58i

Downloaded from ajronline.org by 77.243.27.97 on 11/17/23 from IP address 77.243.27.97. Copyright ARRS. For personal use only; all rights reserved

Review Article

Neoplastic Diseases Affecting the Central Skull Base:

CT and MR Imaging
Lawrence E. Ginsberg1

Modern imaging techniques play a vital role in the diagnostic some of the more common skull base tumors and discusses
evaluation and follow-up of patients with neoplastic disease af- strategies for establishing a differential diagnosis. Not in-
fecting the skull base. Many of these lesions have a high rate of cluded are the congenital (encephalocele, arachnoid cyst,
recurrence if surgical removal is not complete. Newer, more
craniosynostosis), traumatic, infectious (otogenic skull base
aggressive surgical approaches can have a maximal effect only
osteomyelitis), or tumorlike (Paget’s disease, fibrous dyspla-
if the full extent of a lesion is known preoperatively. CT and MR
sia) conditions that can affect the central skull base. Tumorlike
imaging provide the surgeon with detailed information about
every site of tumor involvement. Although these lesions are not conditions should, however, be considered in the differential
common in clinical radlologic practice, the radiologist must be diagnosis of lesions of the skull base as imaging features can
prepared to offer a reasonable differential diagnosis and a full overlap.
evaluation of the extent of disease. This review presents some
of the more commonly encountered tumors that can affect the
skull base and describes their radiologic features, with emphasis Rhabdomyosarcoma
on CT and MR imaging.
Rhabdomyosarcoma is a mesenchymal malignant tumor
that occurs most often in children. This lesion constitutes 4-
The central skull base, generally considered to include the 8% of all malignant tumors in children less than i5 years old
clivus, sella, parasellar region, and greater wing of the sphe- [1] and is the most common soft-tissue sarcoma in children
noid bone, is the site of a variety of tumors, both primary and [2]. Although rhabdomyosarcoma is less common in adults,
secondary. Radiologists are often asked to suggest the pos- Nakhleh et al. [3] reported a series of 12 patients, i 8-36
sible nature of and evaluate the extent of such lesions. years old, who had either embryonal or alveolar rhabdo-
Although a definitive preoperative diagnosis is not always myosarcoma of head and neck origin. Therefore, this diag-
possible, the site of a lesion and its imaging characteristics nosis probably should be considered in a young adult who
often limit the diagnostic possibilities. Certain tumors have a has a mass in the appropriate location. of
Forty percent
strong predilection for specific anatomic locations in the skull rhabdomyosarcomas arise in the head and neck [1 , 2]. The
base. Imaging characteristics such as CT appearance, MR orbit and nasopharynx are affected most often; next are the
signal intensity, tissue homogeneity, and enhancement pat- paranasal sinuses and middle ear. The Intergroup Rhabdo-
tern further aid in narrowing the diagnostic possibilities. The myosarcoma Study [i , 4, 5] divides head and neck rhabdo-
extent of neoplastic involvement can be assessed accurately myosarcomas by site of origin into three groups: (1) orbital,
in all cases. This review describes the imaging features of (2) parameningeal, and (3) other head and neck sites. The

Received December 19, 1991 ; accepted after revision February 28, 1992.
I Department of Radiology. Bowman Gray School of Medicine, Medical Center Blvd., Winston-Salem, NC 27157-1088. Address reprint requests to L. E.
Ginsberg.
AJR 159:581-589, September 1992 0361 -803X/92/1 593-0581 © American Roentgen Ray Society
 582 GINSBERG AJR:159, September 1992

parameningeal group includes those tumors closely adjacent of i 5.5 years. Opinions differ, but the site of origin
is probably
to the skull base meninges: middle ear, posterior nasopharynx in the region of the nasopharynx, [7, i 0], or,
posterior nares
and posterior paranasal sinuses, and nasal cavity and anterior as suggested by Lloyd and Phelps [9], the sphenopalatine
paranasal sinuses [i This subgroup ]. of rhabdomyosarcomas foramen. This lesion accounts for 0.5% of head and neck
has the poorest prognosis because invasion of the skull and neoplasms and is the most common benign tumor of the
intracranial spread are likely. CT shows an enhancing soft- nasopharynx [7]. Clinical presentation is related to common
tissue mass and areas of bone destruction. Meningeal en- sites of tumor extension. Nasal obstruction, epistaxis, and,
Downloaded from ajronline.org by 77.243.27.97 on 11/17/23 from IP address 77.243.27.97. Copyright ARRS. For personal use only; all rights reserved

