Signature: © Pol J Radiol, 2017; 82: 398-409

DOI: 10.12659/PJR.901937
REVIEW ARTICLE

Received: 2016.10.12
Accepted: 2016.11.02 Skull Base Tumors and Tumor-Like Lesions: A Pictorial
Published: 2017.07.25
Review
Authors’ Contribution: Akira Kunimatsu1 ABCDEF, Natsuko Kunimatsu2 E
A Study Design
B Data Collection 1 Department of Radiology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
C Statistical Analysis 2 Department of Radiology, Mita Hospital, International University of Health and Welfare, Tokyo, Japan
D Data Interpretation
E Manuscript Preparation Author’s address: Akira Kunimatsu, Department of Radiology, Graduate School of Medicine, The University of Tokyo,
F Literature Search 7-3-1, Hongo, Bunkyo-ku, Tokyo 113-8655, Japan, e-mail: [email protected]
G Funds Collection

Summary
A number of tumors and tumor-like non-neoplastic lesions with different cell types on histology
occur in the skull base. A wide variety in disease and lesion appearance often complicates the
process of radiological diagnosis. The main role of radiographic imaging is the detection and
characterization of skull base lesions, with evaluation of the extent of invasion or preservation of
adjacent critical organs. Evaluation of the skull base anatomy and surgical planning by using image
guidance are also important for surgeons. Computed tomography (CT) and magnetic resonance
(MR) imaging are the preferred modalities for the evaluation of skull base lesions. CT and MR
are used for lesion detection, tissue characterization and assessment of neurovascular and bone
involvement by the lesions. Both modalities provide useful information, one sometimes of greater
value than the other. T1-weighted MR imaging is useful in detecting skull base lesions, typically
surrounded by abundant fatty bone marrow. T2-weighted MR imaging is generally useful for tumor
tissue characterization. CT surpasses MR imaging in evaluating intratumoral calcification and bone
destruction or hyperostosis. To date, imaging features have been well-reported in individual skull
base tumors; however, correct diagnosis by imaging alone still presents a challenge. Knowledge of
clinical issues and awareness of variants of skull base tumors are of help in making a diagnosis.
The purpose of this article is to review pertinent clinical issues, typical imaging appearances and
certain imaging variations of common skull base lesions.

MeSH Keywords: Magnetic Resonance Imaging • Skull Base Neoplasms • Tomography, Spiral Computed

PDF fi­le: http://www.polradiol.com/abstract/index/idArt/901937

Background and magnetic resonance (MR) imaging; one complements

the other. T1-weighted MR imaging is useful in detecting
The skull base is the anatomic junction of the neural and skull base lesions typically surrounded by abundant fatty
facial viscerocranium. It has clinically unique importance bone marrow. Solid tumors with marked hyperintensity
because it supports the brain and contains the neurovascu- on T2-weighted images are likely to contain chondroid,
lar structures entering or exiting the skull. chordoid or myxoid matrix, while hypointensity suggests
abundant fibrous tissue or hemosiderin within the tumor.
Skull base lesions may originate within the skull base or CT surpasses MR imaging in evaluating intratumoral cal-
involve it by growth from either the intracranial dura or cification and bone destruction or osteosclerosis. Dural-
extracranial structures. A number of tumors and tumor- based tumors with hyperostosis of the underlying bones
like non-neoplastic lesions, with different cell types, can very often suggest meningiomas. Even now, when imaging
thus affect the skull base. This wide variety of lesions often plays an important role in the diagnosis process, knowl-
confuses beginners and experts alike. The purpose of imag- edge and awareness of clinical issues and variants of skull
ing includes detection, localization and characterization of base tumors are also of help. The first purpose of this arti-
skull base lesions. Estimation of tumor extent and inva- cle is to discuss the usefulness of non-contrast T1-weighted
sion to critical organs is important as well. These goals are MR imaging in detecting skull base lesions and the sec-
achievable through the use of computed tomography (CT) ond is to review pertinent clinical issues, typical imaging

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A B C

Figure 1. Normal fatty bone marrow infiltration of the skull base in childhood. (A–C) Non-contrast, T1-weighted images of a 3-month-old baby (A),
and 2.5-year-old (B) and 5-year-old (C) girls demonstrate serial signal change from iso- to hyperintensity of the bone marrow at the skull
base, corresponding to normal conversion from cellular to fatty marrow in childhood.

