SI on T1W and a high signal on
T2W images. The enhancement is strong or moderate.
Cystic components are less frequent than in the trigeminal
nerve schwannoma. In the differential diagnosis, the fol-
lowing lesions must be considered: meningioma, glomus
jugulotympanicum, metastasis, lymphoma, giant cell tumor.
The facial nerve schwannoma can arise in the IAC, at Fig. 16 Axial CT scan through the left petrous bone (1 mm slice
the level of the ganglion geniculi, in the middle ear or in thickness). Facial nerve schwannoma. A soft tissue mass involves
the facial canal. Because the facial nerve is located in the the geniculate ganglion and the proximal tympanic segment of the
left facial nerve. The tumor causes bone erosion with scalloping and
anterosuperior quadrant of the IAC, bony erosion in this enlargement of the geniculate fossa, and extends into the tympanic
vicinity favours a facial rather than a vestibular schwan- cavity, where it abuts the auditory ossicles (arrow)
noma, as would extension of the mass in the labyrinthine
segment of the facial canal. A facial nerve schwannoma
causes enlargement of the facial canal with possible ero- Metastases
sion and extension in the middle ear as a soft tissue mass
[47] (Fig. 16). Metastases (calvarial, dural or leptomeningeal) are the sec-
ond most common extra-axial neoplasms in the supraten-
torial compartment.
Calvarial metastases may appear in many patients with
malignant tumors. Carcinomas of the lung, breast, kidney
and prostate are the most frequent primary neoplasms.
Calvarial metastases appear on plain radiographs as os-
teolytic or osteosclerotic lesions. CT with bone window
algorithm is very sensitive in detecting calvarial metasta-
ses. Contrast-enhanced MRI is used to detect subtle intra-
diploic lesions and to determine extension of the metastatic
lesion of the calvarium into the epidural space with in-
volvement of the underlying dura or brain. These metas-
tatic lesions demonstrate prolonged T2 relaxation times
and therefore are hyperintense on T2-weighted images with
fat suppression contrasted against the normally hypoin-
tense calvarium [48].
Dural metastases may usually occur as an extension of
the tumor to the dura from the adjacent calvarial metas-
tases. Occasionally, dural metastases may occur without
associated bony involvement. The most common primary
tumors associated with dural metastases are breast, lung,
prostate, melanoma and neuroblastoma. Lymphoma and
leukemia are other common tumors. On CT, dural metas-
tases appear as isodense areas of dural thickening and
Fig. 15 Jugular fossa schwannoma. Gd-enhanced sagittal SE T1WI.
An enhanced fusiform soft tissue mass extends from the right enhancement. Post-contrast T1-weighted MRI is more sen-
cerebello-pontine angle through the pars nervosa of the jugular sitive in depicting meningeal involvement (Fig. 17). Since
foramen into the right parapharyngeal space the inner table of the skull is hypointense on MRI the
462

abnormally enhancing dura clearly stands out between the

CSF and the bone of the skull [49].
Leptomeningeal metastases or meningeal carcinomatosis
is usually the result of hematogenous spread from extra-
cranial malignancies. Anaplastic astrocytoma, glioblastoma
multiforme, ependymoma and medulloblastoma may also
show leptomeningeal dissemination through the CSF. Post-
contrast T1-weighted and FLAIR studies offer the best im-
aging method to demonstrate such lesions. Leptomeningeal
metastases presents with increased enhancement in the sub-
arachnoid spaces of the cortical sulci, the fissures and the
cisterns.
Nodular formations can also develop in the subarach-
noid spaces that may invaginate within the adjacent brain
parenchyma. The pattern of leptomeningeal infiltration is
identical to that described on the CT but the intensity of
enhancement and thus the sensitivity is greater in the post
contrast MR studies (Figs. 18, 19) [50–52]. A major draw
back in the diagnosis of meningeal carcinomatosis by
either CT or MRI is that the changes described above lack
specificity. Indeed, abnormal enhancement of the lepto-
meninges identical to carcinomatosis occurs in a variety of
infections including viral, bacterial or fungal diseases [53].
Inflammatory processes such as sarcoidosis or Langerhans Fig. 18 Post-contrast T1-weighted MRI scan of the brain in a
patient with metastatic breast carcinoma. There is evidence of men-
histiocytosis also present with abnormal enhancement of ingeal carcinomatosis manifested by abnormal enhancement of the
the leptomeninges. In the case of dural involvement the leptomininges over the convexity of the right cerebral hemisphere
differential diagnosis is even broader. Besides the infec-
tious or the specific inflammatory processes already dis-
cussed, the dura enhances in response to inflammatory
reaction that occurs following previous subarachnoid hem-
orrhage. Furthermore, the dura enhances in response to a

