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EUROPEAN JOURNAL

OF RADIOLOGY

European Journal of Radiology I7 (1993) 69-84

Review article

Cystic lesions of the brain. A classification based on pathogenesis,


with consideration of histological and radiological features

KG. GOVE,J.M. Hewb, R.L. Karnmanc, W.M. Molenaard, J. Pruime, E.H. Blaauw’
‘Department of Neurosurgery, bDepartment of Neuroradiology, ‘Department of Magnetic Resonance Imaging, dDepartment of Pathology,
‘Department of Positron Emission Tomography, /Medical Electron Microscopy. University Hospital, University of Groningen, Oostersingel 59,
Groningen 9700 RB. The Netherlands

(Received 15 March 1993: revision accepted 26 April 1993)

Abstract

A classification of the existing multitude of cystic lesions of the brain is proposed, which allows an understanding of their genesis
and consequent therapeutic implications, as well as their diagnostic characteristics. Essentially, cerebral cystic lesions may be
classified into the following categories: Cysts containing CSF-like fluid, which include ex vacua type cysts, such as leptomeningeal
cysts, and cysts following surgical resection; cysts with fluid secreting walls and CSF-like content, such as arachnoid cysts; cysts
associated with dysgenesis, for example Dandy-Walker cysts. The ex vacua cysts increase craniospinal compliance, whereas the
other cysts with CSF-like content do not; they are not per se expansive, however, although their occasional location along CSF
pathways may cause obstruction and hydrocephalus. Another category includes cysts with a lining of non-neural epithelium like
colloid cysts, epidermoid cysts, or craniopharyngiomas. They may increase in size and cause symptoms by compression, although
not at the rate of tumour-associated cysts. The cysts associated with gliomas and other tumours have a pathogenesis bearing upon
blood-brain barrier impairment and formation of vasogenic oedema. Finally, one may distinguish a category of cysts with infectious
origin, such as brain abscesses and hydatid cysts. The cysts with CSF-like contents may be recognised by their magnetic resonance
characteristics resembling those of CSF, whereas cysts containing proteinaceous fluid are associated with blood-brain barrier im-
pairment and consequent contrast enhancement. The cysts with a lining of non-neural epithelium exhibit diverse properties of at-
tenuation on comput tomography (CT) and magnetic resonance imaging (MRI), depending on the nature of their cyst contents.

Key words: Computed tomography, brain; Magnetic resonance (MR), brain; Brain. cysts; Brain, CT; Brain, MRI

1. Introduction tion based upon pathogenesis (Table l), that will allow
the understanding of its characteristics (appearance on
Cerebral cystic lesions are unnatural cavities within CT or MRI, possibility of enhancement by contrast),
the brain, in which the continuity of the brain paren- and of its genesis with consequent therapeutic implica-
chyma is disrupted and replaced by fluid. The currently tions (e.g. ex vacua with no need of drainage, or by fluid
available diagnostic modalities of CT and MRI have secretion, requiring drainage). To achieve a proper in-
allowed their early diagnosis, but their multitude and sight into the various mechanisms, it is inevitable to con-
variety are often bewildering and their clinical implica- sider biochemical and morphological evidence, such as
tion is not always readily appreciated. Therefore, it is that acquired by chemical analysis, positron emission
the intention of the present study to present a classifica- tomography (PET), magnetic resonance spectroscopy
(MRS), and electron microscopy or other histological
* Corresponding author. techniques.

0720-048X/93/$06.00 0 1993 Elsevier Scientific Publishers Ireland Ltd. All rights reserved
SSDI 0720-048X(93)00418-X
70 K.G. Go ei al. /Eur. J. Radiol. 17 (1993) 69-84

Table 1
Classification of cysts

I. Cysts containing CSF-like fluid


IA. Ex vacua type cysts
Leptomeningeal cysts
Cysts after surgical resection
Cysts resulting from infarctions
Porencephalic cysts
IB. Cysts with fluid secreting walls and CSF-like content
Arachnoid cysts
Neuroepithelial cysts
IC. Cysts associated with dysgenesis
Dandy-Walker cysts
Interhemispheric cysts
II. Cysts with a lining of non-neural epithelium
Colloid cysts
Cysts associated with craniopharyngiomas
Symptomatic Rathke’s cleft cysts
Epidermoid cysts
Dermoid cysts
III. Cysts associated with gliomas and other tumours
Cysts associated with gliomas, meningiomas, metastases,
haemangioblastomas
IV. Cysts of infectious origin
Brain abscess
Hydatid cyst

