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Diseases of the Sella and

Parasellar Region: An Overview


Anna Derman, MD, Marisa Shields, Adam Davis, MD, Edmond Knopp, MD,
and Girish M. Fatterpekar, MBBS

T he sella and parasellar regions have intricate anatomy


and can be affected by diverse pathology. The current
article reviews the anatomy of this region and outlines the
tropic hormone, prolactin, thyroid-stimulating hormone, lu-
teinizing hormone, and follicle-stimulating hormone. The
pars intermedia is positioned between the pars distalis and
imaging findings of common sellar and parasellar disease the posterior lobe of the pituitary.4 The neurohypophysis
entities. contains the posterior lobe of the pituitary, the infundibular
stem, and the median eminence of the hypothalamus. Vaso-
Anatomy of the Pituitary, pressin and oxytocin are produced in the hypothalamus, and
they are directly transported via the infundibulum to the
Parasellar Regions, and posterior pituitary where they get stored. The posterior pitu-
the Central Skull Base itary and the infundibulum have a direct blood supply from
The central skull base is formed by the posterior aspect of the the inferior hypophyseal artery, whereas the anterior pitu-
presphenoid anteriorly, the basisphenoid centrally, the itary has an indirect blood supply from hypophyseal portal
greater wings of sphenoid laterally, and the dorsum sella and system.5
petrous ridges posteriorly. The sella turcica is a saddle- The pituitary gland is bounded superiorly by the dia-
shaped depression in the basisphenoid and houses the pitu- phragma sella. An opening in the diaphragma sella conducts
itary gland.1 the pituitary stalk or the infundibulum. Above the pituitary
The pituitary gland largely consists of 2 parts: the anterior fossa is the suprasellar cistern containing the optic chiasm,
pituitary or adenohypophysis, and posterior pituitary or neu- the A1 segments of the anterior cerebral arteries, and the
rohypophysis. The adenohypophysis starts as an evagination anterior communicating artery.
of the primitive fetal oral ectoderm (or Rathke’s pouch) that The cavernous sinuses are present along either side of the
migrates from the roof of the primitive oral cavity superiorly pituitary fossa, extending from the orbital apex to the Meck-
into the middle cranial fossa to form the adenohypophysis.2 el’s cave. They are extradural valveless venous structures con-
Majority of the anterior pituitary develops from the anterior tained between the temporal lobe dura (dura propria) and the
wall of the Rathke’s pouch. The neurohypophysis, in turn, periosteal dura. The cavernous sinuses receive venous inflow
forms as an outgrowth of the diencephalon. Subsequently, from the superior and inferior ophthalmic veins and the
the anterior and posterior lobes fuse to form the normal pi- sphenoparietal sinus. They are interconnected anteriorly and
tuitary gland, and the Rathke’s pouch regresses. Abnormal posteriorly. Cavernous sinuses drain posteriorly into the
persistence and enlargement of the Rathke’s cleft (Rathke’s transverse sinuses via the superior petrosal sinuses and infe-
pouch remnant) results in Rathke cleft cyst (RCC).3 riorly into the pterygoid plexus via emissary veins.
The anterior pituitary is made up of 3 parts: the pars tube- Centrally within the cavernous sinuses are the cavernous
ralis, pars intermedia, and pars distalis. The pars tuberalis is segments of the internal carotid arteries surrounded by sym-
a small amount of adenohypophyseal tissue that wraps ante- pathetic nerve fibers. Centrally within the cavernous sinuses
riorly along the pituitary stalk. The pars distalis forms the and inferolateral to the internal carotid arteries are the cranial
main body of the anterior pituitary. It produces multiple nerves (CN) VI. Structures coursing laterally along the dural
hormones, including the growth hormone, adrenocortico- wall of the cavernous sinus, from cranial to caudal, include
CN III, IV, V1, and V2 nerves (Fig. 1). Small fat deposits can
sometimes be seen within the cavernous sinuses.
Department of Radiology, NYU Langone Medical Center, New York, NY.
Address reprint requests to Girish Fatterpekar, MBBS, Department of Radi-
The Meckel’s cave sits within a dural reflection along the
ology, NYU Langone Medical Center, New York, NY 10016. E-mail: inferolateral aspect of the cavernous sinuses. Unlike V1 and
Girish.Fatterpekar@nyumc.org V2 divisions, the V3 division bypasses the cavernous sinus