Fig. 17 Coronal post contrast T1-weighted MRI scan of the brain in

a patient with metastatic prostate carcinoma to the skull. Three Fig. 19 Post-contrast T1-weighted MRI scans of the brain in a
destructive metastatic lesions are seen in the skull. Associated soft patient with melanoma. Enhancing masses are noted in both
tissue tumors are also present extending into the epidural space and cerebello-pontine angles mimicking acoustic schwannomas (ar-
compressing the brain parenchyma. The abnormal enhancement of rows). This abnormality represents part of the spectrum of meningeal
the dura (arrows) represents tumor invasion carcinomatosis
 463

variety of neoplastic or non-neoplastic diseases that in- [56]. These lesions are similar in their development, his-
volve the calvarium. In the case of metastatic or primary tology, behavior and imaging and for this reason are dis-
tumors of the calvarium inflammatory reaction often de- cussed together. As previously discussed, epidermoids and
velops in the dura even in the absence of dural involve- dermoids along with Rathke cleft cysts, arachnoid cysts
ment by the neoplasm. and craniopharyngiomas are considered by some to rep-
Previous published reports suggest that the sensitivity of resent a continuum of ectodermally derived cystic epithe-
contrast enhanced MRI is rather poor, since positive re- lial lesions [57]. Both lesions are generally considered
sults consistent with meningeal carcinomatosis are elicited developmental/congenital masses rather than neoplastic,
in only 36–66% of patients with positive CSF cytology. arising from ectodermal heterotopia. Both cysts are lined
These findings are hardly surprising, considering the fact with stratified squamous epithelium, with dermoids adding
that only a few malignant cells in the CSF are sufficient for mesodermal elements such as hair, sebaceous and sweat
establishing this diagnosis by cytology. On the other hand, glands.
the superior sensitivity of CSF cytology is secured only if Epidermoids are slightly more common than dermoids
multiple spinal taps are performed [48]. intracranially. They typically spread along the basal sur-
faces, with the cerebellopontine angles being the most
common location, followed by the parasellar [58–60].
Arachnoid cysts They are extra-axial lesions with only 1.5% being intra-
cerebral [61]. They are overwhelmingly benign, although
These non-neoplastic cysts are loculated collections of CSF they rarely can be malignant. Average age of presentation is
within a reduplication of arachnoidal membrane. They are 37.3, with a male to female ratio of 3:2 [56]. The symp-
thought to be part of a continuum of epithelial cysts at the tomatic onset is generally slow, lasting 2 years or more,
more benign end of the spectrum behaviorly. They tend to although for suprasellar lesions it is much shorter [61].
present at an older age, usually the 5th decade, with head- Presenting symptoms may include headaches, visual prob-
aches, visual field defects and impotence [54]. They are lems, cranial nerve symptoms and seizures, which typically
most frequently located in the middle cranial fossa. Other indicate rupture. Rupture can produce aseptic meningitis,
sites include the suprasellar area, the frontal convexity, the which can be lethal although not necessarily so. Epider-
quadrigerminal cistern and the foramen magnum. Arach- moids on CT appear as hypodense masses, with irregular
noid cysts show on CT density similar to that of CSF. boarders and rare contrast enhancement (Fig. 20a). Dense
Erosion of the adjacent calvarium is often present. They are lesions have been reported [62] and calcification is oc-
well defined, with no calcification and no enhancement casionally seen [60]. On MR, they typically are of low
[54, 55]. On MRI arachnoid cysts show CSF signal inten- signal on T1- and of increased signal on T2-weighted im-
sity on all pulse sequences. ages, following that of CSF on all pulsing sequences [56]
(Fig. 20b, c). They can demonstrate increased signal on
T1-weighted images, which is due to a high lipid content
Epidermoids and dermoids [60]. Epidermoid and arachnoid cysts can also be dis-
criminated on the basis of diffusion-weighted images. On
Epidermoids and dermoids are uncommon, slow growing conventional spin echo images both show long T1 and T2
masses that account of 1% of all intracranial neoplasms images. On diffusion-weighted images, epidermoid cysts