hancement representing tumor involvement indicates a poor occasionally, facial deformity are the initial signs and symp-
prognosis [6]. Foraminal destruction can be detected. Rhab- toms. At the time of presentation, extensive spread beyond
domyosarcomas generally appear as a bulky soft-tissue mass the site of origin is common. Involvement of the nasal cavity,
with a signal intensity similar to that of muscle on Ti-weighted sphenoidal sinus, and pterygopalatine fossa is likely; involve-
MR images (e.g., 750/i 6 [TR/TE]) and hyperintensity on T2- ment of the pterygopalatine fossa results in the classic ante-
weighted images (e.g., 2000/70) [2] (Fig. 1). Heterogeneity nor bowing of the posterior wall of the maxillary sinus (Fig.
may be seen on T2-weighted images in many cases [2]. In a 2). With involvement of the pterygopalatine fossa, extension
study by Yousem et aI. [2], seven (i 00%) of seven cases into the infratemporal fossa via the pterygomaxillary fissure is
showed contrast enhancement. Signal characteristics alone facilitated. The tumor can gain access into the orbit via the
are not sufficient to distinguish between the histologic inferior orbital fissure and then into the middle cranial fossa
subgroups of rhabdomyosarcoma (embryonal and alveolar) via the superior orbital fissure. Extension into the sphenoidal
[2]. Destruction of the skull base and intracranial involvement sinus is encountered in two thirds of cases at presentation.
should be readily detected on MR images, especially with Posterior invasion into the clivus and cavernous sinuses is
contrast enhancement. In particular, involvement of the cay- possible and must be sought radiologically. Because of the
ernous sinus is common and should be ruled out, as it may vascular nature of this lesion, biopsy is hazardous, and the
result in cranial nerve palsies and is a grave prognostic sign diagnosis generally depends on the clinical presentation, age
[7] (Fig. 1). As with other lesions in this region, perineural and sex of the patient, and radiologic features.
spread is possible and may contribute to intracranial involve- CT shows a homogeneously enhancing soft-tissue mass in
ment. Recent work [8] indicates improved prognosis for non- the nasopharynx and adjacent spaces. Involvement of the
orbital head and neck rhabdomyosarcoma when multiagent pterygopalatine fossa is generally accompanied by anterior
chemotherapy, surgery, and radiation therapy are used. remodeling of the posterior wall of the maxillary sinus (Fig. 2).
Loss of normal infratemporal fossa fat suggests tumor in-
volvement at that site. This neoplasm has low to intermediate
Juvenile Angiofibroma
signal intensity on Ti-weighted MR images (Fig. 3), and is
Juvenile angiofibroma is an uncommon, highly vascular variably hyperintense on T2-weighted images. Internal foci of
tumor that affects adolescent boys. Though benign, it is punctate or serpiginous low signal intensity represent tumor
aggressive. In their series of 30 patients with juvenile angio- vessels and are present on both Ti - and T2-weighted studies
fibroma, Lloyd and Phelps [9] found an average age of onset and after administration of contrast material. As expected,

Fig. 1.-Nasopharyngeal rhabdomyosarcoma Fig. 2.-Juvenile angiofibroma in a 16-year-old Fig. 3.-Juvenile angiofibroma in a 12-year-
in a 10-year-old boy. Coronal Ti-weighted MR boy. Contrast-enhanced axial CT scan shows old boy. Axial Ti-weighted MR image (650/20)
image (750/16) shows mass in right nasophar- mass with widening of left pterygopalatine fossa shows a large isointense nasopharyngeal mass.
ynx extending intracranially through foramen (white arrow) and slight anterior bowing of poste- Left aspect of lesion shows prominent flow voids
ovale (arrows). Absence of normal internal Ca- nor wall of left maxillary sinus (arrowheads). Wid- corresponding to tumor vessels (arrows). This
rotid flow void (arrowheads) represents occlu- ening of left sphenopalatine foramen establishes finding need not always be present, especially
sion. tumor in this location (black dot). Note involvement in smaller or less vascular lesions.
of sphenoidal sinus (black arrow).
 AJR:159, September 1992 NEOPLASTIC DISEASES OF CENTRAL SKULL BASE 583

this vascular neoplasm enhances brightly with gadopentetate stippled and amorphous, not unlike the chondroid calcifica-
dimeglumine. As with other skull base processes, CT is better tions seen with extracranial chondrosarcomas. Careful eval-
suited for detection of bone changes, and MR imaging is uation of the calcifications is necessary to avoid confusion
better for evaluating the overall extent of involvement. Arte- with other lesions such as osteosarcoma, chondroma, osteo-
nography is often used to provide detailed information about chondroma, or chordoma. Other CT findings include bone
the vascular supply to this tumor and to facilitate preoperative erosion and destruction, an enhancing soft-tissue mass, and
embolization. a sharp zone of transition to normal tissue [i 3, i 4]. MR
Downloaded from ajronline.org by 77.243.27.97 on 11/17/23 from IP address 77.243.27.97. Copyright ARRS. For personal use only; all rights reserved