0037-198X/13/$-see front matter © 2013 Elsevier Inc. All rights reserved. 35


http://dx.doi.org/10.1053/j.ro.2012.08.001
36 A. Derman et al

Imaging Protocol
Sellar and parasellar imaging may be sought in patients with
endocrinologic abnormality, cranial nerve III, IV, V, and VI
deficits, and symptoms related to local suprasellar mass ef-
fect, such as visual field cuts.
Dynamic contrast-enhanced magnetic resonance imaging
(MRI) is the preferred method of pituitary imaging at our
institution. High-resolution coronal T1- and T2-weighted
images (WIs) of the pituitary fossa from the orbital apex to
the prepontine cistern are followed by dynamic postcontrast
images of the pituitary fossa in the coronal plane. Delayed
high-resolution postcontrast T1WI of the pituitary fossa are
obtained in the sagittal plane. This study can be supple-
mented with diffusion-weighted imaging (DWI), corre-
sponding apparent diffusion-coefficient maps, and postcon-
trast axial T1WI and fluid-attenuation inversion recovery
sequences of the brain. In addition, evaluation of the cavern-
ous sinuses may be enhanced by adding a fat-saturated con-
trast-enhanced T1W sequence in the coronal or axial plane.
Depending on the indication, additional sequences that can
Figure 1 Normal anatomy of the sella: High-resolution coronal
be obtained include thin-section postcontrast axial images of
T2WI demonstrates normal anatomy of the sella and the parasellar
the brain acquired by 3D-spoiled-gradient technique. Thin-
region. P, pituitary gland; I, infundibulum; OC, Optic chiasm; III,
oculomotor nerve III; IV, trochlear nerve; V1, ophthalmic (first) section 3D heavily T2WIs (constructive interference in
division of trigeminal nerve; V2, maxillary (second) division of tri- steady state may be used to improve visualization of the cra-
geminal nerve; and VI, abducens nerve. (Courtesy: Dr Nafi Aygun, nial nerves.
Johns Hopkins Medical Center, Baltimore, MD.) Multidetector computed tomography (CT) may be used as
an adjunct tool to MR. It is particularly advantageous when
evaluating for tumoral calcifications, and to evaluate for hy-
perostosis, or bone erosion.
diving directly into the foramen ovale to gain access into the
masticator space.
Multiple skull base foramina surround the cavernous si- Age-Appropriate
nuses. Superomedial to the cavernous sinuses are the optic
canals transmitting the optic nerves and the ophthalmic ar- Imaging Appearance
tery. Anterior to the cavernous sinuses, a cleft between the of the Normal Pituitary Gland
greater and lesser wings of the sphenoid bone forms the In the fetus and neonate, the entire pituitary gland is T1
superior orbital fissure that conducts the CN III, IV, V1, VI, hyperintense, and it demonstrates a convex superior border.
and the superior ophthalmic vein. The inferior orbital fissure At 2 months of age, the pituitary assumes its normal expected
is bounded above by the greater wing of the sphenoid, below configuration and becomes concave superiorly. At about the
by the maxilla and the orbital process of the palatine bone, same time, the anterior pituitary gland becomes isointense to
and laterally by the zygomatic bone. The inferior orbital fis- brain on T1WI.6 The posterior pituitary remains hyperin-
sure connects the orbit with the pterygopalatine and infra- tense on T1WI owing to its neurosecretory granules.7 An
temporal fossae and transmits the infraorbital and zygomatic absent posterior pituitary bright spot should be considered
branches of the maxillary nerve, orbital rami from the ptery- abnormal, only when associated with an ectopic bright spot,
gopalatine ganglion, and accompanying vessels. Posterior to often in the suprasellar region (usually associated with an
the orbital fissures, and within the lateral wall of the sphenoid absent infundibulum, and hypoplastic anterior pituitary
sinuses, is the foramen rotundum transmitting CN V2, its gland), or when associated with appropriate endocrine ab-
artery and veins. Posterior and lateral to foramen rotundum normalities, such as diabetes insipidus. Absence of the pitu-
is the foramen ovale transmitting CN V3, lesser petrosal itary bright spot may be seen because of mechanical obstruc-
nerve, artery, and veins. Foramen spinosum is a small ovoid tion of neurosecretory granules transport in the
foramen posterolateral to the foramen ovale, which conducts infundibulum. It is most often seen in the setting of hypotha-
the middle meningeal artery. Foramen lacerum is a space lamic/infundibular tumoral disease, most commonly germi-
between the temporal and sphenoid bones posteromedial to noma. Granulomatous diseases, such as Langerhans cell his-
the foramen ovale, which conducts the internal carotid ar- tiocytosis (LCH) and lymphocytic hypophysitis, may also
tery, and is partially filled with cartilage. Inferomedial to the mechanically obstruct the infundibulum and/or result in au-
foramen rotundum and along the floor of the sphenoid sinus toimmune destruction of the hypothalamic neurons of the
is the vidian canal transmitting the vidian artery and nerve. neurohypophysis. It should be noted that absence of the
Imaging the sella and parasellar region 37

posterior pituitary bright spot may be seen in asymptomatic higher at puberty.6 The height of the pituitary may be
normal individuals without structural abnormality. It is exaggerated by a shallow configuration of the sella turcica.
therefore important to correlate an absent bright posterior The pituitary stalk is a linear structure, extending from the
pituitary spot with appropriate symptomatology. median eminence of the hypothalamus to the pituitary
The normal pituitary measures up to 2-6 mm in height gland. It measures approximately 2.8 mm in transaxial
during childhood, and grows up to 10 mm or slightly diameter near its midpoint, and demonstrates minimal

Figure 2 Pituitary microadenoma seen in a 31-year-old woman with prolactin levels ⬎150 ng/mL. (A) Contrast-
enhanced coronal T1WI during early dynamic phase demonstrates a well-defined, spherical, sub-cm area (arrow)
enhancing less than the surrounding normal pituitary gland. In a different patient, pituitary macroadenoma in a
38-year-old woman with headache and visual disturbance. (B) Contrast-enhanced coronal T1WI image demonstrates
an enhancing sellar mass extending into the suprasellar compartment. The normal pituitary is not identified. The mass
encases the cavernous segment of the right internal carotid artery by 55% (arrow) consistent with cavernous sinus
invasion. Curved arrow denotes the mass effect on the optic chiasm. Pituitary apoplexy seen in a 33-year-old woman,
with known history of pituitary adenoma, who now presents with sudden onset of visual disturbance and headache. (C)
Noncontrast sagittal T1WI demonstrates a T1 hyperintense sellar mass with suprasellar extension and optic chiasm
indentation (arrow). Note the fluid-fluid level suggestive of recent hemorrhage.
38 A. Derman et al