A B C

Figure 2. Bone metastasis to the skull base in a 53-year-old woman with a history of breast cancer. (A, B) On axial T2-weighted (A) and contrast-
enhanced T1-weighted (B) MR images, MR signals from the metastatic bone tumor of the right petrous apex resemble those from
the bone marrow in the contralateral normal petrous apex. (C) Non-contrast, T1-weighted (C) MR image most clearly delineates the
hypointense tumor (arrows).

appearances and certain imaging variants regarding skull Normal Bone Marrow Development and Variation of The
base tumors and tumor-like lesions. Skull Base
The Rationale for Imaging The skull base is comprised of the frontal, ethmoid, sphe-
noid, temporal and occipital bones. The frontal and
Skull base is generally examined using CT, MR imaging or ethmoid bones compose the anterior skull base. The middle
both. Other than diagnosis by using each modality, mul- skull base is formed by the sphenoid bone and the ante-
timodal image fusion techniques across CT, MR imaging rior aspect of the temporal bone. The occipital bone and the
and, in some cases, conventional angiography, are recently posterior aspect of the temporal bone constitute the poste-
favored by neurosurgeons as an intraoperative image guid- rior skull base.
ance [1]. 18F-fluoro-deoxyglucose positron emission tomog-
raphy (PET) or PET-CT is often performed to assess met- It is widely known that conversion from cellular to fatty
abolic activity of skull base lesions and to locate the pri- bone marrow begins early after birth at the clivus and the
mary tumor or other similar lesions located in areas other calvaria [3]. The clivus, one of the key components of the
than the skull base. The purpose of CT and MR imaging is skull base, is comprised of the basisphenoid and the basi-
to evaluate (a) lesion characterization, (b) paranasal sinus occipital bones and contains abundant medullary cavity.
involvement, (c) involvement of extracranial soft tissues, (d) Non-contrast T1-weighted MR imaging is very sensitive to
bone involvement, (e) neurovascular involvement, (f) inva- bone marrow conversion in childhood (Figure 1). An early
sion to brain and (g) variation of skull base anatomy [2]. MR study on cranial bone marrow in children has shown
that the marrow with uniform cellular marrow signal is no

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and absent or hypoplastic sinuses are developmental vari-

ants that are occasionally seen. Arrested pneumatization
is another less recognized variation that may simulate dis-
ease [4]; it corresponds to failure of pneumatization before
respiratory mucosa has fully extended into developing
sinuses. Arrested pneumatization typically appears as a
non-expansile lesion with osteosclerotic borders, internal
fat and curvilinear calcifications in the basisphenoid bone
or adjacent skull base [4].

The Basics of Skull Base Lesions

In the following section, basic clinical knowledge and
imaging features of skull base lesions will be reviewed
based on typical locations where the lesions occur.
Otolaryngologic lesions that typically occur in the temporal
bone are not included here for the sake of conciseness.

Anterior Skull Base

Olfactory neuroblastoma
Figure 3. Olfactory neuroblastoma in a 38-year-old man. Coronal
post-contrast, fat-suppressed, T1-weighted image shows Olfactory neuroblastoma or esthesioneuroblastoma is
homogenously enhancing tumor in the right nasal cavity a malignant neoplasm of the neural crest origin aris-
with intracranial extension (arrow). ing from the olfactory epithelium located in the upper
nasal fossa [5]. It accounts for 3–5% of all intranasal neo-
longer observed in the clivus or the calvaria after the age of plasms [6,7]. It has bimodal incidence peaks in the teens
seven years [3]. Most skull base tumors show intermediate and 40s with a slight female predominance [6]. The typical
to low signals on non-contrast T1-weighted MR images and clinical presentation is nasal stuffiness (70%) and bleeding
therefore skull base lesions are often very clearly deline- (46%) [7]. Because of the high local recurrence rate, treat-
ated by surrounding hyperintense fatty marrow (Figure 2). ment includes surgery and radiation therapy even for a
small tumor confined to the nasal cavity [7].
The process of paranasal sinus, mastoid and accessory skull
base pneumatization follows marrow conversion, develop- Olfactory neuroblastoma appears as iso- to slight-
ing from infancy to adolescence. Accessory pneumatization ly hyperdense on non-contrast CT, iso- to hypointense