imaging is generally less specific and is better suited for

evaluating extent of disease. The tumor is hypointense rela-
Chondrosarcoma tive to brain on Ti-weighted images and hyperintense on T2-
Chondrosarcoma, a slow-growing, malignant cartilaginous weighted images. Heterogeneous internal areas of decreased
tumor, constitutes i i % of malignant bone tumors and affects signal represent calcifications. Enhancement is seen with
the head and neck in approximately 6% of cases [i i , i 2]. gadopentetate dimeglumine and may be heterogeneous for
Chondrosarcomas account for approximately 6% of all skull the same reason (Fig. 4B).
base lesions. They can arise in cartilage; endochondral bone;
or primitive mesenchymal cells in the brain, meninges, mem-
Chordoma
branous bone, or soft tissue [i 0, 13]. Secondary chondrosar-
coma can occur in diseased bone (fibrous dysplasia, Paget’s Chordomas are rare, slow-growing neoplasms of embry-
disease, osteochondroma). As the skull base is derived from onic notochordal derivation. They account for less than i %
cartilage, the presence of chondrosarcoma at this site is of all intracranial tumors [10] and only 3-4% of primary bone
predictable; in fact, three fourths of all cranial chondrosarco- tumors [i 5]. Approximately 35% affect the skull base [7,
mas are located in the skull base [i 4]. Specific sites of i 6], and most involve the clivus. Petrous apex chordomas
involvement include the parasellar region, cerebellopontine are rare [i7]. Although by strict criteria chordomas are his-
angle, and facial region, especially the sphenoethmoid, max- tologically benign, their aggressive behavior and nearly i 00%
illary, and posterior nasal-subsphenoid regions [7, i 3]. These recurrence rate despite radical surgery account for the poor
tumors spread by local invasion. The parasellar and facial prognosis associated with these tumors. Chordomas are also
lesions can cause extensive destruction of the skull base [7, radioresistant [7]. Histologic malignancy or distant metas-
i3]. Systemic metastases are uncommon, and prognosis is tases are rare, and metastases appear to be more likely with
related to histologic grade, site, and extent of involvement. sacrococcygeal chordomas [i 8]. The most common age at
Prognosis also may be affected by the occasional difficulty in onset is 20-40 years, and men and women are affected
distinguishing histologically among chondrosarcoma, benign equally. Intracranial chordomas are rare in children, but Handa
chondroma, and the so-called chondroid chordoma. Many et al. [19] reported this condition in a 9-year-old boy and
lesions have mixed histologic features [i 4]. Presentation de- reviewed i i other cases in the literature. Presenting signs
pends on location, but typically it is manifested by insidious and symptoms include cranial neuropathies, headache, vision
onset of single or multiple cranial neuropathies [i 4]. disturbances, and otolaryngologic symptoms such as nasal
CT and MR play complementary roles in the imaging of obstruction and epistaxis [7, 20].
chondrosarcomas. CT is useful in evaluating the calcified Radiologically, chordomas appear as enhancing soft-tissue
matrix so characteristic of this lesion (Fig. 4A). Lee and Van masses with bone destruction. Chordomas are often quite
Tassel [i 3] found a mineralized tumor matrix in all i 5 of their large, extending into the epidural space and causing compres-
cases. The calcification varies in appearance but can be sion of the vertebrobasilar system and brainstem (Fig. 5). CT

Fig. 4.-Chondrosarcoma in a 55-year-old

woman.
A, Axial CT scan (bone algorithm) shows large
mass arising in nasoethmoid region. Matrix calci-
fications are well seen (arrows). Sphenoidal sinus
is obliterated by tumor.
B, Contrast-enhanced axialTi-weighted MR im-
age (533/20) shows enhancement; marked heter-
ogeneity represents matrix calcification. Note en-
hancement representing tumor involvement ante-
rioriy in both middle cranial fossae (arrows) and
both cavernous sinuses (arrowheads).
 584 GINSBERG AJR:159, September 1992

Fig. 5.-Clival chordoma in a 19-year-old man.

A, Axial CT scan shows large clival mass with
large bone fragment centrally (arrow).
B, Sagittal Ti-weighted MR image (400/20)
shows large heterogeneous, hypointense mass
destroying clivus and extending posterioriy to
compress medulla. Vertebral artery is seen draped
posteriorly (straight arrow). Note involvement of
sphenoidal sinus anteriorly (arrowhead). Inferior
extension involves anterior lip of foramen magnum
Downloaded from ajronline.org by 77.243.27.97 on 11/17/23 from IP address 77.243.27.97. Copyright ARRS. For personal use only; all rights reserved

and dens (curved arrow).

(Courtesy of G. Sze, New Haven, CT.)

shows areas of calcification, many of which are actually CT typically shows an enhancing soft-tissue mass and, as
fragments ofdestroyed bone [15, 21] (Fig. 5A). In their studies expected, shows bone destruction accurately. Ti -weighted
of chordoma, Sze et al. [i 6] and Oot et al. [2i] agreed on the MR images show an isointense to slightly hypointense mass
MR appearance of these lesions. Chordomas are isointense that enhances after administration of gadopentetate dimeglu-
to slightly hypointense on Ti -weighted MR images (Fig. 56) mine. The extent of infrasellar and skull base involvement is
and hyperintense on T2-weighted images; they also may particularly well shown on coronal and sagittal MR sequences
contain areas of decreased signal intensity corresponding to (Fig. 6).
calcification. Cystic areas can be detected [1 6]. Enhancement
occurs with contrast administration. CT and MR are equally
reliable in detecting this tumor, but MR imaging is considered Meningioma
superior for evaluating extent of disease, especially displace-
ment or encasement of vascular structures and degree of Meningiomas are common benign intracranial tumors that
intracranial involvement [i 6]. arise from arachnoid cells on the inner surface of the dura
Chondroid chordomas, a subset of chordomas, appear to mater. They account for approximately 15% of primary CNS
be associated with a considerably better prognosis. Although tumors and are more common in women than in men [7].
Oot et al. [21] found no differentiating features on MR imag- Although the parasagittal and cerebral convexities are more
ing, Sze et al. [i 6] found less hyperintensity on T2-weighted common sites of occurrence, one large series [22] demon-
MR images and shorter Ti values with this variant when it strated a 33% frequency in the skull base. Most of these
was compared with typical chordomas. However, the findings involved the wings of the sphenoid bone. According to Leo-
depended on window setting and photography, and therefore netti et al. [23], 20% of all intracranial meningiomas involve
may be of little practical value. Although chordoma has no the sphenoid ridge. Therefore, this lesion is seen relatively
specific radiologic sign, this tumor should be considered frequently. Meningiomas of the wings of the sphenoid bone
whenever a destructive clival lesion is detected in a middle- are often subclassified into middle, medial, and hyperostosing
aged person. en plaque varieties. Hyperostosing en plaque meningiomas
grow along the lateral aspect and often a large portion of the
greater wing of the sphenoid bone (Fig. 7). An intense hyper-
Invasive Pituitary Adenoma
ostotic bony response indicates invasion of the sphenoid bone
Most pituitary neoplasms in adults are adenomas. These by tumor, unlike the typical reactionary hyperostosis associ-
lesions are generally slow growing, histologically benign, and ated with other meningiomas [22, 24, 25] (Fig. 7A). This bone
confined to the sella or suprasellar cistern. They can extend invasion contributes both to the difficulty in achieving total
laterally into the cavernous sinus or inferiorly into the sphe- resection and to the likelihood of recurrence [22]. Extension
noidal sinus and skull base. Such extension can make it through the lateral orbital wall is common and accounts for
difficult to distinguish pituitary adenoma from other skull base the classic clinical presentation of this lesion: slowly progres-
lesions such as sphenoidal sinus carcinoma, chordoma, or sive, painless unilateral exophthalmos and decreased visual
superior extension of nasopharyngeal carcinoma [7]. Invasive acuity [22, 24]. CT shows the hyperostosis, although it also
pituitary adenomas may not have a suprasellar component can be seen on MR images (Fig. 7B). Kim et al. [25] noted
and may grow only inferiorly (Fig. 6). Except for absence of the potential for confusing this hyperostosis with such bone
the sellar floor, the sella itself may be relatively intact, and conditions as fibrous dysplasia, and suggested differentiating
this finding should not be used to eliminate pituitary adenoma features; however, the patient’s age, the presentation, and
from consideration when an infrasellar mass is found in the the presence of a soft-tissue mass with meningiomas should
appropriate clinical setting. lead to the correct diagnosis.
 AJR:159, September 1992 NEOPLASTIC DISEASES OF CENTRAL SKULL BASE 585
Downloaded from ajronline.org by 77.243.27.97 on 11/17/23 from IP address 77.243.27.97. Copyright ARRS. For personal use only; all rights reserved

