Saudi Journal of Ophthalmology (2012) 26, 373–383

Oculoplastic Imaging Update

Orbital masses: CT and MRI of common vascular lesions, benign

tumors, and malignancies
Sarah N. Khan, MD; Ali R. Sepahdari, MD ⇑

Abstract

A wide variety of space occupying lesions may be encountered in the orbit. CT and MR imaging frequently help confirm the pres-
ence of a mass and define its extent. Characteristic imaging features may help distinguish among lesions that have overlapping
clinical presentations. This review focuses on some of the common orbital masses. Common vascular lesions that are reviewed
include: capillary (infantile) hemangioma, cavernous hemangioma (solitary encapsulated venous-lymphatic malformation), and lym-
phangioma (venous-lymphatic malformation). Benign tumors that are reviewed include: optic nerve sheath meningioma, schwan-
noma, and neurofibroma. Malignancies that are reviewed include: lymphoma, metastasis, rhabdomyosarcoma, and optic glioma.
Key imaging features that guide radiological diagnosis are discussed and illustrated.

Keywords: Benign orbital masses, Orbital vascular lesions, Malignant orbital masses, Pediatric orbital masses

Ó 2012 Saudi Ophthalmological Society, King Saud University. All rights reserved.
http://dx.doi.org/10.1016/j.sjopt.2012.08.001

Introduction Our goal is to review the most commonly encountered le-

sions to make the reader aware of their typical appearances
A wide variety of processes can produce space-occupying and presentations, with emphasis on key imaging features
lesions in and around the orbit. These include benign neo- that may distinguish among similar lesions.
plasms, malignant neoplasms, vascular lesions, inflammatory This review focuses on the most common vascular masses,
disease, congenital lesions, and infection, among other benign neoplasms, and malignant neoplasms. Due to space
causes. Imaging can be used to precisely localize a lesion, constraints, infection1–3 and inflammatory disease3–6 are not
to help establish a diagnosis or generate a differential diag- discussed here. In addition, a number of other vascular le-
nosis that guides management, to follow a known lesion for sions and neoplasms are not discussed, including orbital ve-
progression, or some combination of these. nous varix,3,7,8 arteriovenous malformation,3,9 cavernous
The first step in establishing a differential diagnosis is sinus thrombosis,3,10 carotid cavernous fistula,3,7,8 a variety
obtaining an accurate clinical history. CT is excellent for con- of benign and malignant mesenchymal tumors,3,11,12 epithe-
firming the presence of a mass, is well tolerated, can be rap- lial neoplasms of the lacrimal apparatus,3,7,13–15 and ocular
idly obtained at most medical centers, and gives clues to the tumors that secondarily involve the orbit.3,7 One may also
specific diagnosis. Although MRI can be more difficult to per- encounter other masses that are not true neoplasms, such
form, it can reveal additional key imaging features that allow as dermolipomas,3,16,17 dermoid cysts,3,18–20 and epidermoid
one to more confidently distinguish between similar appear- cysts.3,20 For discussion of these lesions, and others, we di-
ing lesions, and often arrive at a single most likely diagnosis. rect the reader to the referenced sources.

Available online 17 August 2012

Department of Radiological Sciences, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, United States

⇑ Corresponding author. Address: Department of Radiological Sciences, David Geffen School of Medicine, University of California Los Angeles, 757
Westwood Plaza, Suite 1621D, Los Angeles, CA 90095, United States. Tel.: +1 310 267 9785; fax: +1 310 267 3635.
e-mail addresses: [email protected], [email protected] (A.R. Sepahdari).

Peer review under responsibility Access this article online:

of Saudi Ophthalmological Society, www.saudiophthaljournal.com
King Saud University Production and hosting by Elsevier www.sciencedirect.com
374 S.N. Khan, A.R. Sepahdari

Vascular lesions Key features

Bright T2 signal, lobular borders, fine internal flow voids,
Capillary hemangioma (infantile hemangioma) extraconal location, and intense homogeneous enhancement.