By their clinical evolution, cerebral cystic lesions have


traditionally been classified as benign cystic lesions, as
opposed to malignant cerebral cysts. The benign cate- Fig. 1. CT-scan of a 35year-old male with a history of head injury in
gory includes cerebral cysts like arachnoid and lep- his youth, visualising a leptomenineal cyst (C). There is also widening
of neighbouring sulci and dilatation of the ipsilateral ventricle, and
tomeningeal cysts which contain a clear fluid resembling probably the remnants of an old epidural haematoma.
cerebrospinal fluid (CSF); they hardly increase in size
and generally pose no threat to the patient. Malignant
brain cysts, by contrast, are usually tilled with a yellow A. Ex vacua type of cysts
proteinaceous fluid, and are associated with brain For a consideration of their pathogenesis one must
tumours. In the light of the great diversity of cystic reflect on the course of events, when a disruption of tis-
lesions, however, this traditional classification should be sue continuity in the brain has occurred. It is generally
considered inadequate. known that such disruptions of tissue continuity like
those resulting from surgical resections or cerebral
haemorrhage, are initially being occupied by extracellu-
2. Classification lar fluid/CSF after subsidence of the acute phase in
which oedema and haemorrhages prevail. Subsequently
2.1. Cysts containing a CSF-like fluid they tend to collapse and eventually to obliterate, with
the fluid within the cavity being resolved by reabsorp-
Radiologically, the cysts containing CSF-like fluid, tion and drainage to the CSF spaces.
including those of the ex vacua-type, appear on CT- A cavity will persist to a greater or lesser extent, how-
scans with the same attenuation value as that of CSF ever, when the loss of tissue is too extensive to be accom-
[1,2]. On MR-scans they exhibit long Tt- and TZ- modated by the elasticity of the surrounding brain.
relaxation times, and like CSF, they are hypointense on A typical example of such an ex vacua type of cyst, i.e.
Tr-weighted images, while on Tz-weighted images they a cyst resulting from a shortage of brain tissue, is the
present with a high signal intensity. Since there are no traumatic leptomeningeal cyst. These cysts are the con-
inflammatory changes with blood-brain barrier impair- sequence of traumatic injuries [3] (Fig. 1) and constitute
ment in the surrounding tissue, the lesions do not show pockets of the subarachnoid space, which are more or
enhancement upon intravenous administration of con- less separated from the surrounding subarachnoid
trast agents. spaces by adhesions. Nonetheless, their rapid filling with
K.G. Go et al. /Eur. J. Radiol. 17 (19931 69-84 71

intrathecally administered contrast medium or radio-


isotope demonstrated their extensive communication
with the surrounding subarachnoid spaces and cisterns
[4]. They are frequently associated with dilatation and
retraction of adjacent ventricles, which should also be
understood in the light of the lack of tissue. Moreover,
there is often evidence of old fractures.
An ex vacua situation also exists after large resections
of tumours; postoperative scans often show the resulting
cystic lesion (Fig. 2).
Other cavities that exist on account of the ex vacua
situation, i.e. the lack of tissue. are those resulting from
extensive infarctions (Fig. 3)
The category of ex vacua cysts also include the
porencephalic cysts (Fig. 4). These have been defined as
congenital cavities that often, but not always, communi-
cate with the ventricular system or subarachnoid spaces
[5,6]. They constitute an amalgam of more or less exten-
sive defects, which may be the result of developmental
derangements (schizencephalies), or of brain damage
due to vascular occlusions and haemorrhage (destruc-
tive encephaloclastic porencephaly) [7].
Conceivably, the other types of ex vacua cavities may
also be associated with other evidence of loss of tissue,
such as enlargement of ventricles (ex vacua hydroce-
phalus) which, in addition, may show deformation and
retraction, as well as dilatation of sulci on the brain con-
vexity.
Many cavities which follow tissue disruption, such as
Fig. 2. T,-weighted MR-scan (SE-TR: 650. TE: 20 l/l) after adminis-
traumatic leptomeningeal cysts [8] eventually acquire a
tration of Gd-DTPA, depicting an ex vacua type of cyst in an &year- cellular lining, which tends to resist fusion of the walls
old girl 2 weeks after the removal of an astrocytoma. following collapse and thus contributes to persistence of
the cavity.

a b d

Fig. 3. (a) CT-scan visualising a large left occipital cyst in a %-year-old male. which the biopsy showed to be the remnant of an old infarction.
(b) T,-weighted MR-scan (SE-TR: 650, TE: 30), showing the cyst contents to have the same hypointensity as ventricular CSF. (c) T?-weighted MR-
scan (SE-TR: 2000. TE: 100). presenting the cyst contents as hyperintense as ventricular CSF. (d) Autopsy specimen showing a small cortical cyst
as the remnant of an old infarction in a similar case.
K.G. Go et al. /Eur. J. Radiol. I7 (1993) 69-84

Arachnoid cysts often present on CT-scans as straight-


edged triangular or rectangular cavities with well defin-
ed walls and a fluid content with the density of CSF
(Fig. 5a). A preferential location is the Sylvian fissure,
while other locations include the cerebral convexity, the
basal or suprasellar region, and the quadrigeminal
region extending into the posterior fossa. The similarity
of the fluid content of arachnoid cysts to CSF might
suggest communication of the cyst cavity with the sub-
arachnoid space. However, many observations have
refuted this contention [9]. After complete emptying of
arachnoid cysts during operation it may be observed
that no fluid is flowing in from adjacent subarachnoid
spaces, which are separated from the cyst cavity by the
translucent cyst walls. Also, if bleeding occurs within the
cyst, the bleeding does not progress into a subarachnoid
haemorrhage, but rather imposes as a subdural
haematoma. According to early studies, air or dyes
which had been introduced into the cyst, did not enter
the CSF spaces [lo]. Radioisotope cisternography and
CT-cisternography using intrathecal contrast agents
Fig. 4. Tt-weighted MR-scan (SE-TR: 955, TE: 20) of a two months demonstrated retarded filling of arachnoid cysts from
old boy with a conically shaped elongated head and decreased motility adjacent subarachnoid spaces (Fig. 6) Ill].
in utero, depicting a large left temporal porencephalic cyst and an
Expansion of the cyst, indentation and displacement
enlarged ventricle, both exhibiting the signal intensity of CSF.
of adjoining (developing) brain structures (‘temporal
lobe agenesis’), or at least the failure of the cyst to
B. Cysts with jluid secreting walls and CSF-like content collapse, all point to the existence of a mechanism that
These cyst cavities seem to be maintained by some maintains or increases its fluid volume. It has been pro-
mechanism which replenishes their content of fluid. posed by Starkman [9], that at an early stage in its
Examples of this category are the arachnoid cysts and development there may be an open communication with
neuroepithelial cysts. the subarachnoid space, allowing inflow of fluid; while

a b C

Fig. 5 (a) CT-scan of an arachnoid cyst in a 12-year-old boy with typical rectangular shape. (b) Tt-weighted MR-scan (SE-TR: 685, TE: 20 l/l)
showing its contents with signal intensity similar to CSF. (c) T2-weighted MR-scan (SE-TR: 2309, TE: 110 2/2) presenting its contents as
hyperintense as CSF.
KG. Go et ul. /Eur. J. Radiol. 17 119931 69-84 73