Fig. 6.-Invasive pituitary adenoma in a 65- Fig. 7.-Hyperostosing en plaque meningioma involving wing of sphenoid bone in a 66-year-old
year-old man. CT bone windows showed extensive man.
bone destruction. Contrast-enhanced sagittal Ti- A, Axial CT scan (bone window) shows intense hyperostosis (arrows) along greater wing of
weighted MR image (500/20) shows large enhanc- sphenoid bone and lateral orbital wall on right and hyperostosis (arrowhead) of lateral wall of right
ing nss occupyng sphenoidal sinus and replac- sphenoidal sinus.
leg clivus. Absence of thin hypointense cortical B, Axial Ti-weighted MR image (600/20) after contrast administration shows enhancing tumor
bone at sellar floor indicates destruction. Intrasel- Involving right cavernous sinus (small black arrowhead) and extending along parasellar region and
lar contents are preserved. Note differential signal anterior aspect of middle cranial fossa (white arrowheads). Bone disease can be detected by
Intensity between pituitary gland (arrow) and rel- replacement of normal marrow signal of right wing of sphenold bone by minimally enhancing,
atively less enhancing mass below it relatively hypeintense tumor (white arrows). Note normal marrow fat on left side. Right orbital mass
(large black arrowhead) causes medial displacement of optic nerve (black arrow).

Any of the meningiomas of the sphenoid ridge can extend usually show enhancement. Encroachment on neural foram-
extracranially. Extension can occur via skull base foramen or ma (Fig. 9A) and spread to vital structures such as Meckel’s
by destruction and direct extension through the floor of the cave or the cavernous sinus may occur (Fig. 9B). In patients
middle cranial fossa [7]. Medial extension to involve the with of underlying
no history malignant disease, imaging alone
sphenoidal and ethmoidal sinuses may also occur. When often does not allow differentiation between primary and
involvement of the skull base is extensive, diagnostic consid- metastatic neoplasms. The presence of other sites of met-
erations should include other neoplasms such as nasopharyn- astatic disease in the calvaria are of course helpful in the
geal tumors or chordoma (Fig. 8). diagnosis (Fig. 9B).
CT findings include bone hyperostosis and a hyperdense,
enhancing mass. On Ti -weighted MR images, meningiomas
are generally isointense or slightly hypointense relative to gray Invasive Nasopharyngeal Carcinoma
matter (Fig. 8A). On T2-weighted images, approximately i 0% The most common primary malignant lesion that involves
are hypointense, 50% remain isointense, and 40% become the skull base is squamous cell carcinoma, usually arising in
hyperintense relative to brain [26]. Bright homogeneous con- the nasopharynx [27]. In their review of patterns of spread of
trast enhancement is generally observed (Fig. 8B). Again, MR nasopharyngeal carcinoma, Sham et al. [28] found that 82
imaging is ideally suited for determining extent of disease. (3i%) of 262 cases had erosion of the skull base. Despite the
much higher prevalence of this lesion in China, invasion of the
skull base by nasopharyngeal carcinoma is common in the
Metastatic Disease
United States. CT shows an enhancing soft-tissue mass.
Although hematogenous metastases to the skull base oc- Nasopharyngeal carcinoma is isointense relative to skeletal
cur infrequently, they are more common than primary neo- muscle on Ti -weighted MR images (Fig. i 0) and hyperintense
plasms of the skull base [27]. The most likely primary sites of on T2-weighted images. It enhances with gadopentetate di-
such metastases are prostate, lung, breast, and kidney; lym- meglumine. Invasion of the skull base is quite common and is
phomas are less likely. Most of these metastatic lesions are readily detected with CT bone windows (Fig. 1 i). With Ti
seen as areas of lytic bone destruction on CT, except for weighting, MR imaging also shows bone invasion as replace-
prostatic metastases, which are usually osteoblastic. The ment of normal marrow with lower signal intensity tumor (Fig.
tumor may have a soft-tissue component. On Ti -weighted i 0). Involvement of the skull base can result in foraminal
MR images, the hallmark of metastatic disease is replacement destruction, with consequent cranial neuropathy, vascular
of normal bone marrow with material of decreased signal compromise, or both [29] (Figs. i 0 and 1 1). Intracranial
intensity (Fig. 9). On T2-weighted images, metastases are spread can occur via direct extension from the skull base or
generally hyperintense relative to adjacent normal bone mar- by perineural spread, generally affecting branches of the fifth
row. With contrast material, bone and soft-tissue components cranial nerve. The diagnosis of nasopharyngeal carcinoma
 586 GINSBERG AJR:159, September 1992