Figure 3 Spectrum of Rathke cleft cysts: (A) Sagittal T1WI demon-


strates an ovoid, hyperintense, intrasellar lesion (arrow) in the pos-
Figure 4 Spontaneous intracranial hypotension in a patient with
terior aspect of the adenohypophysis. (B) Sagittal T1WI demon-
Ehlers–Danlos syndrome: (A) Sagittal contrast-enhanced T1WI
strates a rounded hyperintense lesion predominantly within the
demonstrates mild prominence of the pituitary gland. There is as-
suprasellar cistern. (C) Sagittal T1WI demonstrates an ovoid, hy-
sociated sagging of the posterior fossa structures. (B) High-resolu-
perintense, predominantly suprasellar lesion with a small intrasellar
tion coronal T2WI (MR myelogram) through the thoracolumbar
component. Note the mild residual pituitary tissue draped along the
spine demonstrates multiple arachnoid diverticula (arrows) and ad-
anterior-inferior aspect of the lesion (arrow).
ditional bright signal intensity foci (arrow heads) in the paraspinal
region suggestive of CSF leak.
Imaging the sella and parasellar region 39

Figure 5 Epidermoid: (A) Axial T2WI demonstrates an extra-axial


hyperintense lesion (arrow) within the left quadrigeminal plate cis-
tern insinuating into the left ambient cistern, suprasellar cistern, and Figure 6 A 2-year-old boy with precocious puberty: (A) Coronal
the proximal left sylvian fissure. Note that the lesion encases the T1WI demonstrates a suprasellar mass (arrow) isointense to gray
Circle of Willis without narrowing or displacing the individual ves- matter abutting the tuber cinereum. (B) Sagittal constructive inter-
sels. (B) Axial fluid-attenuation inversion recovery image demon- ference in steady-state image demonstrates the mass (arrow) to
strates that the lesion is slightly hyperintense than CSF. (C) Axial bulge into the suprasellar cistern posterior to the pituitary stalk. (C)
DWI demonstrates abnormal increased signal within the lesion. No Contrast-enhanced sagittal T1WI does not demonstrate any en-
enhancement was seen after contrast administration (not shown). hancement of the lesion. Diagnosis: Tuber cinereum hamartoma.
40 A. Derman et al

Figure 7 A 13-year-old boy with adamantinomatous cranio-


pharyngioma: (A) Axial noncontrast CT scan, soft-tissue
window, demonstrates low attenuation suprasellar mass
with coarse peripheral calcification (arrow). (B) Axial T1WI
demonstrates the mass to be intrinsically hyperintense. (C)
Contrast-enhanced sagittal T1WI demonstrates sellar-su-
prasellar mass. It is difficult to assess for any enhancement
because of the intrinsic T1 hyperintensity of this lesion. A
73-year-old man with a papillary craniopharyngioma. (D)
Axial T2WI demonstrates a markedly T2 hyperintense su-
prasellar mass (arrow) with a central nodular component
(curved arrow). (E) Contrast-enhanced sagittal T1WI dem-
onstrates heterogeneously (central nodular and peripheral
ring-like) enhancing suprasellar mass.
Imaging the sella and parasellar region 41

widening superiorly, where it measures approximately Pituitary Apoplexy and Infarction


