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Jpn J Radiol (2013) 31:133137

DOI 10.1007/s11604-012-0150-z

C AS E R E P O R T

Imaging appearance of petrous apex dermoid cysts containing little


or no fat
Naomi Ageshio Taro Shimono
Takeo Goto Kenji Ohata Masahiko Ohsawa
Kenichi Wakasa Yukio Miki

Received: 10 August 2012 / Accepted: 1 October 2012 / Published online: 17 October 2012
Japan Radiological Society 2012
Department of Diagnostic Pathology, Osaka City
University Graduate School of Medicine, Osaka,
Japan

Abstract Intracranial dermoid cysts are rare congenital


lesions with typical imaging findings of well-defined
fat-containing masses located along the midline and
infraten-torial regions. We report two rare cases of
petrous apex dermoid cyst, one in the petrous apex
and the other in the interdural space, with unusual
imaging appearances mim-icking epidermoid cysts
because of the presence of little or no fat. Dermoid
cysts with little or no lipid contents, as in these cases,
may be radiologically misdiagnosed as epi-dermoid
cysts. Careful evaluation of preoperative computed
tomography and magnetic resonance images is
necessary because the surgical approaches used for
this region vary depending on the accurate location
and histopathology.

Keywords Dermoid cyst Petrous apex


Magnetic resonance imaging Computed
tomography

Introduction
Intracranial dermoid cysts are rare congenital
lesions that account for less than 1 % of all
intracranial neoplasms [13]. These tumors arise
from ectopic inclusion of the
N. Ageshio (&) T. Shimono Y. Miki
Department of Radiology, Osaka City University
Graduate School of Medicine, 1-4-3 Asahi-machi,
Abeno-ku,
Osaka 545-8585, Japan
e-mail: n-muroya@rinku.zaq.ne.jp
T. Goto K. Ohata
Department of Neurosurgery, Osaka City University
Graduate School of Medicine, Osaka, Japan
M. Ohsawa K. Wakasa

embryonic ectoderm into the neural tube during


embryo-genesis and are usually located along the
midline and in-fratentorial regions [24]. On
imaging, typical dermoid cysts appear as wellcircumscribed fat-containing masses with no or
only peripheral contrast enhancement [2, 5, 6].
We present two cases of histopathologically
confirmed petrous apex dermoid cyst, one in the
petrous apex and the other in the interdural space,
which presented a diagnostic challenge because of
the rare locations and unusual appearance due to
the presence of little or no fat.

Case reports
Case 1
A 52-year-old man with post-traumatic right-sided
hearing loss since childhood presented with a 2-

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year history of right facial paresthesia. Cranial


computed tomography (CT) (Fig. 1a) without
contrast enhancement revealed a well-defined
hypoattenuating lesion without fat density in the
petrous apex. The lesion was associated with
osseous erosion and sclerosing mastoiditis.
Magnetic resonance imaging (MRI) (Fig. 1bf)
showed a hypointense lesion on T1-weighted
imaging with a linear hyperintense area and
heterogeneous hyperintensity on T2-weighted
imaging. On diffusion-weighted imaging (DWI),
the lesion was hyperintense without reductions in
the apparent diffusion coefficient (ADC) and no
suppressed intensity on fat-suppressed T1-weighted
imaging. Fat-suppressed gadolin-ium-enhanced T1weighted imaging showed only linear peripheral
enhancement. A cholesteatoma and an epider-moid
cyst in the petrous apex were suspected.
Gross total resection of the lesion was
performed by the translabyrinthine approach.
Severe sclerosing mastoiditis

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Fig. 1 Case 1: computed tomography (CT) (a) shows a well-defined


hypodense (not fat) mass (arrow) with osseous erosion in the right petrous
apex. Axial T1-weighted (b), T2-weighted (c), diffusion-weighted (d), fatsuppressed T1-weighted (e) and Gd-enhanced T1-weighted (f) magnetic
resonance imaging (MRI) shows a well-defined mass (arrow) in the
petrous apex, which appears hypointense on T1-

Jpn J Radiol (2013) 31:133137

weighted imaging (b), no suppressed intensity on fat-suppressed T1weighted imaging (e), and hyperintensity on T2-weighted (c) and
diffusion-weighted (d) imaging with peripheral linear contrast
enhancement (f). No apparent reduction in diffusion coefficient
(ADC) is observed on the corresponding ADC map (not shown)

Case 2

Fig. 2 Histopathological study of the case 1 lesion (H&E stain;


magnification 94) revealed the presence of keratinous debris and hair
follicles, lined by stratified squamous epithelium, although no lipid
contents were found

made it difficult to identify even auditory ossicles or to


accurately localize the lesion. The histopathological diagnosis was dermoid cyst, based on the presence of keratinous debris and hair follicles, lined by stratified squamous
epithelium, although no lipid contents were evident (Fig.
2).