Fig. 8.-Meningioma in a 56-year-old woman.

Lesion was initially thought to be a chordoma.
A, Axial TI-weighted MR image (550/20) shows
large mass, slightly hypointense relative to brain,
extensively infiftrating skull base and replacing
clivus. Note bilateral encasement of internal ca-
rotid arteries (white arrows) and brainstem
compression caused by extension into prepontine
cistern (arrowheads). Pterygoid plates and masti-
cator space are also involved bilaterally (black
Downloaded from ajronline.org by 77.243.27.97 on 11/17/23 from IP address 77.243.27.97. Copyright ARRS. For personal use only; all rights reserved

arrows).
B, Coronal T2-weighted MR image (550/20)
after contrast administration shows intense tumor
enhancement. Carotid arteries are cleariy encased
by tumor (arrows). Note large right middle cranial
fossa tumor component (arrowhead).

Fig. 9.-Metastatic lung carcinoma in an 84-

year-old man who had right-sided cranial neurop-
athies. Initial CT scan (not shown) was interpreted
as normal.
A, Axial Ti-weighted MR image (550/20) shows
marrow replacement in right half of clivus (straight
arrow). Tumor respects midline and extends into
region of right jugular foramen and probably com-
promises exiting ninth and tenth nerve roots (ar-
rowhead). Note normal nerve roots on left (curved
arrow).
B, Axial TI-weighted MR image (550/20) ceph-
alad to A shows tumor infiltrating right cavernous
sinus and Meckel’s cave (arrows). Cavernous ca-
rotid artery is slightly narrowed. Additional focus
of metastatic disease is seen in left occipital bone
(arrowheads).

may not be known before imaging. The nasopharyngeal corn- the ethmoid complex in iO%, and the sphenoidal and frontal
ponent may be small and must be carefully sought. In the sinus in only i % [30]. These tumors have a strong tendency
absence of an identifiable nasopharyngeal origin, imaging to destroy bone; therefore, lesions in the posterior nasoeth-
findings may be nonspecific, and other diagnoses should be moid region or sphenoidal sinus are a potential source of
considered. Metastases, lymphoma, and primary bone neo- direct tumor spread to the skull base (Fig. i2). Differentiation
plasms can cause destruction of the skull base and a soft- from sinus extension of primary tumors of the skull base may
tissue mass in the nasopharynx. Opacification of the mastoids not be possible. Sinonasal carcinomas typically have low to
or middle ear, often seen in nasopharyngeal carcinoma as a intermediate signal intensity on Ti -weighted MR images and
result of obstruction of the orifice of the eustachian tube, also intermediate signal intensity on T2-weighted images [3i]. This
can be caused by direct invasion of the eustachian tube by appearance often allows important differentiation between
any process and therefore is nonspecific. tumor involvement in a sinus and obstructive or inflammatory
change. Inflammatory tissue or obstructed secretions, be-
cause of higher water content, typically have a higher T2
Sinonasal Carcinoma signal intensity [3i].
Carcinomas arising in the nasal cavity or paranasal sinuses
are uncommon; they account for only 3% of head and neck
Discussion
tumors [30]. Histologically, squamous cell and undifferen-
tiated carcinoma are the main cell types. The maxillary sinus The anatomy of the central skull base is extremely complex.
is involved either directly or by extension in 80% of cases The sphenoid bone is the foundation of the skull base and is
[30]. The site of origin is the nasal cavity in 25-35% of cases, involved in virtually every process affecting the central skull
 AJR:159, September 1992 NEOPLASTIC DISEASES OF CENTRAL SKULL BASE 587
Downloaded from ajronline.org by 77.243.27.97 on 11/17/23 from IP address 77.243.27.97. Copyright ARRS. For personal use only; all rights reserved