Fig. 20 Epidermoid cyst. a Post-contrast CT shows a hypodense that of CSF. d DWI shows an inhomogenous high signal intensity
lesion compressing the right cerebellar hemisphere. On NCT1WI indicative of restricted diffusion
(b) and T2WI (c), the same lesion shows signal intensity similar to
464

Fig. 21 Dermoid cyst. a Sagit-

tal T1- and b axial T2-weighted
images show a suprasellar het-
erogeneous mass. The high sig-
nal areas on T1WI are due to the
presence of fatty material

show high signal intensity due to restricted motion of pro- structures. On T1-weighted images chordoma is iso- or
tons by the presence of membranes of densely layered hypointense and replaces the hyperintense clival fatty
epithelium, while arachnoid cysts are hypointense due to marrow. On T2-weighted images it is inhomogeneous and
their free water motion [63, 64] (Fig. 20d). hyperintense. After the administration of contrast medium,
Dermoids are midline lesions, occurring in the parasel- chordoma shows heterogeneous enhancement (Fig. 22).
lar, frontobasal region or posterior fossa [62]. Average age The differential diagnosis include metastasis, craniopha-
of presentation is 36.2, with a male to female ratio of 3:1 ryngioma, chondrosarcoma, pituitary tumors and nasopha-
[56]. The complications of dermoids are similar to epider- ryngeal carcinoma.
moids. They can present with headaches, seizures, men- Eosinophilic granuloma belongs to the group of dis-
ingeal signs and TIAs [65, 66]. Most of these symptoms orders called histiocytosis X. They are characterized by
are indicative of rupture, which produces a chemical or histiocytic proliferation. The other two conditions are
aseptic meningitis and which can be lethal [65, 67]. Hand–Schuller–Christian and Letterer–Siwe disease. Eo-
The CT appearance of dermoids is similar to epider- sinophilic granuloma represents approximately 70% of the
moids. Their MR appearance depends on the amount of fat total number of histiocytosis X. Although it can affect any
present, although generally they are of increased signal on skeletal site, the most common locations are skull, man-
both T1- and T2-weighted images [56] (Fig. 21). CT or
MR can both make the diagnosis of rupture although MR
is the preferred preoperative study [66].
Treatment is surgery, with 86% being in good or
excellent condition post-operatively. The 20-year survival
of epidermoids is 92.8%, with good survival even with
recurrence [56]. Epidermoids have a classic mother-of-
pearl appearance at surgery.

Bone tumors

Chordoma is locally aggressive but slow-growing tumor

and is thought to derives from notochordal remnants. It
invades adjacent soft tissue structures and metastasize in-
frequently. Chordoma occurs in the late middle age and
most commonly arises in the sacrococcygeal (50–60%) or
sphenooccipital regions (25–40%) [68–70]. The latter usu-
ally causes destruction of the clivus and spreads into the
middle or posterior cranial fossa compressing the brain-
stem or the nasopharynx. CT demonstrates clearly the mass Fig. 22 Chordoma. Sagittal post-contrast T1-weighted image shows
and the bony component of the tumor. MRI depicts better a heterogeneously enhanced lesion which causes destruction of the of
the extent of the lesion and its relation to the adjacent the clivus
 465

Fig. 23 Eosinophilic granuloma of the skull. Axial T1-weighted image (b). On post-contrast T1-weighted image (c) the lesion shows
image (a) shows an isointense destructive lesion of the left temporal a heterogeneous enhancement. Note also the epidural extension of
bone extending into the orbit, which is hyperintense on T2-weighted the tumor

dible, spine, ribs and long bones. In the skull, the tumor weighted images. After the administration of contrast me-
appears as a lytic lesion with epidural extension. The dium, intense enhancement is seen (Fig. 23) [70].
tumor appears hypointense on T1 and hyperintense on T2-

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