Furthermore, subdural neurothelium has been


observed as constituents of arachnoid villi and granula-
tions. In considering mechanisms of fluid secretion,
there is the possibility of a mechanism of fluid secretion
involving Na+/K+-ATPase. Using the K-NPPase reac-
tion (a reaction that is based upon the conversion of K-
nitrophenylphosphate (K-NPP) by Na+/K+ATPase
into an electron-dense reaction product that can be
visualised by electron microscopy), enzyme ultracyto-
chemistry has shown the presence of Na+/K+ATPase in
the luminal plasma membrane of the cells lining
arachnoid cysts (Fig. 7) as well as those covering
arachnoid granulations [ 1,191.
In addition to the membrane Na+/K+ATPase, usual-
ly other mechanisms are involved in processes of fluid
transport and cell volume regulation, such as ion-
exchange and cotransport mechanisms in the cell mem-
brane, although the Na+/K+ATPase is generally
a b regarded as the mechanism that provides the driving
force of the transport [20]. It may be envisaged, that in
Fig. 6 (a) Delayed tilling of a typical rectangular arachnoid cyst in a the process of fluid secretion allegedly the
25-year-old female: l/2 h after intrathecal administration of contrast Na+/K+ATPase of the cyst wall transports Na+ from
agent there is hardly contrast filling of the cyst (19 HU), contrary to
the cell contents into the cyst lumen, with the Na+ then
the adjacent basal cisterns (192 HU). (b) Delayed emptying of the
same arachnoid cyst: 24 h after intrathecal contrast administration attracting osmotically obliged water to form the secreted
there is retention of contrast agent in the cyst (38 HU). whereas con- fluid. The efflux of Na+ proceeds at the expense of
trast has decreased more in the basal cisterns (25 HU). ATP and in exchange with K+, in a ratio of 2 K+ enter-
ing against 3 Na+ leaving the cell. Cellular Na+ is
sequestration may follow at a later stage by closure of replenished by a Na+/H+ ion-exchange mechanism
the opening. This mechanism would, however, not which supplies Na+ in exchange for H+. The H+ may
explain long-term maintenance or expansion of fluid be provided by the carbonic anhydrase reaction:
volume. H,O + CO2 - H+ + HC03-, in which H+ and HCO?-
Valvular mechanisms have also been suggested, e.g. are formed from HZ0 and C02. The HC03- con-
by way of a single small opening serving both as an comitantly formed with H’, may be exchanged against
entrance and an exit of fluid [12], presumably with the
CSF pulse pressure being the driving force. It is, how-
ever, difficult to envision how the CSF pulse pressure
can act as a driving force and achieve a fluid accumula-
tion, when the latter may cause an elevated intracranial
pressure that exceeds the driving force.
Finally, there is the possibility of secretion of fluid by
the cyst walls, which may occur by a mechanism similar
to that of CSF absorption, as has been considered by
Krawchenko and Collins [ 131in a significant analysis of
conceivable mechanisms.
By virtue of several characteristics, such as the posses-
sion of desmosomal junctions, -tonoIilaments in their
cytoplasm (of the type of vimentin-positive intermediate
filaments [ 141, interdigitating cytoplasmic processes,
large cytoplasmic vacuoles and pinocytotic vesicles, and
an underlying basal lamina [15], the cells lining
arachnoid cysts have been recognised to resemble mor-
phologically those of the outer layer of the arachnoid Fig. 7. K+-NPPase immunocytochemistry showing Na+/K+-ATPase
activity (R) in the luminal plasmamembrane of the cellular lining of an
mater bordering the dura, designated as the subdural
arachnoid cyst, which comprises several layers of subdural neuro-
neurothelium [ 16,171. Like meningeomas, the cellular thelial cells. These present desmosomal junctions (arrow), inter-
lining of arachnoid cysts appear to possess progesterone digitating cellular processes, cytoplasmic vacuoles, and dilated
rather than estrogen receptors [ 181. intercellular clefts: L..,cvst lumen. Scale bar: I urn.
K.G. Go et al. /Eur. J. Radiol. 17 (1993) 69-84

parenchyma and they have a lining that consists of epen-


dyma or choroid plexus [22-291. From the resemblance
of the fluid contents to CSF it may be presumed that in
these cysts as well the mechanism of secretion is similar
to that in arachnoid cysts. Neuroepithelial cysts contain-
ing choroid epithelium may be immunohistochemically
identified by their immunoreactivity to CEA, S-100
protein and transthyretin [21].

C. Cysts associated with developmental malformations


The content of these cysts also consists of CSF.
Dandy- Walker cysts constitute cystic dilatations of
the fourth ventricle (Fig. 9), with dysgenesis of the
cerebellar vermis, as a result of atresia of the foramina
of Magendie and Luschka; consequently there is
hydrocephalus involving the other ventricles. Often
there are other developmental malformations such as
agenesis of the corpus callosum [30-321. The relevance
of foraminal atresia as a pathogenetic factor has been
Fig. 8. Schematic presentation of alleged mechanisms of fluid secretion doubted, because the foramina have occasionally been
in the lining of an arachnoid cyst. In the process of fluid secretion observed to be patent, while shunting of the fourth ven-
Na+ is transported out of the cell by the Na+/K+ATPase (4) and
together with K+ and Cl- by the Na+K+2Cl--cotransport mechanism
tricle has often not been capable to arrest lethal progres-
(5), the Na+ is replenished by the Na+/H+ exchange mechanism (2) sion. The observation of a cystic dilatation of the fourth
and the Cl- by the anion exchange mechanism (3), by exchange ventricle with patency of the foramina, however, may
against H+ and Cl‘, respectively, which are formed from Hz0 and suggest a neuroepithelial cyst within the fourth ventricle
CO, catalysed by the carbonic anhydrase (1).