Fig. 10.-Nasopharyngeal carcinoma in a 70- Fig. 11.-Nasopharyngeal carcinoma in a 68- Fig. 12.-Pooriy differentiated sinonasal carci-
year-old man. AxialTi-weighted MR image (600/ year-old woman. Axial CT scan (bone window) noma In a 73-year-old man. Axial CT scan shows
16) shows a large left-sided nasopharyngeal shows extensive left-sided destruction of skull a very destructive sinonasal mass. Left ethmoidal
mass that has extended posteriorly to invade base. Left vidian canal (long whIte arrow), left air cells and sphenoidal sinus are obliterated by
skull base. Left anterior foramen magnum shows carotid canal (black arrow), left aspect of clivus tumor. Orbital fissures, orbital apex, foramen ro-
replacement of marrow by tumor. Note normal (small arrowheads), and left petrous apex (large tundum, and anterior floor of left middle cranial
marrow on right (arrowhead) and normal right arrowhead) are destroyed. Bone margins of left fossa are destroyed.
hypoglossal canal (open arrow). Left hypoglos- foramen ovale and spinosum probably are
sal canal is replaced by tumor(long solid arrow). eroded (short whIte arrow).
Note encasement of left carotid artery (short
solId arrow).

base and most of the pathologic conditions discussed in this base are involved? Is foraminal destruction present? Is pen-
article. The sphenoid bone contains most of the important neural spread present? Are the cavernous sinuses intact? Is
foramina and fissures of the skull base, constitutes the floor vascular compromise present? Is there intracranial extension
and anterior wall of the middle cranial fossa, contains the or compression of brain parenchyma? Answers to these
pituitary gland within the sella turcica, and forms part of the questions ultimately may be more important than precise
clivus. The body of the sphenoid bone contains the paired diagnosis because they provide the information needed for
sphenoidal sinuses. Therefore, to a large extent, imaging the complete surgical excision. They may also save a patient with
central skull base means imaging the sphenoid bone. extensive disease from lengthy, disfiguring, and ultimately
A large variety of primary#{149}and
secondary pathologic proc- unsuccessful surgery.
esses can affect the central skull base. To some extent, the With MR imaging, extent of disease can be precisely out-
radiologic features of many lesions of the skull base are lined in virtually every case. Occasionally, the findings on
nonspecific. For example, an enhancing soft-tissue mass contrast-enhanced images can be inconclusive if a tumor
replacing the clivus that is hypointense on Ti-weighted im- component enhances to an intensity similar to that of adjacent
ages and hyperintense on T2-weighted images could be fat. In these cases, unenhanced Ti -weighted MR imaging or
chordoma, meningioma, chondrosarcoma, metastasis, or the recently developed fat-suppression techniques (after en-
even pituitary adenoma. However, when the features of the hancement) can be used to solve specific problems [32, 33].
more common lesions are considered systematically, some After the extent of a tumor has been determined, the
diagnostic possibilities can be eliminated or placed lower on challenge remains to make a predictive histologic diagnosis
the list, and others become more likely. In this regard, CT and on the basis of the imaging findings. Despite some nonspec-
MR imaging are complementary in their capacity to help the ificity, clinical and imaging features often suggest the correct
radiologist predict the histologic diagnosis of skull base tu- diagnosis.
mors. With meningioma or chondrosarcoma, for example, the In children less than i 0 years old, a nasopharyngeal or
presence of characteristic bone changes can narrow the sinus mass associated with destruction of the skull base is
differential diagnosis substantially. CT generally shows bone likely a rhabdomyosarcoma. Less likely considerations include
changes or destruction better than MR does. Once a lesion metastatic disease from a remote primary neoplasm or the
in the skull base has been fully imaged, the list of possibilities rare melanotic neuroectodermal tumor of infancy. In a poorly
can be reduced. Still, it is often not possible to predict a controlled diabetic or immunocompromised patient, opportun-
tumor’s histologic diagnosis with certainty because of the istic infection is a possible diagnosis. In adolescent boys,
similarity of these lesions on CT and MR, and because of juvenile angiofibroma has a characteristic presentation and
their ability to extend from one anatomic location to another. radiologic features (Figs. 2 and 3). In particular, widening of
Probably the most important role of imaging, however, is in the pterygopalatine fossa and sphenopalatine foramen sec-
evaluating the extent of disease. What aspects of the skull ondany to slow growth of this tumor and flow voids on MR
 588 GINSBERG AJR:159, September 1992

studies facilitate diagnosis of juvenile angiofibroma. In equiv- no longer signifies unresectable disease. Indeed, newer, ag-
ocal cases, angiography shows the typical vascular blush gressive surgical techniques are being used not only for cure
associated with this lesion. but, in the case of cranial nerve involvement, for palliation.
Meningiomas often can be diagnosed on the basis of im- Wide dural or cavernous sinus involvement probably would
aging characteristics. Typical features such as en plaque be unresectable for cure. Although more posterior sphenoid-
growth along the wings of the sphenoid bone and hyperos- based or intracranial lesions can spread anteriorly to involve
tosis of the sphenoid bone are highly suggestive. If these the sinonasal region, any mass predominantly centered about
Downloaded from ajronline.org by 77.243.27.97 on 11/17/23 from IP address 77.243.27.97. Copyright ARRS. For personal use only; all rights reserved