Clinicopathologic features ‘Cavernous hemangioma’ (solitary, encapsulated

Although the term ‘‘hemangioma’’ is sometimes used to venous-lymphatic malformation)
describe certain vascular malformations, it more appropri-
ately describes a true neoplasm with vascular channels lined
Clinicopathologic features
by proliferating endothelial cells.21 Capillary hemangiomas
‘Cavernous hemangioma’ is a term that is widely used to
are the commonest tumors of infancy,7,22–24 typically appear-
describe a solitary, encapsulated venous-lymphatic malfor-
ing as a reddish macule in the first 6 months of life. A prolif-
mation, the most common vascular lesion of the orbit.30
erative phase occurs up to 10 months, followed by a slow
The typical clinical presentation is of mostly painless propto-
involution phase for up to 10 years.21,25,26 Though typically
sis (mean 5–6 mm), pain, lid swelling, diplopia, lump, and
sporadic, they may occur as part of a genetic syndrome such
recurrent obstructed vision. Middle-aged women are the
as PHACES syndrome.25
most commonly affected group, and the average duration
from symptom onset to presentation is 4 years.22 The most
Imaging findings frequent locations are the retrobulbar muscle cone, espe-
Contrast enhanced MRI is the preferred modality for imag- cially the lateral aspect of the intraconal space.31 However,
ing hemangioma, although CT can also be considered if it is a small minority (less than 10%) of these lesions are extraco-
not possible to sedate the patient for MRI. The diagnosis is nal.32 Cavernous hemangiomas rarely bleed33 due to firmer
typically well-established clinically, but imaging is indicated texture from the surrounding support of rich fibrous tissue.34
to assess the extent of the lesion and mass effect on adjacent Orbital cavernous hemangiomas are typically solitary and uni-
structures. With deep lesions, the clinical diagnosis may be lateral, though multifocal and bilateral lesions have been re-
challenging. Capillary hemangioma typically shows: (1) lobu- ported.35–38
lar contour borders, (2) bright T2 signal with T2 dark septa
between lobules, (3) fine internal flow voids, (4) intense,
Imaging appearance
homogeneous enhancement, and (5) preservation of adja-
Cavernous hemangioma typically appears as a well-
cent bone (Fig. 1). Capillary hemangioma can have an atypi-
circumscribed intraconal mass.39–42 Although most lesions are
cal appearance without all of these features. Nevertheless, if
ovoid or round, larger lesions have lobulated margins. Larger
these features are not all present, then alternative diagnoses
lesions will distort surrounding structures, as opposed to
should be considered, including rhabdomyosarcoma. DWI
lymphoma which molds around structures. CT shows homo-
may help distinguish these lesions in challenging cases, as
geneous soft tissue density, and may show small calcifications
rhabdomyosarcoma typically shows lower ADC and brighter
or phleboliths.39 MR shows isointense T1 signal, bright T2
DWI signal than hemangioma.27–29 CT may demonstrate
signal, dark internal septations, and a dark circumferential
bony orbital expansion or scalloping with rapidly growing
rim that represents a fibrous pseudocapsule (Fig. 2).22,34,43,44
lesions. True bone invasion and calcifications are rarely seen.
MR classically demonstrates nodular enhancement on
CT and MR may both demonstrate fat deposition and
early post-contrast images, and progressive accumulation
heterogeneous appearing enhancement, if imaging is
of contrast on delayed post-contrast images.31 The same
performed during the involution phase.9,23–25
findings are seen with multiphase CT, though concerns
related to the increased radiation exposure of multiphase
Differential diagnosis CT make this technique relatively less desirable. Small lesions
Rhabdomyosarcoma, vascular malformation, infantile often show early, uniform enhancement. MR angiography
fibromatosis, and infantile fibrosarcoma. and CT angiography are generally not able to identify the

Figure 1. A 7 month-old girl with capillary (infantile) hemangioma. A. Axial T2 shows a well-defined, lobular, hyperintense mass with fine internal signal
void (arrow) that likely represents an internal high-flow vessel. Septa in between lobules are slightly more linear. B. Axial fat-suppressed post contrast T1
shows intense, homogeneous enhancement typical of a proliferative phase lesion. The intact bony cortex at the zygoma is well demonstrated as a
hypointense line (arrow).
Orbital masses: CT and MRI of common vascular lesions, benign 375