Cl- by way of a Cl-/HCOs- anion-exchange mecha-


nism. This provides Cl- that (for the sake of elec-
troneutrality) accompanies the Na+ efflux, or is
associated with both a Na+ and K+ efflux by way of a
Na+K+/Z Cl- cotransport mechanism (Fig. 8). A bio-
chemical mechanism of fluid secretion that is inherently
independent of pressure may well account for the de-
velopment of fluid accumulations that may eventually
result in elevation of intracranial pressure. In contrast to
the morphological situation in arachnoid villi or
granulations, in which CSF is transported from the sub-
arachnoid space into the lumen of a venous vessel,
arachnoid cysts lack the blood circulation as the
drainage pathway, and therefore the transported fluid
accumulates in the cyst cavity in the same way as epider-
moid cysts are formed by accumulation of desquamated
keratin. The situation may arise as a developmental
defect of the leptomeninges, in the sense that rudiments
of arachnoid villi are developing without the establish-
ment of a connection to draining veins. Arachnoid cysts
may be immunohistochemically differentiated from
other cysts by their lack of immunoreactivity to glial
fibrillar acidic protein, carcinoembryonic antigen
(CEA), S-100 protein, and transthyretin (prealbumin)
1211.
Neuroepithelial cysts (or ependymal cysts) also contain Fig. 9. Autopsy specimen of a Dandy-Walker cyst with its occluding
fluid similar to CSF. They may be located in a range membranes (arrows) at the foramina of Magendie and Luschka
from the cerebral convexity to deep within the brain removed.
K.G Go et al. /Eur. J. Radiol. 17 (1993) 69-84 75

a b
Fig. 10. (a) CT-scan in a 64-year-old male. depicting a typical isodense colloid cyst in the anterosuperior angle of the third ventricle, which has
caused hydrocephalus. (b) T,-weighted MR-scan (SE-TR: 775, TE: 20, l/l) presenting the cyst with much higher signal intensity than CSF. (c) T?-
weighted MR-scan (SE-TR: 2649, TE: 50. l/2) showing the hyperintense cyst contents with characteristic central darkening due to paramagnetic
effects.

rather than a Dandy-Walker cyst [29]. The inefficacy of values less or similar to those of brain tissue, making
shunting of the fourth ventricle in relieving the them hyperintense on Ti-weighted images (Fig. lob).
hydrocephalus has been reported to be due to kinking or Usually they have prolonged T,-values, which cause
compression of the aqueduct when the cysts were large them to appear hyperintense on Tz-weighted images [2],
[30], and lethal progression may well be accounted for but characteristically many of them present with a cen-
by severe dysgenetic changes such as absence of vital tral hypointense area consistent with a Tz-shortening
structures in the medulla oblongata [6]. (Fig. lOc), which has been ascribed to paramagnetic
Interhemispheric cysts are generally associated with effects of the mutinous contents [40]. These features
partial or total agenesis of the corpus callosum [32], would for example be most consistent with the hypo-
while their lining has been reported to constitute a vari- thetical presence of an iron-sulfur containing protein
ety of constituents such as arachnoid, glia, ependyma or constituent.
choroid plexus [33]. In other words, these cysts may well Far less frequent are colloid cysts which originate be-
belong to the arachnoid or neuroepithelial categories. tween both leaves of the septum pellucidum, and which
occasionally protrude into a lateral ventricle [30].
2.2. Cysts with a lining of non-neural epithelium Symptomatic Rathke’s cleft cysts derive from
Rathke’s pouch, but are much less frequent in incidence.
Their contents usually bear no resemblance to CSF. They tend to be located within the sella but often show
CoIZoid cysts of the third ventricle have a lining which a suprasellar extension. They contain white mucous
has been considered as entodermal, with two types of fluid, while their lining has been described to consist of
cells, a darker non-ciliated and a lighter ciliated cell, ciliated as well as non-ciliated squamous or cuboidal
while their contents is described as mutinous [34-361. epithelial cells and mucous containing goblet cells [41].
On CT-scans (Fig. 10a) they have the characteristic On CT-scans they present as homogeneous hypodense
appearance of isodense to hyperdense, homogeneous lesions. On MR-scans they have been reported to exhibit
spherical lesions at the foramen of Monro [37]; the high the same intensity as CSF on both Ti- and Tz-weighted
attenuation value has been ascribed to an increase in images [42]. This is quite unexpected in view of the mu-
electron density of their mutinous contents rather than cinous contents, which would rather favour relaxation
to a prevalence of elements with high atomic number characteristics similar to those of proteinaceous cysts
[38], although a case of a colloid cyst has been reported (Fig. 1la-c).
to contain sodium, magnesium and calcium in small Cysts associated with craniopharyngiomas contain the
amounts, and silicium, copper, iron and aluminium in typical yellow to brownish fluid with suspended glitter-
trace amounts [39]. On MR-scans they exhibit short T,- ing cholesterol crystals (Fig. 12) colloquially described
KG. Go et al. /Eur. J. Radiol. 17 (1993) 69-84

a b C

Fig. 11.(a) sagittal Tr-weighted MR-scan (SE-TR: 650, TE: 20 l/l) of a Rathke’s cleft cyst in a 47-year-old female with hypopituitarism, showing
like an intrasellar hyperintense lesion (arrow) with suprasellar extension (arrowhead). The strong T,-shortening may be due to the highly concen-
trated constituents, since during operation the lesion appeared to consist of a capsule enclosing a turbid mass with a viscous to pastelike consistence.
(b) Coronal T,-weighted MR-scan (SE-TR: 2000, TE: 40 l/2) of the Rathke’s cleft cyst (arrow), turning up slightly hypointense. (c) Histology of
the lining of the Rathke’s cleft cyst, showing a lining of ciliated columnar epithelium. Haematoxylin and eosin stain. Scale bar: 100 pm.

