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Intracranial epidermoid cyst

A.Prof Frank Gaillard et al.

Intracranial epidermoid cysts are relatively common congenital lesions


which account for about 1% of all intracranial tumours. They result from
inclusion of ectodermal elements during neural tube closure, and typically
present in middle age due to mass effect on adjacent structures. Their
content, derived from desquamated epithelial cells, mimics CSF on CT and
MRI, with the exception of DWI which demonstrates restricted diffusion.
Management is surgical and prognosis is good.

Epidemiology
Although predominantly congenital, epidermoid cysts are usually very slow
growing and as such take many years to present. Typically patients are
between 20 and 40 years of age . There may be increased prevalence in
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males, although this is not found in all series .


5-6

Epidermoid cysts account for approximately 1% of all intracranial tumours .


2,8

An uncommon association exists with anorectal anomalies, sacral anomalies


and presacral mass, and is known as the Currarino triad .
5

Clinical presentation
Signs and symptoms of epidermoid cysts are due to gradual mass effect, with
presentation including : 4

headaches: most common


cranial nerve deficits
cerebellar symptoms
seizures
raised intracranial pressure
Recurrent aseptic meningitis is uncommon but recognised, similar to the less
common dermoid cyst . 4

Pathology
Epidermoid cysts may be congenital (most common, arising from ectodermal
inclusion during neural tube closure ) or acquired (post surgical or post
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traumatic implantation). Pathologically, intracranial epidermoid cysts are


identical to the petrous apex and middle ear congenital cholesteatomas. They
differ from dermoid cysts which have both epidermal and skin appendages
such as hair and sebaceous cysts and mature teratomas which have all three
layers.

They have a thin capsule which is made of a thin layer of squamous


epithelium, which macroscopically is white and pearly, and may be smooth,
lobulated or nodular. Internal cystic components are often filled with
desquamated epithelial keratin and cholesterol crystals; grossly appearing as
a creamy, waxy material. It should be noted that some authors contest that
epidermoid cysts are along a spectrum which includes cystic
(adamantinomatous) craniopharyngiomas, which have some histological
similarities . Cholesterol and keratin components account for their distinct
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imaging characteristics . 2

Location
intradural: 90%
o cerebellopontine angle: 40-50% 2,8

third most common cerebellopontine angle


mass, after acoustic schwannomas and meningiomas, and accounts
for approximately 5-10% of all tumours in this region
2,4,8

o suprasellar cistern: 10-15% 8

o fourth ventricle: ~17% 8

o middle cranial fossa


o interhemispheric
o spinal (rare)
extradural: 10% 8

o most within skull

Radiographic features
Intracranial epidermoid cysts appear as lobulated lesions that fill and expand
CSF spaces and exert gradual mass effect, insinuating between structures
and encasing adjacent nerves and vessels. A common feature of posterior
fossa cysts is displacement of the basilar artery away from the pons.

CT
The combination of cellular debris along with a high cholesterol content lower
the density of epidermoids to approximately 0 HU, and can thus be identical in
density to CSF, and look the same as an arachnoid cyst.
Calcification is seen in a minority of cases (10-25% ), and rarely an
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epidermoid cyst may be hyperdense due to haemorrhage, saponification or


high protein content ("white epidermoids").

They do not enhance, and only very rarely demonstrate any enhancement of
the wall.

MRI
Appearances on MR are similar to those on CT, with epidermoids often being
indistinguishable from arachnoid cysts or dilated CSF spaces on many
sequences.

T1
o usually isointense to CSF
o higher signal compared to CSF around the periphery of the lesion
is frequently seen
o rarely they can be of high signal and are known as white
epidermoids
o rare intralesional haemorrhage can also result in intrinsic high
signal
T1 C+ (Gd)
o thin enhancement around the periphery may sometimes be seen
o in the rare cases of malignant degeneration, enhancement
becomes more pronounced
T2
o usually isointense to CSF (65%)
o slightly hyperintense (35%)
o rarely hypointense, usually in the setting of the so called white
epidermoid (the term refers to the T1 appearance) 2

FLAIR
o often heterogeneous/dirty signal; higher than CSF
o beware of flow artifact from CSF pulsation which can mimic this
appearance
DWI: useful for differentiation from arachnoid cysts due to increased
signal (due to a combination of true restricted diffusion and T2 shine
through) which is not seen with arachnoid cysts

Treatment and prognosis


Surgical excision is the treatment of choice if symptomatic. However, complete
resection is difficult as not all tissue can be removed, especially from around
cranial nerves and vessels. Recurrence is therefore not uncommon, although
growth is typically slow and many years can elapse without new symptoms.

Differential diagnosis
The differential for an epidermoid cyst include:

CSF collections, e.g. arachnoid cyst or mega cisterna magna


o less lobulated
o follows CSF on all sequences, including FLAIR and DWI
dermoid cyst
o often fat density due to sebum
o typically located along the midline
inflammatory cyst, e.g. neurocysticercosis
o smaller, but may be multiple
o may enhance peripherally
o may have associated oedema
o usually no restricted diffusion
cystic tumours, e.g. acoustic schwannoma or craniopharyngioma
o solid enhancing component is usually identifiable
neurenteric cyst

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