3.5 mm.8 Pituitary apoplexy or more precisely “pituitary tumor apo-
plexy” is an acute hemorrhage or infarction within a preex-
isting pituitary adenoma. It is a clinical syndrome character-
Pathology of Sella ized by acute onset of headache, diplopia, and decreased
and Parasellar Region visual acuity because of mass effect on optic chiasm, mental
status change, and variable endocrine abnormality.11 Imag-
Pituitary Adenoma ing features include sudden expansion of the previously
Pituitary adenoma is the most common intrasellar mass. De- known adenoma with hemorrhage within it. Fluid-fluid level
pending on its size, it can be classified into microadenoma is often demonstrated on MRI (Fig. 2C). Indentation on the
and macroadenoma. optic nerve is commonly seen. Sheehan syndrome, unlike
Pituitary microadenoma is a benign tumor, measuring ⬍1 pituitary tumor apoplexy, is infarction of normal pituitary
cm in size. It may be symptomatic or completely asymptom- tissue often seen in the postpartum period because of exten-
atic and incidental.5 When symptomatic, which is often the sive hypotension. The pituitary gland in such cases may show
case, it produces endocrine abnormalities. Prolactinoma is
peripheral enhancement and no central enhancement. Asso-
the most common type of pituitary microadenoma, and usu-
ciated hyperemia of the sphenoid sinus mucosa may be
ally is seen with prolactin levels ⬎150 ng/mL.5 This results in
seen.12 Chronic stages often demonstrate volume loss of the
menstrual irregularities and galactorrhea. Other relatively
pituitary gland.
common microadenomas include growth hormone-secreting
adenoma, which results in acromegaly, and adrenocortico-
tropic hormone (ACTH)-producing adenoma, which results Empty Sella
in Cushing syndrome. The microadenoma is usually hypoin-
Empty sella is a condition in which the pituitary is flattened
tense or isointense to the pituitary on T1WI, and enhances
against the floor of the sella by herniating arachnoid with
less than the normal pituitary gland during early dynamic
cerebrospinal fluid (CSF). This may be a normal variant or
phase imaging, gradually increasing in contrast retention
may be associated with pseudotumor cerebri and may resolve
over a period (Fig. 2A). In fact, after approximately 60 sec-
after treatment. The sella may or may not be enlarged by the
onds or longer, the microadenoma may retain more contrast
CSF pulsations. The pituitary enhances normally in individ-
than the surrounding pituitary, as the normal gland washes
uals with partially empty sella.
out contrast sooner than the microadenoma.7 Pars interme-
dia cysts, otherwise known as colloid cysts, can mimic pitu-
itary microadenoma. These are often asymptomatic, small, Rathke Cleft Cysts
sub-cm, nonenhancing lesions centered between the anterior
RCCs are cystic, midline, sellar, and/or suprasellar lesions
and posterior lobes of the pituitary, often appearing hypoin-
lined by cuboidal epithelium. They may be a few millimeters
tense on T1WI and hyperintense on T2WI.9
to several centimeters in size. RCCs occur owing to incom-
Pituitary macroadenomas, by definition, measure ⬎1 cm in
plete obliteration of the primitive craniopharyngeal duct. The
size. These tumors are present because of mass effect on adjacent
cyst does not enhance, but the normal pituitary tissue draped
structures or because of endocrine abnormalities. The tumor
over the cyst margin does, sometimes making it difficult to dis-
follows gray matter on all pulse sequences, and the normal pi-
tuitary parenchyma is indistinguishable from the tumor on im- tinguish it from an adenoma. RCCs demonstrate variable signal
aging. Macroadenomas with suprasellar extension assume a fig- intensity on MRI, depending on the cyst contents (Fig. 3). A T1
ure-of-eight or snowman configuration because of focal hyperintense and T2 hypointense intracystic cholesterol
restriction of tumor expansion at the level of the diaphragma nodule, when seen, is pathognomonic for RCC.13
sella (Fig. 2B). As the tumor enlarges, it encroaches on the
optic chiasm. Macroadenomas can be locally aggressive ex-
Other Miscellaneous
tending into the cavernous sinuses, sphenoid sinus, and the
dorsum sella. Prolactin levels ⬎1000 ng/mL are seen with Conditions Involving the Pituitary Gland
cavernous sinus invasion.5 On invading the cavernous si- Enlargement of the pituitary is also observed with nontu-
nuses, the tumor can encase the internal carotid artery, but moral conditions, such as spontaneous intracranial hypoten-
unlike meningioma, it is less likely to narrow it. A minimum sion, and primary hypothyroidism. However, the presenta-
of 30% encasement of the internal carotid artery has been tion in such clinical scenarios is different. Also, certain
reported to correlate with cavernous sinus invasion.10 additional imaging features, such as sagging of the posterior
Macroadenomas often hemorrhage, particularly when the fossa structures, and enlarged epidural veins observed in a
patient is treated with bromocriptine. Hemorrhagic mac- clinical setting of spontaneous intracranial hypotension can
roadenomas can be T1 and T2 hyperintense because of ex- help in making the correct diagnosis (Fig. 4).
tracellular methemoglobin, and may or may not demonstrate Pituitary abscess is a rare occurrence. It can be seen in
a fluid-fluid level. Macroadenomas can also undergo cystic immunocompromised patients. It results from hematoge-
degeneration. nous spread or spread from adjacent sphenoid sinusitis.
42 A. Derman et al