A B

Figure 4. Perineural tumor spread of nasopharyngeal carcinoma in a 63-year-old woman. (A) Coronal, non-contrast, T1-weighted, MR image shows
nasopharyngeal mucosal wall thickening. The tumor invades into the basisphenoid and spreads through the left Vidian canal (arrow) to
the pterygopalatine fossa (not shown), and then goes up to the left cavernous sinus along the maxillary branch of the left trigeminal
nerve. The left cavernous sinus is also replaced by the tumor (arrow head). (B) Axial, T2-weighted MR image shows the mass with
intermediate intensity along the maxillary branch of the left trigeminal nerve (arrows).

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A B

C D

Figure 5. Fibrous dysplasia of the sphenoid in a 61-year-old man. (A, B) Axial, T1-weighted (A) and T2-weighted (B) MR images show a mass lesion
(arrows) of the sphenoid with mixed intensity ranging from hypo- to hyperintense. (C) Axial, fat-suppressed, contrast-enhanced MR
image reveals inhomogeneous but strong enhancement of the mass (arrows). (D) Sagittal-reformatted bone window CT image shows a
mixed ground-glass and cystic appearance that is typical of fibrous dysplasia. A convex margin (arrows) also suggests fibrous dysplasia
rather than arrested pneumatization. Fibrous dysplasia incidentally found in the elderly can be mistaken for a neoplasm on MR images.

on non-contrast T1-weighted MR images and hyper- Anterior and Middle Skull Base
to isointense on T2-weighted MR images (Figure 3).
Intratumoral cyst formation or necrosis is not uncom- Skull base invasion from head and neck tumor
mon [8]. Intracranial extension of olfactory neuroblas-
toma sometimes has characteristic marginal cysts where Head and neck tumors sometimes invade the anterior or
the tumor approaches the brain [9]. On contrast-enhanced the middle skull base by either direct invasion or perineu-
T1-weighted images, the tumor shows homogeneous ral tumor spread. Perineural tumor spread into the intrac-
enhancement but can be inhomogeneous with necrosis. ranial space can occur in adenoid cystic carcinoma, squa-
Diffusion-weighted imaging typically shows restricted mous cell carcinoma, lymphoma and melanoma [10]. It is
diffusion. most common in patients aged 40 to 60 years with a male
predominance and typically spreads along the branches of
the trigeminal nerve or the facial nerve.

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A B C

Figure 6. Pituitary adenoma in a 38-year-old man. (A) Axial, non-contrast, T1-weighted MR image shows a large, infiltrating, hypointense mass
(arrows) at the central skull base. Bone invasion can be confirmed by the loss of normal high signals from fatty bone marrow. Intratumoral
hemorrhage (arrow head) can be also seen. (B) On coronal-reformatted, contrast-enhanced MR image, the mass shows moderate
enhancement with a very indistinct margin (arrows). (C) Axial, bone window CT image shows bone destruction of the central skull base
(arrows). A pathological analysis revealed a prolactin-producing pituitary adenoma.