Figure 2. Cavernous hemangioma (solitary encapsulated venous-lymphatic malformation). A. Axial T2 shows an ovoid, homogeneous hyperintense mass
() that distorts the medial rectus muscle (black arrow) and the optic nerve (white arrow). B. Axial T1 with contrast, obtained immediately after contrast
injection, shows nodular enhancement. C. Para-sagittal oblique fat suppressed T1 with contrast, obtained 12 min after contrast injection, shows more
uniform contrast enhancement. This slowly progressive nodular enhancement is typical of cavernous hemangioma, though also occasionally seen in
schwannoma.

feeding vessels of a cavernous hemangioma, likely due to components of the tumor are hyperdense compared to brain
their small caliber. tissue on unenhanced CT scans.50–52
MR is accurate for delineating the anatomic location and
Differential diagnosis vascular components, and fluid filled levels or menisci can
Venous varix, schwannoma, optic nerve sheath meningi- be seen.46 The mass is usually isointense to slightly hyperin-
oma, and lymphoma. tense relative to normal brain tissue on T1 weighted imaging
and very hyperintense relative to the brain on T2 weighted
imaging46, with internal septations (Fig. 3). T1 and T2 signal
Key imaging features intensity vary depending on the presence and age of internal
Intraconal location; very bright T2 signal with hypointense blood products. The venous component of a lesion will typi-
pseudocapsule; and an early nodular enhancement with pro- cally enhance, whereas the lymphatic component will only
gressive accumulation of contrast on later phase images. show fine enhancement of septations. No flow voids or en-
larged feeder vessels are usually found, in keeping with the
Lymphangioma (venous lymphatic malformation) low flow nature of lymphangioma and differentiating it from
high flow lesions, including high-flow vascular malformations
Clinicopathologic features and true neoplasms such as capillary hemangioma.50,53,54
‘Lymphangiomas’ are benign vascular malformations (ve-
nous lymphatic malformations), commonly affecting children
and rarely involving the orbit. They are unencapsulated and
composed of fibrous material including endothelial lacunae
filled with blood or serious fluid.45 Lymphangiomas tend to
enlarge suddenly due to intralesional bleeding, but may also
enlarge with upper respiratory infection.46 Symptoms in-
clude: swelling, intraorbital hemorrhage, ocular proptosis,
blepharoptosis and cellulitis. Ocular complications include:
astigmatism, corneal exposure, hyperopia secondary to pres-
sure on posterior globe, strabismus, glaucoma and compres-
sive optic neuropathy.47–49

Imaging findings
Despite belonging to the same pathological category as
‘cavernous hemangioma’ (i.e. low flow vascular malformation
without endothelial proliferation), lymphangioma has a differ-
ent appearance and growth pattern. In keeping with their
unencapsulated nature, lymphangiomas exhibit an infiltra-
tive, trans-spatial growth pattern, often involving both the
intraconal and extraconal compartments and pre- and
post-septal compartments, violating fascial planes. Both Figure 3. Lymphatic malformation (lymphangioma). Axial T2 shows a
hyperintense, trans-spatial mass involving the intra- and extra-conal
microcysts and macrocysts may be identified.50 CT may dem- compartments. Hypointense septations (black arrows) are typical of this
onstrate phleboliths in the venous component of a lesion, lesion. Layering blood products (white arrows) are variably present, and
and may demonstrate bony abnormalities.46 Venous or solid indicate recent intralesional bleeding.
376 S.N. Khan, A.R. Sepahdari

Figure 4. Optic nerve sheath meningioma. A. Axial T2 shows a homogeneous, hypointense mass () that mildly distorts the globe (arrow). B. Axial fat
suppressed T1 with contrast shows homogeneous enhancement with a well-defined margin (blue arrow), and encasement of the optic nerve (white
arrow). Globe distortion helps distinguish this lesion from lymphoma, as does the clinical history of slowly progressive, painless vision loss.