A 44-year-old man complained of a 6-month history of


worsening right trigeminal neuralgia. Neurological examination revealed decreased sensation on the right side of
the face and hearing loss of the right ear. Cranial CT (Fig.
3a) without contrast enhancement demonstrated a welldefined hypoattenuating lesion without fat density in the
right middle cranial fossa. This lesion was associated with
osseous erosion of the petrous apex. Subsequent MRI (Fig.
3bf) of the brain revealed the lesion to be predom-inantly
hypointense on T1-weighted imaging with minimal
punctiform hyperintense areas and heterogeneous hyperintensity on T2-weighted imaging. On DWI, the lesion was
hyperintense without reduction of ADC. Peripheral linear
enhancement was seen after gadolinium administration,
and the minimal punctiform hyperintensity of the lesion
was suppressed on gadolinium-enhanced fat-suppressed
T1-weighted imaging. The preoperative diagnosis was an
interdural epidermoid cyst.
The patient underwent a right middle fossa approach to
resect the mass. During the surgical procedure, the lesion
was found to be located in the interdural space of the
middle cranial fossa, extending to the petrous apex. Subtotal removal was performed as part of the capsule was
tightly adherent to the Gasserian ganglion of the trigeminal

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Jpn J Radiol (2013) 31:133137
Fig. 3 Case 2: CT (a) showing a
well-defined hypodense (not fat)
mass (arrow) in the right middle
cranial fossa extending to the
petrous apex. Axial T1-weighted
MRI (b) showing a punctiform
hyperintense region (arrowhead)
in the hypointense mass (arrow),
which is hyperintense on axial
T2-weighted MRI (c) and
diffusion-weighted imaging (d)
with no ADC reduction on the
corresponding ADC map (e).
The punctiform hyperintense
region on T1-weighted imaging
is suppressed (arrowhead) on
Gd-enhanced fat-suppressed T1weighted imaging (f)

contain products of skin appendages and epithelium, such


as lipid metabolites, cholesterol crystals, hair, decomposed
epithelial cells and calcification. On imaging, dermoid
cysts are typically well-defined, non-enhancing or only
peripherally enhancing lesions [2, 5, 6], and are usually
hypodense on CT and hyperintense on T1-weighted
imaging because the cyst contents are high in lipids.
Although intracranial dermoid cysts are rare, locations
such as suprasellar, parasellar, temporal and frontonasal
regions as well as the posterior fossa have been reported.
The lateral wall of the cavernous sinus is the most frequent
site [7].

Fig. 4 Histopathological study of the case 2 lesion (H&E; magnification 94) revealed a squamous epithelium-lined cavity containing
hair follicles, sebaceous glands and small amounts of lipid

nerve and could not be dissected safely. The histopathological diagnosis was dermoid cyst based on the presence of a
squamous epithelium-lined cavity containing hair folli-cles,
sebaceous glands and some lipid contents (Fig. 4).

Discussion
Dermoid cysts comprise stratified squamous epithelium and
skin appendages such as hair follicles, sebaceous glands and
sweat glands. The origin of these cysts is strictly ectodermal,
so dermoid cysts do not contain adipose tissue, which arises
from mesodermal elements [5]. Dermoid cysts

Case 1 represented a dermoid cyst originating in an


extremely rare region, the petrous apex, and showed an
atypical appearance on various imaging modalities because of
a lack of fat contents. Case 2 represented a dermoid cyst
originating in the interdural space of the middle cranial fossa
extending to the petrous apex, with minimal fat contents.
In case 1, the dermoid cyst existed in the petrous apex with
sclerotic mastoiditis. Differential diagnoses for lesions of case
1 in the petrous apex include a wide spectrum of diseases:
primary bone tumors, metastasis, cholesterol granulomas,
cholesteatomas, epidermoid cysts, mucocele and aneurysms of
the carotid artery [1]. Cholesterol gran-ulomas are the most
frequent surgically treated lesions of the pneumatised petrous
apex (up to 60 %), which usually appear hyperintense on T1weighted imaging; however, this case 1 appeared almost
hypointense. Taking the scle-rosing mastoiditis into
consideration, imaging appearances in case 1 were most
consistent with cholesteatoma or epi-dermoid cyst, considered
to be a result of the dense keratinous content of the cyst,
which limits the diffusibility