Suprasellar Pathology the pachy- and leptomeninges. Lipomas are thus maldiffer-
entiated cisternal spaces. Abnormal persistence of the prim-
Cystic-Appearing Lesions itive meninx can result in associated maldevelopment of ad-
Common suprasellar cystic-appearing lesions include RCCs, jacent structures, for example, suprasellar lipomas are often
arachnoid cysts, epidermoid cysts, and dermoid cysts. All of associated with hypoplastic mamillary bodies and tuber ci-
these entities have a specific appearance on CT and MRI, and nereum. Lipomas share similar imaging characteristics to
they do not demonstrate any enhancement after contrast ad- dermoids, including presence of chemical shift artifact be-
ministration. RCCs have been discussed earlier. Arachnoid cause of presence of fat and calcification. However, there are
cysts follow CSF signal on all pulse sequences, including subtle important differences. Like cisternal spaces, intracra-
DWI, and can exert mass effect, remodeling the adjacent nial lipomas respect the traversing vessels, conducting, rather
calvarium from chronic CSF pulsation. Epidermoid cysts and than displacing them; an important distinguishing feature
dermoid cysts both result embryologically from inclusion of from dermoids. Also, lipomas are more homogeneous on
ectoderm into the neural tube. Both cysts are lined by squa- T1WI than dermoid cysts.14
mous epithelium. In addition, dermoid cysts contain hair
follicles and sebaceous elements in their walls. Dermoid cysts Suprasellar Tumors
are usually midline, heterogeneous, fat-containing lesions
Tuber Cinereum Hamartoma
appearing markedly hypodense on CT studies, heteroge-
Tuber cinereum hamartoma is a congenital suprasellar lesion
neous but typically hyperintense on T1WI, and variable sig-
of childhood. There are 2 types of tuber cinereum hamarto-
nal on T2WI. Dermoid cysts can demonstrate signal dropout
on fat-saturated sequences, such as DWI. Epidermoid cysts can mas: sessile and pedunculated. The sessile type is encased in
occur in- or off-midline. They often appear hypodense on CT the hypothalamus and deforms the third ventricle. It results
scans, hypointense on T1WI, and hyperintense on T2WI. How- in gelastic seizures. Pedunculated tuber cinereum hamar-
ever, on fluid-attenuation inversion recovery images, these le- toma attaches to the tuber cinereum by a stalk, and extends
sions typically are hyperintense to CSF. Also, epidermoids clas- inferiorly into the suprasellar cistern posterior to the pituitary
sically demonstrate abnormal increased signal on DWI. They stalk. It results in precocious puberty.15 Tuber cinereum
often insinuate into the adjacent cisterns, surrounding but not hamartomas are largely composed of gray matter and do not
distorting the adjacent blood vessels (Fig. 5). enhance after contrast administration (Fig. 6).

Craniopharyngiomas
Craniopharyngiomas are common tumors of the supra-
Lipoma sellar region. They have a bimodal age distribution and 2
Embryologically, intracranial lipomas are thought to arise distinct histologic subtypes: adamantinomatous and pap-
from maldifferentiation of the primitive meninx, which forms illary. Adamantinomatous tumors occur in children and

Figure 8 6-year-old boy with hypothalamic pilocytic astrocytoma: (A) Sagittal T1WI demonstrates a predominantly
hypointense mass centered in the suprasellar cistern. There are no T1 hyperintense components. Asterisk denotes the
displaced optic chiasm. Arrow points to the displaced normal pituitary tissue. (B) Sagittal postcontrast T1WI demon-
strates heterogenous enhancement.
Imaging the sella and parasellar region 43

adults, and are thought to arise from the primitive ecto-


dermal rests along the Rathke pouch pathway.5 Ada-
mantinomatous tumors are mostly suprasellar in location,
although some such tumors have been reported to be in-
trasellar, mixed intra- and suprasellar, within the third
ventricle, and rarely within the sphenoid sinus. Ada-
mantinomatous craniopharyngioma is a variable T1W sig-
nal intensity uni- or multilocular cystic mass with promi-
nent calcifications, enhancing cyst walls, with or without a
solid enhancing component. The cystic components fre-
quently demonstrate T1 hyperintensity because of pro-
teinaceous contents helping distinguish craniopharyngi-
oma from other cystic tumors16 (Fig. 7A-C). Signal
intensity on T2WI may be hyper- or hypointense depend-
ing on presence of free methemoglobin and protein con-
centration. Vessel encasement is common. Papillary cra-
niopharyngiomas usually occur in adults, and are thought
to arise from cells of the pars tuberalis found along the
pituitary stalk, explaining tumor predilection for the su-
prasellar cistern. A solid, and slightly less often, cystic-
solid mass can be seen. Calcification is rarely seen16 (Fig.
7D and E).

Optic Chiasmatic Astrocytoma


Optic chiasmatic astrocytoma can be sporadic or can be
associated with neurofibromatosis 1 (NF1). In NF1 pa-
tients, the tumor is a pilocytic astrocytoma and carries a
better prognosis, whereas in sporadic cases, the astrocyto-
mas are more aggressive.17 Optic chiasmatic astrocytomas
may be solid appearing, or may have enhancing cystic and
solid components. Occasionally, they can calcify and
mimic craniopharyngiomas. However, unlike craniophar-
yngiomas, the tumor is iso- to hypointense to the brain on
T1WI and never shows any T1 hyperintensity. Variable en-
hancement is seen after contrast administration (Fig. 8).18

Meningioma
Meningioma is a common supra/parasellar tumor, al-
though it occurs most frequently along the greater wing of
the sphenoid. Suprasellar meningiomas may be centered
on the planum sphenoidale, clinoid, tuberculum sella,
diaphragma sella, or a combination of the above. Gener-
ally, the tumor follows gray matter on all pulse sequences,
avidly enhances and demonstrates a dural tail. Hyperosto-
sis is a distinguishing feature. Planum sphenoidale menin-
giomas may cause pneumosinus dilatans. Meningiomas
can invade the cavernous sinuses and may or may not
narrow the internal carotid artery. It should be noted that
narrowing of the internal carotid artery is more often seen
with meningioma than any other sellar/parasellar tumor
(Fig. 9).
Figure 9 Meningioma: (A) Axial T2WI demonstrates a homoge-
Lesions of the Pituitary Hypothalamic Axis neous mass (arrow) isointense to gray matter involving the left cav-
ernous sinus. (B) Axial postcontrast T1WI demonstrates homoge-
Germ Cell Tumors
nous enhancement of this mass. An enhancing dural tail (arrow) is
Germinomas, the most common of the germ cell tumors,
seen extending along the left petroclinoid ligament. (C) Contrast-
occur predominantly in adolescent males, usually in the enhanced coronal T1WI demonstrates narrowing of the cavernous
pineal and suprasellar regions. Patients often present with segment of the left internal carotid artery (arrow).
central diabetes insipidus. On imaging, the tumor is seen
44 A. Derman et al