Differential diagnosis producing the classically described ‘tram track’ sign.59 In

Cavernous hemangioma, capillary hemangioma, dermoid, these cases, fat-suppressed post-contrast T1-weighted
and rhabdomyosarcoma. images in the coronal plane are essential in making the diag-
nosis (Fig. 5). Any part of the nerve may be involved, and
Key imaging features radiologic diagnosis can be challenging when only the intra-
Trans-spatial, markedly T2 bright non-enhancing mass canalicular portion of the nerve is affected. Fat suppression is
with internal septations, with or without layering blood prod- helpful in these cases, but can also be degraded by blooming
ucts and solidly enhancing components. artifact around a well-pneumatized sphenoid sinus. For this
reason, coronal post-contrast images without fat suppression
Benign neoplasms should also be considered.
Optic nerve sheath meningioma is distinguished from op-
Optic nerve sheath meningioma tic glioma by the fact that the optic glioma expands the optic
nerve. Orbital lymphoma can sometimes surround the optic
nerve and simulate optic nerve sheath meningioma by imag-
Clinicopathologic features
ing (Fig. 6). In such cases, lymphoma’s tendency to mold
The typical clinical presentation of optic nerve sheath
around structures helps differentiate it from meningioma,
meningioma is painless, gradual vision loss and proptosis in
which tends to distort structures. Lymphoma also typically
a woman between ages 30 and 50. Optic nerve sheath
shows less intense enhancement than meningioma. Meningi-
meningioma is typically unilateral but can be bilateral, partic-
oma frequently shows calcification on CT, whereas untreated
ularly in the setting of type II neurofibromatosis. Primary
lymphoma almost never calcifies. Perineuritic inflammatory
meningioma arises from capillary cells of the arachnoid
disease can produce a similar appearance to en plaque
around the intraorbital or intracanalicular portions of the op-
meningioma, but is clinically distinguished by the presence
tic nerve. Secondary meningioma arises intracranially from
of pain. Demyelinating optic neuritis is distinguished from
the sphenoid ridge, tuberculum sellae, or olfactory groove
en plaque meningioma by its typical homogeneous enhance-
and invades the optic canal and orbit by extension between
ment of the substance of the nerve, as opposed to the target-
the dura and arachnoid of the optic nerve.55,56 Visual distur-
like enhancement of meningioma.
bance in the affected eye is common. While some patients
only suffer transient visual loss lasting a few seconds, others
experience visual loss in a particular field of gaze.57,58 Optic Differential diagnosis
neuropathy, proptosis and strabismus occur later. Sarcoidosis, pseudotumors, lymphoma, and optic neuritis.

Imaging findings Key imaging features

The key imaging finding of optic nerve sheath meningi- Mass surrounding and constricting the optic nerve; calcifi-
oma is a homogeneously enhancing mass that surrounds cation on CT; extension of tumor into the optic canal; ‘‘tram
the optic nerve. CT may show calcification. MRI typically track’’ or ‘‘target’’ like enhancement.
shows homogeneous, intermediate T1 and T2 signals. The
optic nerve may be in the center of the lesion, or may be Nerve sheath tumors
eccentrically positioned. In some cases, the optic nerve will
show abnormal T2 hyperintense signal, which is presumably Clinicopathologic features
related to chronic venous insufficiency. Nerve sheath tumors can be divided into schwannomas
Optic nerve sheath meningioma may produce an expan- and neurofibromas, which are less common and typically
sile mass (Fig. 4). Alternatively, it may only form a thin sheet associated with NF-1. Schwannomas constitute 1% of orbital
around the optic nerve, termed ‘‘en plaque meningioma’’, tumors, typically occurring in young and middle-aged
Orbital masses: CT and MRI of common vascular lesions, benign 377

Figure 7. Schwannoma. Axial CT with contrast shows a hypodense, well-

Figure 5. En plaque optic nerve sheath meningioma. Coronal fat defined, extraconal mass with only weak enhancement. This appearance
suppressed T1 with contrast shows an enhancing mass (blue arrow) is nonspecific, and is often seen with schwannoma in addition to other
surrounding and constricting the optic nerve (white arrow). Fat sup- benign and malignant masses.
pressed post contrast coronal images are essential in identifying menin-
giomas with this growth pattern.

adults.30,60 They typically arise from intraorbital branches of

V1,60 and produce symptoms related to local mass effect.
Schwannomas are associated with NF-1 in 2–18% of pa-
tients.61 Neurofibromas are more strongly associated with
NF-1, and also produce symptoms related to mass effect.