CT typically shows the expansion of the skull base foramen location is the most common form of pituitary adenomas. A
with an enlarged nerve in it. Non-contrast T1-weighted subgroup of giant pituitary adenomas, called invasive pitu-
MR imaging demonstrates a hypointense, enlarged nerve itary adenoma, may show aggressive invasion into the skull
with a loss of perineural fat. The enlarged nerve shows base and thus can be easily mistaken for other skull base
variable intensity on T2-weighted MR imaging. A tubular, malignancies (Figure 6) [14,15].
enhanced mass is shown on contrast-enhanced MR imaging
(Figure 4) [11]. Clinical symptoms are caused by the local mass effects and/
or hormonal abnormalities. Prolactin-producing adenoma
Fibrous dysplasia accounts for 57% of all pituitary adenomas [16]. A dopa-
mine agonist should be used for initial treatment for prol-
Fibrous dysplasia (FD) can affect any bones in the body; the actin-producing pituitary adenomas [17]. Trans-sphenoidal
skull and the facial bones are affected in 10–25% of patients surgery is most commonly employed for initial treatment
with monostotic FD and in 50% of patients with polyostotic of other pituitary adenomas. Radiation therapy is employed
FD [12]. FD most commonly presents in the teens or 20s with when a residual tumor grows progressively.
female predominance but can be incidentally found in the
elderly on CT or MR imaging performed for other purpos- Pituitary adenomas typically show iso- to mild hyper-
es. Symptoms depend on the site of the lesion. Observation density on non-contrast CT, iso- to hypointensity on
is chosen in asymptomatic patients. Curettage or volume T1-weighted MR images and mild hyper- to isointensity on
reduction surgery may be employed if there is bone pain, T2-weighted MR images. They sometimes contain intratu-
skeletal deformity or progressive neurological deficit. moral cysts or hemorrhage. Contrast enhancement of the
tumor is moderate and mostly homogeneous. Large adeno-
FD shows three dominant patterns on CT: the ground-glass mas expand the sella. Invasive pituitary adenomas may
pattern (56%), the homogeneously sclerotic pattern (23%) erode or destroy adjacent bones.
and the predominantly cystic pattern (21%) [12]. FD often
appears as a mixture of these patterns and may show Chondrosarcoma
variable appearances on CT. FD is predominantly hypoin-
tense on both non-contrast T1-weighted and T2-weighted Skull base chondrosarcoma is thought to arise from the car-
MR images. Non-mineralized areas and regions of cystic tilage, bones with endochondral ossification and primitive
changes show hyperintensity on T2-weighted MR images. mesenchymal cells of the meninges. It accounts for 6% of
Heterogeneous enhancement is sometimes seen on con- skull base tumors [18] and can occur at all ages from the
trast-enhanced T1-weighted MR images, which can be eas- teens into the 90s. No sex predominance has been reported.
ily confused with a skull base neoplasm (Figure 5) [12]. Skull base chondrosarcoma most commonly occurs along
the petrooccipital fissure (Figure 7). The sphenoid is the next
Middle Skull Base most common location (Figure 8) but is far rarer [18]. Typical
clinical symptoms include abducens nerve palsy and head-
Pituitary adenoma ache [18]. Surgery and adjuvant radiation therapy are most
commonly employed for treatment [18]. Skull base chondro-
Pituitary adenomas are benign tumors arising from the sarcoma is well-differentiated, grade 1 chondrosarcoma in
anterior lobe of the pituitary gland. They are the most many cases, and the prognosis is rather favorable [18].
common cause of sellar masses that occur from the age of
20 years, accounting for up to 10% of all intracranial neo- Skull base chondrosarcoma typically forms an expansile
plasms [13]. Intrasellar or combined intra- and suprasellar mass with multilobulated margins. CT shows an osteolytic

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A B

C D

Figure 7. Skull base chondrosarcoma in a 46-year-old woman. (A) Axial, non-contrast, T1-weighted MR image shows an expansile, hypointense
mass (arrows) engulfing the left petrous apex and the clivus. The epicenter of the tumor is likely located at the left petroclival fissure.
(B) On an axial, fat-suppressed, T2-weighted MR image, most of the tumor shows very high signals (arrows). The left mastoid air cells lose
aeration, likely because the Eustachian tube is obstructed. (C) Axial, contrast-enhanced, MR image with fat suppression shows peripheral
enhancement (arrows). (D) On an axial, bone window CT image, punctuate calcification is seen in the tumor (black arrow). The margin of
the tumor is rather distinct (white arrows).

mass. Typical ring and arc calcification can be seen in 41% remnant or from benign notochordal cell tumors [20]. Skull
of the tumors [19]. The tumor demonstrates iso- to hypoin- base chordomas account for 1% of primary brain tumors
tensity on non-contrast T1-weighted and hyperintensity on and occur in the vicinity of the clivus, constituting one-
T2-weighted MR images. Hypointense foci on T2-weighted third of all chordomas [21]. Chordomas can occur at all ages,
images may correspond to hemorrhage or fibrous tissue. with the incidence peak at 30 to 50 years and a male-to-
The contrast enhancement pattern is heterogeneous with a female ratio of 2: 1 [21]. Typical clinical symptoms of clival
predominance at the periphery in 73% of patients (Figures 7 chordomas include headache and diplopia [22]. Surgery and
and 8) and homogenous in 27% [19]. adjuvant radiation therapy are most frequently employed.