Figure 10 Spectrum of pituitary hypothalamic axis lesions: A


6-year-old boy with suprasellar germinoma and central di-
abetes insipidus. (A) Coronal postcontrast T1WI demon-
strates abnormal thickening and enhancement of the pitu-
itary stalk (arrow). A 13-year-old girl with suprasellar LCH
and central diabetes insipidus. (B) Sagittal postcontrast
T1WI demonstrates abnormally thickened enhancing pitu-
itary stalk (arrow). A 23-year-old woman with hypopituitar-
ism secondary to lymphocytic hypophysitis. (C) Sagittal
T1WI demonstrates mild enlargement of the pituitary and
abnormal marrow signal in the basisphenoid (arrow). (D)
Coronal T2WI demonstrates a rim of peripheral hypointen-
sity (bold arrow) surrounding the enlarged pituitary gland.
(E) Sagittal postcontrast fluid-attenuation inversion recov-
ery image demonstrates enhancing sellar-suprasellar lesion,
with ill-defined enhancement within the basisphenoid.
Imaging the sella and parasellar region 45

as a small homogeneously enhancing mass centered in the mic infiltrates to the pituitary have also been reported.26,27
pituitary stalk. The normal posterior pituitary bright spot Primary pituitary hypothalamic axis lymphoma has also
is often absent (Fig. 10A). This imaging presentation looks been described.28,29
identical to LCH or lymphocytic hypophysitis.19 Exten-
sion superiorly to involve the hypothalamus can be seen. Cavernous Sinus Disease
Because of a high cellularity and chromaffin-to-cytoplasm A myriad of malignant, inflammatory, infectious, and vas-
ratio, germinomas can sometimes demonstrate restricted cular lesions occur in the cavernous sinuses. Clinically,
diffusion on DWI. Germinomas spread through CSF seed- mass effect on the anatomic structures coursing centrally
ing, thus it is important to interrogate the craniospinal axis within the cavernous sinus or within the cavernous sinus
for leptomeningeal metastasis. walls can result in a cavernous sinus syndrome. Cavernous
Langerhans Cell Histiocytosis sinus syndrome is a combination of chemosis, extraocular
LCH is a systemic disease. Intracranial involvement with- muscle ophthalmoplegia, Horner syndrome, with or with-
out skeletal involvement elsewhere in the body is uncom- out pupillary function abnormality, and variable facial
mon. Imaging features are similar to germinoma and in- sensory loss in the V1 and V2 distribution.30
clude an enhancing suprasellar mass in the region of the
pituitary stalk (Fig. 10B). The normal posterior pituitary Cavernous Sinus Tumors
bright spot is often absent.20 The lesion can also increase in Schwannoma
size and extend into the hypothalamus.19 Schwannoma is the most common tumor observed in the
region of the cavernous sinus. Of the schwannomas, tri-
Lymphocytic Hypophysitis geminal nerve schwannoma is most common. Oculomo-
Lymphocytic hypophysitis is a chronic inflammation of tor, trochlear, and abducens nerve schwannomas are less
the pituitary axis. It usually occurs in women during late common. Schwannomas appear hypo- to isodense on non-
pregnancy or postpartum period, but can sometimes occur contrast CT images. On MR, schwannomas typically ap-
in men and children. Endocrine workup commonly shows pear as well-defined lobulated masses, hyperintense on
deficiency of ACTH in adults and deficiency of growth T2WI, and demonstrate moderate enhancement (Fig. 11).
hormone in children.21 The lesion is mildly hypointense Rarely, these tumors can be cystic. Intratumoral hemor-
on T1WI, iso-hyperintense on T2WI, and enhances after rhage is extremely uncommon. Depending on their origin
contrast administration. A thin rim of hypointense signal from the underlying nerve, they can involve the Meckel’s
can be seen surrounding the gland or along the cavernous cave, expand the underlying foramina, extend into multi-
sinuses, thought to be because of fibrotic changes associ- ple cranial compartments, and sometimes extend extracra-
ated with infiltration of lymphoplasmacytic cells.22 In- nially as well.31
flammation can extend into the cavernous sinuses and
sphenoid sinus mucosa.5 Associated basisphenoid osteitis Meningiomas
is sine qua non of this lesion (Fig. 10C-E). Meningiomas are dural-based lesions commonly observed
along the lateral wall of the cavernous sinus, and can extend
Sarcoidosis into the cavernous sinuses. The tumors follow gray matter on
Sarcoidosis is a great mimicker. It is the most common all pulse sequences and show avid enhancement and pres-
granulomatous disease of adulthood that involves the pi- ence of an enhancing dural tail. When a mass lesion in the
tuitary stalk.23 Other preferred sites of involvement in the region of the cavernous sinus demonstrates narrowing of the
central nervous system (CNS) include the meninges, the cavernous internal carotid artery, it is highly suggestive of a
cranial nerves, hypothalamus, and perivascular paren- meningioma (Fig. 9).
chyma. A minority of patients with sarcoidosis get neuro-
sarcoid. Moreover, in only 1% of patients with neurosar- Other Tumor or Tumor-Like Lesions
coid, the disease is isolated to the CNS.24,25 Usually Other tumor or tumor-like lesions observed within the
abnormal leptomeningeal, as well as nodular perivascular cavernous sinus include lymphoma, leukemia, pseudotu-
parenchymal, enhancement is seen. mor, and metastasis. Breast and lung are common tumors
to metastasize to the dura. Perineural metastasis is com-
Tuberculosis mon with head and neck tumors, such as squamous cell
Tuberculosis may also involve the pituitary hypothalamic carcinoma and adenoid cystic carcinoma. Any cranial
axis. CNS tuberculosis presents with extensive basilar cis- nerve can provide means of perineural spread. However,
tern pachymeningitis and parenchymal tuberculomas. In most commonly, the tumors gain access to the cavernous
the suprasellar cistern, tuberculomas may be present as sinuses by growing perineurally along the V2 or V3 divi-
solid-enhancing or ring-enhancing masses.18 sions of the trigeminal nerve. Skip areas of enhancement
may be present.
Metastases
Patients with pituitary hypothalamic axis metastasis usu- Chondrosarcomas
ally have a known malignancy. The most common metas- Chondrosarcomas are cartilaginous, off midline skull base
tases to the sella are from breast and lung.26 Cases of tumors that occur in the middle cranial fossa. Chondro-
metastatic colon, thyroid, renal cell carcinoma, and leuke- sarcomas affect the cavernous sinuses because of their pre-
46 A. Derman et al