bone changes are associated with an orbital mass and a the paranasal sinus/nasal region should suggest that location
history of painless exophthalmos, the diagnosis is almost as the primary source. In adults, other diagnostic considera-
certain. If, however, these features are lacking, or if extracra- tions include minor salivary gland tumors (adenoid cystic,
nial spread into the sinonasal region is extensive, findings are mucoepidermoid carcinoma), melanoma, malignant schwan-
less specific and other diagnoses must be considered. noma, lymphoma, plasmacytoma, fibrosarcoma, metastases,
Pituitary adenoma should be considered when evidence of chondrosarcoma, and fibroosseous lesions (osteosarcoma,
endocrine dysfunction is found. If imaging is done before osteoblastoma, osteoma) [30]. Esthesioneuroblastoma also
laboratory tests or in patients without physical stigmata of should be considered for a more anteriorly situated ethmoidal
endocninopathy, an intrasellar mass with infrasellar or para- lesion with intracranial spread. These do not generally involve
sellar extension suggests the possibility of invasive pituitary the central skull base. Diabetic or immunocomprornised pa-
adenoma. However, there need not be a discernible intrasellar tients with a destructive sinonasal process may have an
component (Fig. 6). In such cases, only a differential diagnosis opportunistic infection such as mucormycosis or aspergillosis.
can be offered, and the clinical history must be reviewed and Less common considerations include Wegener’s granuloma-
correlated with imaging findings. tosis and lethal midline granuloma.
Imaging of chondrosarcoma can be specific if the mineral Hematogenous metastases to the skull base, although
matrix is seen on CT (Fig. 4A). This lesion also has a predi- uncommon, occur more frequently than primary tumors of the
lection for the parasellar and sphenoethmoid/vomer region skull base. Therefore, such metastases should always be
[i 3]. Of course, when chondrosarcoma occurs in an atypical considered when an adult has a clival mass. The tumor may
location or in the absence of characteristic tumor calcification, have a soft-tissue component. Although CT is generally su-
radiologic findings are less specific. penior to MR in the detection of bone disease, MR imaging is
Nasopharyngeal carcinoma frequently is accompanied by preferable when the bone is infiltrated rather than completely
destruction of the skull base. A nasopharyngeal origin usually destroyed (Fig. 9), in which case only minimal changes may
is detected easily with CT or MR imaging. Smaller lesions are be noted on CT. If a high-resolution bone algorithm is not
more easily detected on MR because of MR’s superior soft- used, tumor infiltration of the skull base can be overlooked.
tissue contrast resolution. In addition to causing destruction Differentiation between chordorna and skull base metastases
of the skull base by direct extension, this tumor has a strong may be impossible. Chordorna generally manifests as a large
tendency to spread via a penineural mechanism. Branches of destructive mass (Fig. 5) with fragments of destroyed bone
the fifth cranial nerve are affected most often. This condition centrally (Fig. 5A). However, when the lesion is smaller or no
can be detected on coronal MR images well before CT scans fragmentation of bone occurs, differentiation from metastatic
show foraminal erosion. Fat-suppressed contrast-enhanced disease may be impossible. In our patient who had multiple
Ti -weighted MR images are useful for this purpose. Pen- skull base metastases, the diagnosis was facilitated not only
neural spread is considered a poor prognostic sign. Isointense by the knowledge of underlying lung carcinoma but also by
thickening of a cranial nerve such as V3 on unenhanced Ti - evidence of disease involving other calvanial sites (Fig. 9B).
weighted images indicates penneural tumor involvement (Fig.
i). Enhancement after administration of gadopentetate di- Conclusions
meglumine can help show any associated intracranial corn-
Although not encountered on a daily basis, neoplastic in-
ponent. Denervation atrophy of the masticatory muscles also
volvement of the central skull base can be seen by any
may follow as a result of penineural spread. Because of the
radiologist affiliated with a busy otolaryngology or neurosur-
proximity of the nasopharynx to the skull base, intracranial
gery service. The role of the radiologist is to evaluate the
involvement also can result from spread through the foramen
lacerurn. Tumor also can spread along the carotid canal, extent of disease and to offer a reasonable differential diag-
nosis. When all aspects of a case are considered, these
gaining access to the cavernous sinus without destroying
objectives can be accomplished readily. Although imaging
bone, and can result in neuropathies of cranial nerves Ill, IV,
results are sometimes nonspecific, the combination of CT and
Vi, V2, and VI. Vascular thrombosis can be detected
generally
with routine spin-echo MR imaging. In difficult cases there may MR imaging is invaluable in assessing the extent of disease
and in the treatment and follow-up of patients with neoplastic
be a role for gradient-echo sequences or MR angiography.
Sinonasal carcinomas are uncommon, and most involve the disease of the central skull base.
maxillary sinus or nasal cavity. Unfortunately, these lesions
are often quite large at presentation, causing extensive de- ACKNOWLEDGMENTS
struction. The site of origin cannot always be determined (Fig. I thank Julianne Berckman and Donna S. Garrison for manuscript
12). Involvement of the skull base and pterygopalatine fossa preparation.
 AJR:159, September 1992 NEOPLASTIC DISEASES OF CENTRAL SKULL BASE 589

REFERENCES imaging. Radiology 1988:1 66: 1 87-1 91

17. Brown RV, Sage MR, Brophy BP. CT and MR findings in patients with
1. Latack JT, Hutchinson RJ, Heyn RM. Imaging of rhabdomyosarcomas of
chordomas of the petrous apex. AJNR 1990;1 1 :121-124
the head and neck. AJNR i987;8:353-359
18. KroI G, Sze G, Arbit E, Marcove R, Sundaresan N. Intradural metastases
2. Yousem DM, Lexa FJ, Bilaniuk LT, Zimmerman RI. Rhabdomyosarcomas
of chordoma. AJNR i989;1 0: 193-1 95
in the head and neck: MR imaging evaluation. Radiology 1990:177:
19. Handa J, Suzuki F, Nioka H, Koyama T. Clivus chordoma in childhood.
683-686
Surg Neurol i987;28:58-62
3. Nakhleh RE, Swanson PE, Dehner LP. Juvenile (embryonal and alveolar)
20. Mills RP. Chordomas of the skull base. J R Soc Med 1984;77: 10-16
rhabdomyosarcoma of the head and neck in adults: a clinical, pathologic,
21 . Oot RF, Melville GE, New PFJ, et al. The role of MR and CT in evaluating
Downloaded from ajronline.org by 77.243.27.97 on 11/17/23 from IP address 77.243.27.97. Copyright ARRS. For personal use only; all rights reserved