Imaging findings
Schwannoma appears as a smooth, ovoid, orbital retrobul-
bar mass, found in the intra- or extra-conal space with the
long axis in the anterior–posterior direction (Fig. 7).39,62 CT
shows isodensity or hypodensity compared to gray matter.
MR shows isointense T1 signal, and T2 hyperintensity. Although Figure 8. Plexiform neurofibroma. Axial fat suppressed T2 shows a
some schwannomas may be cystic and non-enhancing,63 hyperintense mass in the left orbit, with extension through the superior
orbital fissure (arrow) into the parasellar region.
most schwannomas enhance with contrast. Although
schwannoma enhancement is typically more homogeneous
than cavernous hemangioma, in some cases schwannoma more infiltrative growth pattern (Fig. 8), and are frequently
can show the same type of early nodular enhancement with associated with other stigmata of NF-1.
progressive fill-in that is commonly seen with cavernous hem-
angioma. The MR and CT appearance of schwannoma can
overlap with optic nerve sheath meningioma. In challenging
Differential diagnosis
Cavernous hemangioma, meningioma, and lymphoma.
cases, one should look carefully for extension into the supe-
rior orbital fissure, which would favor schwannoma, or for
extension into the optic canal, which would favor meningi- Key imaging features
oma. Neurofibromas often have a similar appearance to sch- Smooth, homogeneous spherical mass. Extension into
wannomas, though plexiform neurofibromas typically show a superior orbital fissure. Enhancement is typically more

Figure 6. Lymphoma. A. Coronal fat suppressed T2 shows a homogeneous, somewhat hypointense mass that surrounds the optic nerve (white arrow).
Although the mass is mostly well-defined, there is a slightly ill-defined lateral margin (yellow arrow). B. Axial fat suppressed T1 shows molding around the
globe without globe distortion (arrow). Although optic nerve encasement is typical of meningioma, molding around the globe and poor margin definition
are features that suggest lymphoma. Lymphoma also commonly shows slightly less intense enhancement than meningioma, as demonstrated in this case
compared to Fig. 4.
378 S.N. Khan, A.R. Sepahdari

Figure 9. Lymphoma (multiple cases). A. Axial fat suppressed T1 with contrast shows ill-defined intraconal enhancement (arrow), representing infiltrative
tumor. B. Parasagittal oblique T1 with contrast in a different patient shows an enhancing mass expanding the superior rectus muscle (arrow). C. Axial T2
in a third patient shows diffuse expansion of the right lacrimal gland, with slightly lobular contour. The mass respects the cortex of the zygoma (white
arrow) and molds around the globe without distorting it (blue arrow). D. Axial DWI (same patient as ‘C’) shows very bright signal in both lacrimal glands
(yellow arrows), reflecting involvement by hypercellular tumor. Left lacrimal gland disease is better detected with DWI than with conventional images,
due to the relatively normal size of the gland and homogeneous appearance.

Figure 10. Breast cancer metastasis. A. Axial T2 shows a hypointense retrobulbar mass (arrow) that produces slight posterior tenting of the globe. B.
Axial fat suppressed T1 with contrast shows ill-defined enhancement. Enophthalmos and a T2 dark mass are highly suggestive of breast cancer
metastasis.