as motor oil [43]. They are located in the suprasellar On MR images (Fig. 13c,d) they are characterised by
region, and their lining consists of epithelium considered considerably prolonged Ti- (around 1000 ms) and Tz-
to originate from Rathke’s pouch (Fig. 13a). On CT- relaxation times (around 500 ms) [45].
scans they exhibit attenuation values ranging between Epidermoid cysts also have a lining of stratified
those of CSF and brain, with calcifications (Fig. 13b) squamous epithelium lacking the dermal structures like
being more often in children; contrast administration sebaceous glands and hair follicles (Fig. 14a). They have
usually visualises the solid portion of the tumour [44]. a soft creamy content of keratinised material which is
rich in cholesterol and is derived from the desquamating
epidermal cells [46]. Their origin from ectopic rudiments
of epidermis is well illustrated by their occurrence in the
spine as a result of the inadvertent introduction of skin
fragments during lumbar punctures [47]. Intracranial
epidermoid cysts have a preferential location in the
basal regions of the brain, such as the cerebellopontine
angle or the suprasellar area, and are more common off
the midline contrary to dermoid cysts [48]. On CT-scans
(Fig. 14b) epidermoids present as lesions with a density
similar to that of CSF, often with peripheral hyperdense
foci of calcification. Contrast administration does not
seem to cause significant changes of the image. On MR-
scans they exhibit long relaxation times which make
them appear hypointense on Ti-weighted and
hyperintense on T2-weighted images (Fig. 14c,d), with
occasional visualisation of the stratified layers of des-
quamated keratin [48-501.
Dermoid cysts have a lining of stratified squamous
epithelium (Fig. 15a) with underlying dermis that con-
tain sebaceous glands and hair follicles [51]. In their
contents therefore the buttery keratinised debris often
Fig. 12. Cholesterol crystals in the fluid of a craniopharyngioma (uns- includes hairs and the secretion of the sebaceous glands.
tained preparation, viewed with polarised light). Occasionally the cyst may contain an opalescent yellow
K. G Go et cd. /Eur. J. Rudiol. I7 ( 1993) 69-84 77

a b d
Fig. 13. (a) Histology (haematoxylin and eosin stain) of a craniopharyngioma in a 66-year-old male patient. demonstrating islands of simple
squamous epithelium with cyst formation. Scale bar: 100 pm. (b) CT-scan of the same craniopharyngioma. There is a hypodense multilocular cyst
with adjacent calcification (arrow). (c) Tt-weighted MR-scan (SE-TR: 685, TE: 20, l/l) depicting at a different level the hypointense cyst contents
and hyperintense solid portion of the tumour. (d) Tz-weighted MR-scan (SE-TR: 2069, TE: I IO. 212) showing the hyperintense cyst contents. and
the adjacent hypointense calcification.

fluid. Contrary to epidermoid cysts, dermoid cysts have intravenous injection of contrast medium. On MR-scans
a preferential location in the midline. Dermoid cysts they exhibit a signal like that of fat with short Tt and
may be associated with a dermoid sinus. On CT-scans Tz; the lipid of the epidermal stratum corneum from
(Fig. 15b) they appear as irregular but well-defined which the cyst content originates, consists almost
structures with attenuation values in the negative range, exclusively of ceramides, in which very-long-chain
corresponding to those of lipids; often with peripheral (C2+s2) amide-linked fatty acids prevail [52], with
calcifications. There is no significant enhancement upon similar stretches of CHz-groups that produce the fat

+;.
a b d
Fig. 14. (a) Histology (haematoxylin and eosin stain) of an epidermoid cyst in the right cerebellopontine angle in a 47-year-old male, with vertigo.
tinnitus and loss of hearing on the right side. Note the lining of cornifying squamous epithelium. Desquamated horn lamellae are visible on the
luminal side. Scale bar: 100 pm. (b) CT-scan of the epidermoid cyst (A) in the right cerebellopontine angle, density measurement of the cyst gave
a value of around 0 HU. (c) Tr-weighted MR-scan (SE-TR: 650. TE: 30) visualising the epidermoid cyst in the right cerebellopontine angle with
low signal intensity. (d) T,-weighted MR-scan (SE-TR: 2000, TE: 50) of the epidermoid cyst, which now appears hyperintense.
78 KG. Go et al. /Eur. J. Radiol. I7 (1993) 69-84

a b d
Fig. 15. (a) Histology (haematoxylin and eosin stain) of a recurrent dermoid cyst in a 32-year-old female patient, demonstrating desquamation of
keratin from the epidermal surface, sebaceous glands (S) and hair follicles (H) in the dermal layer. Scale bar: 100 pm. (b) CT-scan of the dermoid
cyst with a very low density (-4 HU) compared to that of ventricular CSF (14 HU). (c) Tt-weighted coronal MR-scan (SE-TR: 650, TE: 20, l/l)
showing a hyperintense dermoid cyst content with laminated structure. (d) T,-weighted MR-scan (SE-l-R: 2309, TE: 110, 212) shows the dermoid
cyst with a heterogeneously high signal intensity, surrounded by a hyperintense cisternal space; (far frontally there is a metal artifact from the
previous craniotomy).