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of protons. The hyperintensity of epidermoid cysts on DWI


has been confirmed to originate from the T2 shine-through
effect rather than the reduction of ADC values [8].
The localization of the dermoid cyst in case 2, however,
was different from that in case 1, and the tumor existed in the
interdural space of the middle cranial fossa, extending to the
petrous apex. The location and imaging appearance of the
tumor in case 2 were very similar to the previously reported
case by Sturiale et al. [1]. In this region, the dura mater of the
middle cranial fossa floor duplicates itself as a double layer,
the outer dural layer (dura propria) and the inner membranous
layer, to constitute Meckels cave and the lateral wall of the
cavernous sinus [1, 9]. The trigeminal ganglion, trigeminal
cistern and postganglionic trigeminal rootlets are located
within Meckels cave. Access to this region might be
challenging because of the presence of surrounding important
neurovascular structures. However, the removal of an
interdural tumor can be relatively easy and safe because the
inner membranous layer of the lateral wall of the cavernous
sinus separates interdural tumors from the venous channels of
the cavernous sinus. On MRI, interdural localization can be
recognized by their typical smooth contours, oval shape and
medial displacement of the intracavernous portion of the
internal carotid artery without effacement or narrowing [9].

Differential diagnoses for the lesions of case 2 in the


interdural space of the middle cranical fossa include: trigeminal schwannomas, oculomotor schwannomas, trochlear schwannomas, epidermoid cysts, metastatic tumors,
melanomas and cavernous angiomas [9]. Considering the
smooth contours, oval shape and signal characteristics,
imaging appearances in case 2 were most consistent with
an interdural epidermoid cyst.
To the best of our knowledge, only four English reports
have described dermoid cysts originating at the petrous
apex [1, 2, 10, 11]. The case reported by Behnke et al. [11]
is strictly a teratoma, not a dermoid cyst, containing mature
fat and skeletal muscle that arises from mesodermal elements. Among the remaining three cases, no image was
given in the case reported by Misaswa et al. [10], so the
exact location of the dermoid cyst could not be compared.
One of the three reports described the dermoid cysts as
including fat density [2], while the remaining two
descriptions did not mention lipid contents [1, 10]. Only
two intracranial dermoid cysts without radiologically proven fat contents have been reported [4, 6]. One of those
two reports described a case of intracranial dermoid cyst
showing hyperattenuation on CT and containing high
protein and calcium levels and low lipid levels [4].
Generally, most dermoid cysts are found in the ovaries.
Among ovarian dermoid cysts, no fat is found in 7 % on
MRI [12]. Ovarian dermoid cysts are defined as mature
cystic teratomas, which are composed of ectodermal,

Jpn J Radiol (2013) 31:133137

mesodermal and endodermal elements [12]. Adipose tissue


arises from the mesodermal elements. However, trapped
mesodermal elements are not seen in intracranial dermoid
cysts. Consequently, dermoid cysts contain no adipose
tissue, and the fatty content of these cysts actually represents keratinous debris, liquid cholesterol, sebaceous and
sweat secretion as well as ectodermal appendages [1]. If
these fatty contents are minimal, intracranial dermoid cysts
may show little or no fat contents on imaging, as in our
cases mimicking epidermoid cysts. Though dermoid cysts
and epidermoid cysts are similar congenital dysembyogenetic tumors, dermoid cysts have the more solid and welldefined boundaries. So, microsurgical resection of dermoid
cysts tends to be technically easier than that of epidermoid
cysts [6], and it can be important to suggest radiological
differentiation of dermoid cysts from epidermoid cysts.
Intracranial dermoid cysts are generally benign legions.
However, depending on the location, focal neurological
deficits may occur, as well as epileptic seizures, persistent
headache, mental changes and psychoorganic syndromes.
Rupture of a dermoid cyst can lead to severe complications
such as meningitis, hydrocephalus, reactive vasospasm,
infarction and even death [3, 6]. The possible mechanisms
underlying rupture include an increase in glandular secretions due to age-dependent hormonal changes, movement
of the head or brain pulsation. Malignant changes have
also been reported in intracranial dermoid cysts.
In conclusion, we presented two cases with petrous apex
dermoid cysts mimicking epidermoid cysts on imaging and
containing little or no lipid contents, even in histopathological studies. The fatty contents described as typical
imaging findings of dermoid cysts actually represent
keratinous debris, ectodermal appendages and associated
secretions. Dermoid cysts may depict no fat density or
intensity if the components are insufficient to be detected
on CT and MRI. Although cases of histopathologically
confirmed petrous apex dermoid cysts have been rare in
previous reports, dermoid cysts containing little or no lipid
contents as in these cases may be radiologically misdiagnosed as epidermoid cysts.

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