heterogeneous than lymphoma or meningioma, but less nod- orbital masses.66 Malignant lymphoma of the orbit is typically
ular than cavernous hemangioma. B cell lymphoma of a non-Hodgkin type, arising from muco-
sa-associated lymphoid tissue of ocular adnexa (MALTOM-
A).67 Systemic lymphoma may involve the orbit in 1.5–5% of
Malignant neoplasms patients.68,69 Up to 75% of patients with orbital lymphoma
may later develop systemic disease.70 Suspicion of malignant
Orbital lymphoid tumors (including malignant lymphoma is increased in the presence of systemic disease,
lymphoma) lacrimal duct or gland disease, and bilateral disease.71

Clinicopathologic features Image appearance

Reactive lymphoid lesions overlap histologically with Lymphoid tumors typically appear as homogeneous, lobu-
malignant lymphoma, and are sometimes grouped as ‘orbital lated masses on CT and MRI. They mold around normal struc-
lymphoid lesions’.64 The typical presentation is low-grade tures without deforming them, and typically do not erode
proptosis with minimal pain.1 Lymphoid masses comprise adjacent bone. They may be well defined, or infiltrative in
10–15% of all orbital tumors65 and up to 55% of malignant appearance. CT shows density similar to skeletal muscle.
Orbital masses: CT and MRI of common vascular lesions, benign 379

Metastatic lesions are found more in the anterior orbit

than the posterior orbit.76 The most frequent metastases to
the orbit are from: breast, lung, prostate, melanoma, carci-
noid, GI, renal cell, neuroblastomas and rhabdomyosarco-
mas. Rapidly progressive symptoms occur including:
diplopia, proptosis, decreased vision, pain, ptosis, and palpa-
ble mass.75

Imaging findings
CT is usually performed for orbital imaging, although MR
has superior soft tissue contrast. Tumor pattern can vary from
diffusely infiltrative with obscured anatomical landmarks to a
Figure 11. Carcinoid tumor metastasis. Parasagittal oblique fat sup-
pressed T1 with contrast shows a peripherally enhancing mass centered in
focal well-defined mass. Orbital metastases from breast can-
the distal portion of the inferior rectus muscle (arrow). cer are often diffuse and irregular growing along the rectus
muscles and fascial planes.76 Scirrhous (fibrotic) breast can-
cers are unique in their ability to produce enophthalmos
MRI demonstrates homogenous, intermediate T1 and T2 sig- and ophthalmoplegia. In these cases, the metastatic lesion
nals, and homogeneous contrast enhancement.64,72,73 is typically very T2 dark, reflecting its fibrotic nature
Lymphomas most commonly involve the superolateral as- (Fig. 10). Metastases from carcinoid (Fig. 11), renal cell carci-
pect of the orbit,72 and bilateral disease is common.1 When noma and melanoma tend to be circumscribed. All orbital
multiple sites of disease are present, the intraconal space is metastases show some degree of MR enhancement.77 Metas-
most affected (85%). Lymphoma can involve virtually any part tases may involve any structure in the orbit, including the
of the orbit, including the lacrimal gland, extraocular mus- intraconal or extraconal space, globe, extraocular muscles
cles, lacrimal sac, periorbital fat, and retrobulbar fat (Fig. 9). and bone (Fig. 12).
Lymphoma can be very difficult to distinguish from orbital
inflammatory disease (‘‘pseudotumor’’). However, diffusion- Differential diagnosis
weighted imaging (DWI) is an MRI technique that has recently Lymphoma, infection, dysthyroid orbitopathy, orbital
been proven effective in distinguishing these entities. Lym- inflammatory disease, carotid cavernous fistula, and cavern-
phoma typically shows brighter DWI signal and lower appar- ous sinus thrombosis.
ent diffusion coefficient (ADC) than normal orbital structures,
due to its hypercellular nature. Inflammatory lesions, on the Key imaging findings
other hand, tend to show intermediate DWI and ADC signals, Imaging findings vary widely. Globe retraction is highly
similar to normal lacrimal gland.27 Very low T2 signal (darker suspicious for scirrhous breast cancer metastasis.
than normal white matter) should also suggest an inflamma-
tory lesion.5 DWI is also helpful for identifying lacrimal gland Rhabdomyosarcoma
lymphoma.
Clinicopathologic features
Differential diagnosis This is the most common soft tissue malignancy of child-
Orbital inflammatory syndrome, optic nerve sheath menin- hood and most common primary orbital malignancy.78,79
gioma, and orbital metastasis. There is a bimodal distribution of affected ages, commonly
occurring between 2 and 5 years80 but is reported in all age
groups.81 The embryonal subtype occurs in childhood (mean
Key imaging features (lymphoma) age 8 years; male to female ratio 5:3); the pleomorphic sub-
Homogeneous intermediate T2 signal, lobulated margins type occurs in adults; the alveolar subtype occurs in young
with molding around normal structures, homogeneous children and adults and is associated with a poor prognosis.79
enhancement, brighter DWI signal and lower ADC than sur- Primary rhabdomyosarcoma can occur from the conjunc-
rounding normal orbital tissues. tiva, iris, ciliary body, or extension of primary orbital rhabdo-
myosarcoma.82 Secondary orbital rhabdomyosarcoma occurs
Metastatic tumors of the orbit