signal. Therefore they appear with hyperintensity on amount of protein leakage across the capillary wall that
T,-weighted images (Fig. 15c), while on T2-weighted reduces the effect of M. This is the colloid-osmotic
images (Fig. 15d) they may show variable or in- pressure gradient, i.e. the difference between the colloid-
homogeneous signal intensity, corresponding to variable osmotic pressure exerted by the plasma proteins (II,,)
relaxation times which seem to depend on the propor- favouring fluid absorption, and that exerted by the pro-
tion of the lipid with respect to the protein component tein present in the interstitial space (J&f). Thus reab-
of the desquamated keratin [48,50]. The simultaneous sorption is retarded, when the fluid is rich in proteins
occurrence of a dermoid and an epidermoid cyst in the (exerting an increased JIir), as it is with vasogenic brain
same patient has allowed the comparison of their respec- oedema or in brain tumour-associated cysts. With the
tive MR characteristics [53]. low protein content of normal extracellular fluid or
arachnoid cyst fluid, the colloid-osmotic pressure of the
2.3. Cysts associated with gliomas and other tumours plasma proteins prevail and the interstitial (or cyst) fluid
tends to be reabsorbed [ 191.
These cysts typically contain a yellow-coloured pro- From a pathogenetic point of view, cysts associated
teinaceous fluid, which is not separated from the tissue with gliomas probably derive from the vasogenic brain
by a special lining. A high protein content may oedema surrounding the tumour [54]. Analysis of the
counteract reabsorption of fluid on account of the Starl- proteins contained in tumour cyst fluid has demon-
ing fluid flux equation. The Starling fluid flux equation strated that more than 92% of total cyst fluid protein
comprises: consisted of plasma protein fractions. This implies that
the greater part of the cyst fluid proteins must derive
J, = L,(AP - uAlT>,with AP = P, - Pir, and All = from plasma, while the remainder (less than 8%) may
constitute endogenous brain proteins. If on the other
Qf - npI hand, the cyst fluid had been the result of necrosis,
endogenous proteins would have prevailed. Moreover,
in which J, is the net amount of fluid moving in or out the concentration of various plasma protein fractions
of the capillary; L, being the so-called hydraulic con- within the tumour cyst fluid, when normalised for their
ductivity of the capillary wall for the movement of fluid; plasma concentrations, appeared to be increased by as
AP the hydrostatic pressure gradient, i.e. the difference much as 50-fold with respect to plasma protein fractions
between intracapillary blood pressure (P,) forcing the in normal CSF, which indicates increased barrier perme-
fluid out, and tissue pressure (Pi,) favouring fluid ab- ability. Both observations thus point to breakdown of
sorption into the capillary. u is Staverman’s reflection the blood-brain barrier in relation to tumour-associated
coefficient of the capillary wall for protein, denoting the cysts. Increased blood-brain barrier disruption is com-
K.G. Go et al. /Eur. J. Radiol. 17 (1993) 69-84 19

otherwise the pressure/volume relations would soon be


severely deranged, i.e. intracranial pressure would soon
be considerably elevated. From experimental evidence
the resolution of brain oedema is considered to proceed
by drainage into CSF and by degradation by pro-
liferating glial elements. In this light cyst formation may
indicate a possible deficiency of the resolution mecha-
nism. Another factor to be considered may be the
geometry of cysts, which by their globular shape imply
an unfavourable surface/volume ratio, as resolution may
be assumed to proceed across the surface into the sur-
rounding tissue.
On the other hand, ischaemic/hypoxic factors are un-
doubtedly relevant to account for the gradual liquefac-
tion of tissue in the areas flooded by oedema fluid. In
these regions the density of feeding vessels tend to be
greatly reduced and by the increased distance to the ves-
sels these areas may consequently experience inadequate
b supply of nutrients and oxygen. Significant is the finding
a
of low tissue oxygen pressures in brain tumours,
Fig. 16. (a) CT-scan of a cyst (C) associated with a glioblastoma in a measured with in situ oxygen electrodes [55]. The cyst
56 year old male; there is a vaguely defined area of the cyst wall
(arrow) at the site of the tumour nodule on the PET scan, next to a
cavity (but so may an area of necrosis) typically presents
relatively well-defined area of cyst wall (arrowhead). (b) 18FDG PET as a metabolic void for glucose utilization on 18FDG-
scan visualising a metabolic void which corresponds to the cyst (CL PET scans (Fig. 16b, 17e). On proton MR spectroscopy
and hypermetabolism in the tumour nodule (T). of brain tumours, however, associated cysts exhibit a
high lactate content (Fig. 17b), this has also been
mon in gliomatous tumours, particularly in those of observed in an experimentally implanted glioma [56].
malignant grade, as may be inferred from the degree and Compared to gliomas, metastases and meningiomas
extent of contrast exudation on CT-scans. These areas have a much lower incidence of associated cysts. This
tend to correspond with the regions exhibiting increased may pertain to the nature of gliomas as neural tissue
metabolism, as indicated by an increased glucose con- proper, being endowed with a blood-brain barrier,
sumption rate on ‘*F-fluorodeoxyglucose positron which may be more or less deficient. Also, gliomas are
emission tomography (“FDG-PET) scans and a high in immediate contact with the surrounding tissue, which
(posphoryl)choline content on the proton magnetic allows direct propagation of oedema fluid from the
resonance spectroscopic image (MRSI) (Fig. 16b, 17c,e). tumour into its environment. Metastases, on the other
Blood-brain barrier disruption is considered inherent to hand, constitute alien tissue, and possess their own sys-
the formation of vasogenic brain oedema. Gliomas are tem of capillaries devoid of a blood-brain barrier;
frequently associated with vasogenic brain oedema metastases also immediately border on surrounding
which typically accumulates in white matter, (as brain tissue, which allows propagation of oedema fluid
characterised on CT-scans by their appearance as from the tumour into peritumoural tissue. Meningiomas
hypodense areas with lingerlike extensions conforming are original!y extracerebrally located and they lack a
to the configuration of the white matter). Moreover, blood-brain barrier. Oedema fluid from a meningioma
there is histological evidence of a gradual transition of obviously has to cross separating layers of arachnoid
the spongy appearance of oedematous tissue to the con- and pia mater, before it can enter surrounding brain tis-
tents of the cyst lumen. Also, CT- and MR-scans may sue, and the degree of oedema around a meningioma
show areas of the cyst wall, in which delineation from proved to correlate with evidence of disruption of these
the surrounding oedematous tissue is fuzzy, showing the separating layers [57]. Large cysts are typically associ-
gradual liquefaction of oedematous tissue into cyst con- ated with haemangioblastomas, although there is usual-
tents (Fig. 16a and 17d). Other areas of the cyst wall ly only a moderate amount of oedema. Histologically,
which show sharp delineation on the scans (Fig. 16a, liquefaction of the tissue into microcysts has been
17a) probably visualise those sides of the cyst, which im- observed in these tumours, with confluence into larger
pinge upon the tissue by expansive growth, and which is cystic cavities; a factor predisposing to liquefaction may
consistent with the histological finding of a distinct be hyalinic occlusion of the abnormal capillaries [58,59].
cellular lining. It may generally be assumed, that with Radiologically, cysts containing proteinaceous fluid
longstanding oedema there is some balance between the exhibit prolonged Ti- and T2-values that are less than
rates of oedema formation and oedema resolution, as those of CSF, contrary to cysts containing CSF-like
80 K.G. Go et al. /Eur. J. Radiol. 17 (1993) 69-84