Chordoma Skull base chordoma typically forms a multilobulated expan-

sile mass in the clivus (Figure 9). However, approximately
Chordoma is a low to intermediate, locally aggressive one-third of skull base chordomas occur in off-midline
malignancy, presumably arising from the notochordal positions (Figure 10) [23]. Chordomas show mild hyper- to

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A B

Figure 8. Chondrosarcoma of the sphenoid in a 56-year-old man. (A) Axial, T2-weighted MR image shows a lobulated mass at the sphenoid
(arrows). The mass contains abundant intermediate signals but also includes hyperintense foci. (B) Axial, contrast-enhanced, MR image
with fat suppression shows peripheral enhancement that indicates chondrogenic tumor. A pathological analysis revealed grade 1
chondrosarcoma.

hypodensity on non-contrast CT. Bone destruction may be will not be discussed in this section. Vestibular schwannoma
more aggressive in chordomas than in chondrosarcomas (VS) is most common, constituting more than 80% of skull
(Figure 9). Chordomas show iso- to hypointensity on non- base schwannomas. Trigeminal schwannoma (TS) accounts
contrast T1-weighted and hyperintensity on T2-weighted for 1–8% of intracranial schwannomas [26], followed by jug-
MR images. Signal intensity of the tumor can be modified ular foramen schwannoma (JFS) at 2.9% [27]. Hypoglossal
by intratumoral hemorrhage and calcification. Contrast- schwannoma is extremely rare. Skull base schwannomas
enhanced T1-weighted MR imaging shows moderate to most commonly occur between the ages of 20 to 60 years, and
strong enhancement with a typical honeycomb appear- women are more commonly affected. VS commonly causes
ance [21]. The imaging characteristics of chordomas are sub- chronic progressive hearing loss. TS causes facial pain and
stantially shared by chondrosarcomas, but chordomas typi- paresthesia in 91% of patients [26]. JFS may cause palsies in
cally arise in the midline location (clivus) and will infiltrate the 9th to 11th cranial nerves, but may be tolerated by their
into surrounding tissues more often than chondrosarcomas. opposite nerve functions [28]. As a result, up to 50% of the
patients with JFS may seek medical help only after tumors
Giant cell tumor have become large and hearing loss has developed [27,28].
Surgery is the treatment of choice for skull base schwanno-
Giant cell tumor (GCT) is a rare benign intraosseous neo- mas. Stereotactic radiosurgery or other radiotherapy tech-
plasm containing multinuclear giant cells. It is rare in the niques may be employed to follow incomplete resection.
skull base and most commonly occurs in the sphenoid bone
followed by the temporal bone [24]. GCT is most common in Imaging studies show a tubular or dumbbell-shaped mass
young adults, with a slight female predominance [25]. along the course of the cranial nerves with distinct mar-
gins (Figures 11, 12). Skull base schwannomas are typical-
GCT shows an expansile intraosseous mass with reac- ly hypodense on non-contrast CT, iso- to hypointense on
tive bone remodeling on CT. GCT shows low to intermedi- non-contrast T1-weighted MR images and hyperintense on
ate signal intensity on both T1-weighted and T2-weighted T2-weighted MR images. Intratumoral cyst or hemorrhage
MR images. Intra-lesional hypointensity on T2-weighted are common. Contrast-enhanced CT or MR show variable
images corresponds to hemosiderin or calcification typical and inhomogeneous enhancement depending on the pre-
to GCT [24]. Contrast-enhanced T1-weighted MR imaging dominance of the cell-rich Antoni type A and the looser
shows variable enhancement patterns [24]. Antoni type B lesions, and the presence of cyst or hemor-
rhage in the tumor [29].
Middle and Posterior Skull Base
Anywhere in the Skull Base
Schwannoma
Meningioma
Skull base schwannomas include vestibular, trigeminal, jug-
ular foramen and hypoglossal schwannomas. Facial nerve Skull base meningiomas account for about 36–50% of all
schwannoma commonly occurs within the temporal bone and meningiomas [30,31]. Their common locations include the