ponderance for petrooccipital synchondrosis. The tumor


may displace the cavernous sinuses superiorly, narrow
them, or invade them directly because of the inferiosupe-
rior vector of growth. On imaging, chondrosarcoma is
seen as a lytic-appearing lesion on CT, which demon-
strates rings and arcs of calcification. On MR, the tumor
appears markedly hyperintense on T2WI, shows chon-
droid matrix, is locally aggressive, and demonstrates vari-
able enhancement.

Inflammatory Diseases
of the Cavernous Sinuses
Tolosa-Hunt Syndrome
Tolosa-Hunt syndrome is a painful ophthalmoplegia
caused by an inflammatory process of unknown etiology,
involving typically the cavernous sinus and the adjacent
superior orbital fissure. On imaging, the inflammatory tis-
sue in the cavernous sinus appears isointense to muscle on
T1WI, iso- to hyperintense on T2WI, and enhances after
contrast administration.5 A dramatic response to steroids
is often seen.
Wegener’s Granulomatosis
Wegener’s granulomatosis typically involves the paranasal
sinuses, kidneys, and lungs. Inflammation can progress from
the paranasal sinuses into the orbits and orbital apex. Cav-
ernous sinus and contiguous intrasellar extension have been
reported. Granulomatous tissue is typically hypointense on
both T1WI and T2WI and demonstrates marked enhance-
ment.32

Infectious Diseases
of the Cavernous Sinuses
Cavernous Sinus Thrombophlebitis
Cavernous sinus thrombophlebitis is a life-threatening con-
dition occurring because of facial, paranasal sinus, and odon-
togenic infection. The most common bacterial agents are
Staphylococcus aureus followed by Streptococcus species. The
valveless superior and inferior ophthalmic veins and the
pterygoid venous plexus provide an avenue for spread of
infection from the facial veins into the cavernous sinuses with
secondary thrombosis. On MRI, thrombosis is visualized as a
filling defect within the cavernous sinuses following contrast
administration. Indirect signs of cavernous sinus thrombosis
include dilatation of the cavernous sinus, dilatation/throm-
bosis of the superior ophthalmic vein, exophthalmos, strand-
ing of the retrobulbar fat, edematous extraocular muscles,
and increased dural enhancement along the lateral cavernous
Figure 11 A 56-year-old woman with trigeminal schwannoma: (A) sinus border.33
Axial CT scan, bone window, demonstrates benign remodeling of
Fungal Cavernous Sinus Infections
the right anterior clinoid (curved arrow) and the right petrous apex.
(B) Axial T2WI demonstrates a well-defined, lobulated, heteroge- Fungal cavernous sinus infections can be life-threatening,
nous mass involving the right cavernous sinus region extending and they have been reported with actinomycosis (observed
posteriorly to involve the Meckel’s cave and medially to extend into more commonly in immunocompetent patients), aspergil-
the sella turcica and sphenoid sinus. Note posterior displacement of losis (observed in both immunocompetent and immuno-
the right cavernous internal carotid artery flow void. (C) Axial post- compromised patients), and mucormycosis (observed in
contrast T1WI demonstrates a moderately enhancing mass. immunocompromised patients and diabetics). Actinomy-
cosis and aspergillosis usually spread intracranially from
Imaging the sella and parasellar region 47