and immunohistochemical study of 12 cases. Cancer 199i;67: lOi 9-1 024

clival chordomas and chondrosarcomas. AJR i988;1 51 :567-575
4. Raney RB Jr, Donaldson MH, Sutow WW, Lindberg RD, Maurer HM, Teffi
22. Ojemann RG. Meningiomas: clinical features and surgical management. In:
M. Special considerations related to primary site in rhabdomyosarcoma:
Wilkins RH, Rengachary SS, eds. Neurosurgery, vol. 1 . New York:
experience of the Intergroup Rhabdomyosarcoma Study, 1972-76. NCI
McGraw-Hill, 1985:635-654
Monogr 1981;56:69-74
23. Leonetti JP, Reichman OH, Smith PG, Grubb RL, Kaiser P. Meningiomas
5. Teffi M, Femandez C, Donaldson M, Newton W, Moon TE. Incidence of
meningeal involvement by rhabdomyosarcoma of the head and neck in of the lateral skull base: neurotologic manifestations and patterns of
children: a report of the Intergroup Rhabdomyosarcoma Study (IRS). recurrence. Otolaryngol Head Neck Surg i990;103:972-980
Cancer i978;42:253-258 24. Pompili A, Derome PJ, Visot A, Guiot G. Hyperostosing meningiomas of
6. Feldman BA. Rhabdomyosarcoma of the head and neck. Laryngoscope the sphenoid ridge-clinical features, surgical therapy, and long-term
1982;92:424-440 observations: review of 49 cases. Surg Neurol i982;17:41 1-416
7. Braun IF, Nadel L. The central skull base. In: Som PM, Bergeron RT, eds. 25. Kim KS, Rogers LF, Goldblatt D. CT features of hyperostosing meningioma
Head and neck imaging, 2nd ed. St. Louis: Mosby, 1991:875-924 en plaque. AJR i987;i49:1017-1023
8. Anderson GJ, Tom LWC, Womer RB, Handler SD, Wetmore RF, Potsic 26. Curtin HO, Hirsch WL Jr. Base of the skull. In: Atlas SW, ed. Magnetic
WP. Rhabdomyosarcoma of the head and neck in children. Arch Otolar- resonance imaging of the brain and spine. New York: Raven, 1991:
yngol Head Neck Surg i990;1 16:428-431 669-707
9. Lloyd GAS, Phelps PD. Juvenile angiofibroma: imaging by magnetic reso- 27. Whelan MA, Reede DL, Meisler W, Bergeron RT. CT of the base of the
nance, CT and conventional techniques. Clin Otolaryngol i986;1 1: skull. Radiol Clin North Am 1984;22 :177-217
247-259 28. Sham JST, Cheung YK, Choy 0, Chan FL, Leong L. Nasopharyngeal
1 0. Lame FJ, Nadel L, Braun IF. CT and MR imaging of the central skull base: carcinoma: CT evaluation of pattems of tumor spread. AJNR i991;12:
Part 2. Pathologic spectrum. RadioGraphics 1990;10:797-821 265-270
11. Dahlin DC, Unni KK. Bone tumors, 4th ed. Springfield, IL: Thomas, 29. Teresi LM, Lufkin RB, Vinuela F, et al. MR imaging of the nasopharynx
1986:227-259 and floor of the middle cranial fossa: Part II. Malignant tumors. Radiology
12. Pritchard DJ, Lunke RJ, Taylor WF, Dahlin DC, Medley BE. Chondrosar- i987;164:817-821
coma: a clinicopathologic and statistical analysis. Cancer i980;45: 30. Som PM. Tumors and tumorlike conditions. In: Som PM, Bergeron RT,
149-1 57 eds. Head and neck imaging. 2nd ed. St. Louis: Mosby, 1991:169-227
1 3. Lee Y-Y, Van Tassel P. Craniofacial chondrosarcomas: imaging findings in 31 . Som PM, Shapiro MO, Biller HF, Sasaki C, Lawson W. Sinonasal tumors
15 untreated cases. AJNR i989;10:165-170 and inflammatory tissues: differentiation with MR imaging. Radiology
14. Kveton JF, Brackmann DE, Glasscock ME III, House WF, Hitselberger WE. i988;1 67:803-808
Chondrosarcoma of the skull base. Otolaryngol Head Neck Surg 32. Barakos JA, Dillon WP, Chew WM. Orbit, skull base, and pharynx: contrast-
1986;94:23-32 enhanced fat suppression MR imaging. Radiology 1991;179:191-198
1 5. KroI G, Sundaresan N, Deck M. Computed tomography of axial chordomas. 33. Tien RO, Hesselink JR, Chu PK, Szumowski J. Improved detection and
J Comput Assist Tomogr 1983;7:286-289 delineation of head and neck lesions with fat suppression spin-echo MR
16. Sze G, Uichanco LS III, Brant-Zawadzki MN, et al. Chordomas: MR imaging. AJNR 199i;12:19-24