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A B

C D

Figure 9. Clival chordoma in a 67-year-old man. (A) Axial, non-contrast, T1-weighted MR image shows a lobulated, hypointense mass lesion
(arrow) in the upper part of the clivus. (B, C) The mass shows hyperintensity on an axial, T2-weighted MR image (B) and honeycomb-like
enhancement on a sagittal, fat-suppressed, contrast-enhanced T1-weighted MR image (C). (D) Axial bone window CT shows cortical bone
destruction of the clivus and likely a residual bone fragment within the tumor (arrow). The pathologic analysis demonstrated chordoma.

olfactory groove, the tuberculum sellae, the sphenoid ridge radiotherapy or radiosurgery alone are indicated only for
for the anterior skull base, petroclival and pericavernous tumors that can be treated adequately with these modalities
regions for the middle skull base, and the foramen mag- or for cases that are associated with postsurgical complica-
num and the jugular foramen for the posterior skull base. tions (e.g. cavernous sinus or petroclival meningiomas) [31].
Middle-aged women are most commonly affected, which is
similar to meningiomas in other locations. Meningioma is The imaging features of skull base meningiomas are simi-
often slowly growing and asymptomatic. Larger tumors can lar to those of typical meningiomas, showing a sessile or
cause both non-specific symptoms (headache and dizziness) lentiform, well-circumscribed mass with a broad-based
and site-specific symptoms (anosmia, proptosis, ophthal- dural attachment. It typically demonstrates hyperdensity
moplegia, and lower cranial nerve palsies). Surgery is usu- on non-contrast CT, and iso- to hypointensity on both T1-
ally employed for treatment of symptomatic meningiomas and T2-weighted MR images. Uniform enhancement can
and asymptomatic tumors that are large and infiltrating. be seen after contrast administration [30]. Meningiomas
Adjuvant radiotherapy over a series of sessions or stereo- cause hyperostosis in the underlying bones in up to 90%
taxic radiosurgery with a high single dose of radiation may of the tumors [32] (Figure 13) but can also cause osteolysis.
be employed in cases of incomplete resection. Stereotactic Skull base meningioma with atypical imaging features may

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A B

Figure 10. Chordoma in a 35-year-old man. (A) Axial, fat-suppressed, T2-weighted MR image shows a lobulated, very hyperintense mass (arrows)
in the left petrous apex. The tumor extends to the left Meckel’s cave but the epicenter of the tumor lies in the left petrous bone. (B) Axial,
contrast-enhanced MR image with fat suppression shows rather homogeneous, strong enhancement of the tumor (arrows). This is a case
of lateral variants of chordoma. About one-third of skull base chordomas occur at off-midline positions.

A B

Figure 11. Trigeminal schwannoma in a 38-year-old man. (A) Axial, T2-weighted image show a dumbbell-shaped, partly cystic mass (arrows)
extending from the left parasellar region to the left pterygopalatine fossa. (B) Sagittal, contrast-enhanced MR image with fat
suppression shows both intra- and extracranial components of the tumor (arrows). The third branch of the trigeminal nerve seems
normal (arrow head). Schwannoma of the second branch of the trigeminal nerve was confirmed on surgery.

mimic other skull base malignancies including metastasis malignancies. The skull and the skull base are frequent sites
and multiple myeloma [33]. for bone metastasis. Bone metastasis can be either osteolyt-
ic or osteoblastic, depending on the activities of osteoblasts,
Bone metastasis osteoclasts and relevant cytokines [34]. Common clinical
symptoms include headache, nausea and local neurological
It is estimated that more than one-third of people who deficits. Treatment of bone metastasis depends on tumor
die of cancer have bone involvement [34]. Bone metas- volume, lesion location, tumor pathology and the general
tasis often occurs in patients with breast, lung or pros- condition of the patient. Radiotherapy and/or systemic
tate cancer, but can be found in most cases of advanced chemotherapy are frequent choices of treatment.

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A B

Figure 12. Jugular foramen schwannoma in a 66-year-old man. (A) Axial, T2-weighted image shows a mixed solid and cystic mass (arrows)
extending through the left jugular foramen. (B) Axial, post contrast, T1-weighted image shows heterogeneous enhancement of the
tumor (arrows).

A B

Figure 13. Skull base meningioma in a 29-year-old woman. (A) Coronal-reformatted, post contrast, T1-weighted image shows a meningioma with
typical dural tail sign (arrows). (B) Coronal-reformatted, bone window CT image demonstrates hyperostosis of the underlying bones
(arrows).