d e
Fig. 17. (a) T2-weighted MR-scan of a right temporal glioblastoma (T) in a 46 year old male containing a cyst (C). SE-TR: 1500, TE: 100). with
region of interest pertaining to the MR-spectroscopic imaging (MRSI) of lactate and choline. Well-defined occipital area of cyst wall (arrowhead).
(b) MRSI of lactate in the same glioblastoma and associated cyst, showing high lactate concentration within the cyst (C) (also visualising fat in
the orbits (B)). (c) Proton MRSI of choline in the same cystic glioblastoma, showing its presence particularly in the tumour (T), but not in the
cyst (C). (d) CT-scan of the cystic glioblastoma following contrast administration. There is enhancement of the cyst walls, part of which shows
a vague delineation from the lumen (arrow). (e) ‘sF-FDG PET-scan of the cystic glioblastoma. The tumour (T) at the frontal side of the cyst wall.
which showed a high choline content on the proton spectroscopic image, and strong enhancement on the CT-scan, also exhibits an increased glucose
consumption, while the cyst (C) appears as a metabolic void.
K.G. Go er trl. /Eur. J. Radioi. 17 (19931 69-84 81

fluid. Consequently, they have a hypointense appear- trast medium shows a well defined and smooth ring of
ance on T,-weighted, but a reasonable brightness on enhancement of uniform thickness (Fig. 18a). Quite
Tz-weighted MR-images [60,2]. The associated blood- often also, the enhancing ring may be irregular in its
brain barrier impairment also characterises them by a outline and thickness [63]. On MR-scans (Fig. 18b,c)
rim of enhancement on intravenous contrast adminis- abscesses are characterised by a zone of peripheral
tration. oedema producing slight hypointensity on T,-weighted,
and strong hyperintensity on Tz-weighted images; the
2.4. C_vstsof infectious origin central area of necrosis exhibiting long relaxation times,
tends to present with hypointensity with the former, and
Although they are usually not classified among cysts, hyperintensity with the latter sequence; on the T,-
brain abscesses are included in the present study because weighted images, however, the capsule of the abscess has
of their aspects which may make them difftcult to dif- similar to slightly higher signal intensity, and on the T1-
ferentiate from cystic tumours. Brain abscesses are weighted images it tends to appear with similar to slight-
cavities filled with liquefied necrotic tissue debris and ly lower signal intensity relative to brain [64].
pus. The central zone of liquefaction is surrounded by ffydatid cysts result from an infestation with the lar-
a zone of granulation tissue with a heavy infiltration by vae of the tapeworm Taenia solium. The solitary or mul-
polymorphonuclear and plasma cells. The zone of tiple cysts may be located in the brain parenchyma, or
granulation tissue may be absent in early acute stages, protrude into the ventricle. The cyst wall has been
but tends to become better defined and to increase in described as a thin translucent vesicular membrane,
thickness in chronic stages, in which the zone of which is actually part of the parasite. It is often sur-
granulation tissue is surrounded by closely packed rounded by concentric layers of gliosis and fibrosis,
astrocytes. In the adjacent white matter there may be se- which is the host tissue’s response, with an inffamma-
vere oedema, which presumably originates in young tory reaction mainly consisting of lymphocytes and plas-
capillaries of the granulation tissue; these are also re- ma cells. There may also be severe associated oedema in
sponsible for the enhancement in radiological studies adjacent areas, which may provoke intracranial hyper-
[61,62]. The classical appearance on CT is that of a tension. The cysts mostly contain a clear fluid, and in
hypodense area, which upon the administration of con- viable cysts scolices of the parasite with attached suckers