Figure 12 Middle-aged woman with a giant left cavernous internal


carotid artery aneurysm: (A) Axial CT scan, brain soft tissue win- Figure 13 Direct carotid cavernous fistula: (A) Axial CT scan, soft-
dow, demonstrates a large hyperdense left cavernous sinus mass tissue window, demonstrates left proptosis and mild stranding of
encroaching the sella. Note the peripheral thin rim of eggshell cal- the intraconal orbital fat. (B) Coronal CT scan, soft-tissue window,
cification (arrow). (B) Axial T1WI demonstrates a large flow void demonstrates marked asymmetric enlargement of the left superior
central to the mass and surrounding crescentric T1 hyperintensity, ophthalmic vein (arrow) and enlargement of the extraocular mus-
suggestive of a thrombus. Of note is pulsation artifact in the phase- cles. (C) Digital subtraction angiography, lateral projection, left in-
encoding direction. (C) 3D time-of-flight MRA, MIP reconstruction, ternal carotid artery injection, arterial phase, demonstrates early
confirms a giant cavernous internal carotid artery aneurysm (arrow abnormal contrast opacification of the left cavernous sinus (arrow)
head). and left superior ophthalmic vein (bold arrow).
48 A. Derman et al

Figure 14 Hemangioma of the cavernous sinus: (A) Axial T2WI dem- Figure 15 A 52-year-old woman with ecchordosis physaliphora: (A)
onstrates a lobulated markedly T2 hyperintense mass approximat- Axial T2WI demonstrates a lobulated, predominantly hyperintense
ing CSF signal, expanding the right Meckel’s cave and the adjacent lesion within the prepontine cistern (arrows), causing mild mass
cavernous sinus (arrow). (B) Axial T1WI demonstrates the mass to effect on the pons, and minimally displacing the basilar artery to the
be isointense to gray matter. There are punctate areas of superim- left. The lesion is seen to project anteriorly into the clivus. (B) Axial
posed T1 hyperintensity consistent with hemorrhage (arrow). (C) T1WI demonstrates the lesion to be isointense to minimally hyper-
Axial contrast-enhanced T1WI demonstrates avid, near homoge- intense than CSF. (C) Axial postcontrast T1WI demonstrates com-
nous enhancement of the mass. plete lack of enhancement.
Imaging the sella and parasellar region 49

paranasal sinuses and ear, or via hematogenous dissemi-


nation. Dental carries is also a risk factor.34 Typically, the
fungal concretions observed within the paranasal sinuses
appear hyperdense on noncontrast CT images, and hy-
pointense on T1WI and T2WI. The intracranial fungal
lesion appears hypointense on T2WI, enhances after con-
trast administration, and narrows the internal carotid ar-
tery; imaging features identical to a meningioma. How-
ever, the patients are sick, and the infection is rapidly
progressive. Chronic symptoms with sudden fulminant
progression have also been reported.35 Mucormycosis is
highly angioinvasive. It frequently involves the paranasal
sinuses and orbits, with or without extension into the
cavernous sinuses and the skull base.36 When cavernous
sinus invasion occurs, it results in thrombosis (rarely an-
eurysm) of the cavernous segment of the internal carotid
artery.37 Vascular invasion and rapid disease progression
are distinguishing features of invasive mucormycosis.

Vascular Lesions
Aneurysms
Aneurysms are important to consider in the differential diagno-
sis of sellar and parasellar masses. Both, infradiaphragmatic and
supradiaphragmatic aneurysms can extend into the sella. The
infradiaphragmatic aneurysms arise of the cavernous internal
carotid artery and extend medially into the sella. The supradia-
phragmatic aneurysms arise of the ophthalmic division of inter-
nal carotid artery or of the anterior communicating artery and
extend inferomedially into the sella.38 Such aneurysms can be
identified on CT or MR angiogram, the gold standard still being
conventional angiogram. On conventional MRI, a patent saccu-
lar aneurysm is seen as a prominent focal flow-void related to the
parent artery. When partially thrombosed, a large flow void can
be seen centrally with a lamellated appearance of the surround-
ing thrombus (Fig. 12).

Carotid Cavernous Fistula


Carotid cavernous fistula (CCF) can be direct and indirect.
Direct CCFs may be post-traumatic or spontaneous from
collagen vascular disease, such as Ehlers–Danlos syn-
drome, or from ruptured internal carotid artery aneurysm.
Patients present with proptosis, extraocular muscle en-
largement, and orbital edema. Direct CCFs result in cav-
ernous sinus enlargement and arterialized flow in the su-
perior ophthalmic vein. Angiography demonstrates early
prominent filling of the cavernous sinus and visualization
of the superior ophthalmic vein on arterial phase images
(Fig. 13). Indirect CCFs may result from rupture of dural
Figure 16 A 21-year-old man with chondroid chordoma: (A) axial vessels. They have a slower onset and are more insidious.
CT scan, bone window, demonstrates an expansile lytic appearing Arterialized flow within the indirect CCF is less prominent
lesion (M), involving the floor of the anterior and middle cranial compared with the direct variety, and therefore, the cav-
fossa. (B) Axial T2WI demonstrates a well-defined lobulated, pre- ernous sinuses can remain normal in size. However, arte-
dominantly hyperintense mass, which abuts but does not narrow rialized flow can be observed in superior ophthalmic vein.
the cavernous internal carotid arteries. Foci of low signal seen Stranding of the retrobulbar fat, and engorgement of the
within this mass likely represent residual calcification. (C) Sagittal
extraocular muscles, is often seen. Mild proptosis can be
postcontrast T1WI demonstrates avid enhancement of the lesion.
seen in indirect CCFs.
50 A. Derman et al

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