Bone metastasis typically forms a destructive mass cen- Multiple myeloma

tered in the bone with no sclerotic rim on CT. Osteolytic
metastasis is more common. Bone metastasis shows typi- Multiple myeloma is a low grade hematologic malignancy
cally hypointense to normal bone marrow on non-contrast characterized by monoclonal proliferation of B lympho-
T1-weighted images (Figure 2). Diffusion-weighted MR cytes in the plasma cell lineage. It accounts for 10% of all
images can improve detection of skull metastasis for breast hematologic tumors [36] and most commonly occurs in the
and lung cancer but may be insensitive to prostate can- elderly, with a slight male predominance [37]. Anemia and
cer [35]. Signal intensity on T2-weighted images is variable. bone pain are typical symptoms found in 73% and 58% of
On contrast-enhanced T1-weighted images, bone metastasis myeloma patients, respectively [37]. Treatment depends on
sometimes enhances to normal bone marrow signal, thus the disease status, ranging from observation with support-
the addition of a fat suppression technique is desirable. ive care to chemotherapy.

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A B

Figure 14. Multiple myeloma in a 76-year-old man. (A) Axial, non-contrast T1-weighted MR image shows a hypointense mass (arrow) destroying
the left petrous bone and the left lower clivus, in contrast to normal hyperintense fatty bone marrow. (B) On an axial, diffusion-weighted
image, the mass is hyperintense, equivalent to the brain tissue.

Although the traditional Durie and Salmon staging system differential diagnosis for skull base tumors in pediatric
employs plain radiographs [38], CT is faster, more sensitive populations. Bone pain and palpable mass are typical clini-
and better tolerated by patients [39]; although radiation cal symptoms. Treatment depends on whether LCH affects
exposure to the patient is a drawback [40]. Multiple mye- a single system or many systems, and on the number and
loma typically appears as multiple, punched-out, lytic bone location of LCH lesions. Patients with bone involvement
lesions on CT, and solitary bone plasmacytoma as a sin- of the skull base are at increased risk of developing CNS
gle lytic bone lesion without sclerotic borders. Skull base involvement by LCH and endocrine abnormalities [41].
extramedullary plasmacytoma may erode adjacent bones.
LCH appears as a soft tissue mass replacing bones of the
MRI is the most sensitive modality for detection of both skull base on CT. LCH shows non-specific hypointensity
diffuse bone marrow involvement and solid plasma cell on non-contrast T1-weighted and mild hyperintensity on
tumors [39]. MR imaging shows focal myeloma lesions T2-weighted MR images. Uniform and strong enhancement
in approximately 30% of patients [39]. Focal myelo- can be observed after contrast administration [42].
ma lesions show hypointensity to normal fatty mar-
row on T1-weighted MR images and hyperintensity on Conclusions
T2-weighted MR images (Figure 14). Diffusion-weighted
images can detect smaller myeloma lesions. Diffuse infil- A variety of lesions can occur in the skull base, and both
tration is less common and shows a diffuse decrease in CT and MR imaging contribute beneficially to the charac-
the signal of the marrow on T1-weighted MR images terization of these lesions. Skull base lesions are often most
and a variable increase in the signal of the marrow on noticeable on non-contrast T1-weighted MR images. Some
T2-weighted MR images. Up to 30% of myeloma patients skull base lesions may develop in typical locations and
have normal-looking bone marrow signals on MR imag- show characteristic signals on MR images; however, varia-
es [39]. Contrast-enhanced MR studies show marked tions do occur. Knowledge of clinical issues and awareness
enhancement. Multiple myeloma has multiple lesions or of variations of imaging features would help improve diag-
diffuse marrow abnormality shown on MR images. A single nostic accuracy for skull base lesions.
lesion may suggest plasmacytoma.
Ethical concern
Langerhans cell histiocytosis
This review study was approved by the local ethical com-
Langerhans cell histiocytosis (LCH) is a rare histiocytic mittee and written informed consent was waived.
disorder characterized by single or multiple osteolytic
bone lesions demonstrating histiocytic infiltration with Conflicts of interest
or without invasion of the extraskeletal organs. LCH most
commonly occurs in children. Bones are affected in 77% None.
of patients with LCH [41] and therefore LCH can be a top

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