a b
Fig. 18. (a) CT-scan of a frontal abscess in a 62 year old male patient, secondary to a septic condition. showing the circular ring enhancement.
(b) Tt-weighted MR-scan (SE-TR: 650, TE: 20 l/l) of the same abscess which appears as a 3-layered structure: a hypointense cavity surrounded
by an isointense capsule and a hypointense outer rim. (c) Tz-weighted MR-scan (SE-TR: 2309, TE: I10 2/2): a hyperintense lesion surrounded by
an isointense capsule and an extensive hyperintense area of oedema. (Motion artefacts are often unavoidable with these restless and very sick
patients).
82 K.G. Go et al. /Eur. J. Radiol. 17 (1993) 69-84

and hooklets may be found attached to the cyst wall when they are compressed by surrounding brain, e.g.
[65-671. On CT-scans they may appear as spherical le- during movements of the head. In terms of craniospinal
sions with a well-defined border, but sometimes also as pressure/volume relations this implies that these lesions
ill-defined lucencies. The fluid content has the same den- do not reduce compliance by acting like solid incom-
sity as CSF. There may be enhancement of the capsule pressible tumors.
or of part of the content. Radiological aspects include In contrast, the other types of cerebral cysts that do
the occurrence of multiple calcifications [68-701. not communicate with the CSF compartment have no
way of emptying their contents upon compression; these
3. Differential diagnosis of cystic lesions lesions behave like real space occupying lesions which
may well reduce cranial compliance. Therefore,
By their CT and MR characteristics similar to those
arachnoid cysts that do not even cause intracranial pres-
of CSF, and by their well-defined appearance, the
sure elevation, may well require surgical treatment by
categories of cysts containing CSF or CSF-like fluid
marsupialization or shunting, when they are responsible
usually present no problem in the differentiation from
for symptoms on account of their behaviour as incom-
other lesions. On Ti-weighted images giant aneurysms
pressible masses that impinge upon the adjacent brain
with their appearance as well-defined areas of hypo-
during movements of the head, or when they interfere
intensity (jlow void) may cause confusion, but the flow
with cerebral development in young children.
void on Tz-weighted images clearly distinguishes them
There are also a number of cysts which on account of
from cysts with CSF-like contents. Confusion may also
their strategic location along the CSF pathways, may
arise, when bleeding has occurred, such as may happen
sooner or later cause obstruction and result in intracra-
within arachnoid cysts. Haemorrhage tends to increase
nial pressure elevation. They include arachnoid cysts in
the attenuation value of the cyst contents on CT-scans,
the suprasellar, pineal and posterior fossa locations,
and to shorten relaxation times, which may make them
Dandy-Walker cysts and craniopharyngiomas, while
resemble the cysts with proteinaceous contents on Tz-
colloid cysts have been reported to be responsible for
weighted images, although their hyperintensity on Ti-
sudden death by obstruction of the foramina of Monro.
weighted images would still differentiate them from pro-
Whereas otherwise the cysts with CSF-like contents
teinaceous cysts.
do not really behave as expanding lesions, those with a
The cysts with a lining of non-neural epithelium may
lining of non-neural epithelium may actually increase in
resemble various neoplastic lesions. Contrary to
size and by compression, invoke dysfunction of adjacent
tumours, however, they usually do not show enhance-
structures, viz. the impairment of pituitary function by
ment upon intravenous contrast administration.
craniopharyngiomas or Rathke’s cleft cysts. Their ex-
The tumour-associated cysts with proteinaceous con-
pansion, however, does not proceed at the rate exhibited
tents may be difficult to delineate from the solid part of
by proteinaceous cysts associated with tumours or by
the tumour, while identification of the cyst cavity may
brain abscesses. Another clinical hazard posed by cysts
be relevant in the management of the neoplasm. For this
lined with non-neural epithelium, such as craniopharyn-
purpose the use of contrast agents, magnetic resonance
giomas, epidermoid and dermoid cysts, is intermittent
spectroscopic imaging and PET-scanning may be useful.
leakage of their irritative contents into CSF spaces,
Brain abscesses may also resemble cystic gliomas, par-
causing an aseptic or chemical meningitis, while der-
ticularly when they do not present as a spherical lesion
moid cysts may cause true bacterial meningitis by exter-
with an enhancing ring of equal thickness, but rather
appear as a cavity with a wavy and fuzzy outline. nal contamination through an associated dermoid sinus
Calcifications may be found with craniopharyngio- [43,71,72].
Cysts that are associated with tumours, such as
mas, epidermoid and dermoid cysts, and with hydatid
gliomas and craniopharyngiomas, provide the oppor-
cysts.
tunity to relieve intracranial hypertension by mere
evacuation of the cyst without the necessity of removing
4. Clinical aspects of cystic lesions
the neoplasm itself. This may be important in cases in
The ex vacua type of cystic lesions typically contain which the patient’s condition does not allow an exten-
CSF, which has flown into the cyst from adjoining sive surgical intervention. Evacuation of the cyst con-
cisterns or ventricles. Because of their genesis from a tents may be accomplished by means of stereotactic
shortage of tissue rather than an excess of mass with re- techniques, and catheters connected to subcutaneous
spect of available space, they do not need measures that reservoirs may be inserted into the cyst, allowing subse-
reduce intracranial pressure, and generally they do not quent repeated percutaneous evacuations. Finally, cysts
require surgical intervention. Moreover, in view of their associated with tumours have been used as reservoirs
communication with normal CSF spaces, ex vacua into which cancer chemotherapeutic drugs, or antibody-
cystic lesions can empty their contents into these spaces bound tumouricidal agents can be introduced.
K.G. Go et al. /Eur. J. Radiol. I7 (1993) 69-84 83

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