Clinicopathologic features
In adults orbital metastases usually arise from carcinoma-
tous origin, in children sarcomatous or neural embryonal tu-
mors are more common.74 The spread of tumor to the orbit
and ocular adnexa is usually due to hematogenous spread
of primary cancer, and is associated with a poor prognosis.
Primary metastatic orbital tumors are estimated to account
for 1–13% of all orbital tumors, and in cancer patients are
prevalent from 2% to 4.7%.75 Improved cancer therapy, im-
Figure 12. Colonic adenocarcinoma metastasis. Axial T1 with contrast
proved diagnostic imaging and increased awareness in med- shows a necrotic mass centered in the right sphenoid triangle (), with
ical literature have allowed for better diagnosis of these enhancing tumor and surrounding inflammation along the margins
tumors. extending into the middle cranial fossa and extraconal orbit.
380 S.N. Khan, A.R. Sepahdari

these entities, through demonstration of restricted diffusion

of pus in an abscess cavity,89 as opposed to elevated diffu-
sion in the necrotic portion of a tumor.90

Differential diagnosis
Leukemic metastatic deposits; Langerhans cell histiocyto-
sis; aggressive fibromatosis; ruptured dermoid cyst; subperi-
osteal hematoma; plexiform neurofibromatosis, capillary
hemangioma.

Key imaging features

CT: variable definition, bony destruction; MR: bright T2
signal.

Figure 13. A 26 year-old woman with rhabdomyosarcoma. CT shows an

aggressive mass that deforms the globe and grossly destroys bone Optic nerve glioma
(arrow), invading the ethmoid sinus through the lamina papyracea.
Clinicopathologic features
Optic nerve glioma comprises 1.5–3.0% of orbital tumors,
from direct extension to orbits from the paranasal sinus, 0.6–7.0% of all intracranial tumors, 1.7–7.0% of gliomas, and
pterygopalatine fossa, infratemporal fossa or nasopharynx. 2–5% of gliomas in the pediatric age group.91,92 The peak
Orbital metastases can also occur from other head and neck incidence is in 2–8 year old but can occur at any age and
rhabdomyosarcomas and carries a poor prognosis. The orbi- has been reported at up to 79 years of age.93,94 Females
tal location varies: extraconal (37%), intraconal (17%), both are more affected than males. There is a high association
(47%). The upper outer quadrant is involved in 67%. Present- (12–37%) with type I neurofibromatosis type 195, which fre-
ing complaints include: unilateral proptosis (80–100%), globe quently shows bilateral lesions. Notably, optic gliomas asso-
displacement (80%), ptosis (30–50%), conjunctival and eyelid ciated with NF-1 typically have more indolent behavior than
swelling (60%), palpable mass (25%), and pain (10%).83,84 isolated optic nerve gliomas. Symptoms include: proptosis,
decreased visual acuity,96 nystagmus, strabismus, central/
Imaging findings paracentral field defects, dyschromatopsia, and afferent
CT shows moderately well defined to ill-defined margins, pupillary defect.92 Disk edema and atrophy may be evident
irregular shape, and mild-moderate contrast enhancement. by 8 weeks. The tumors are slow growing with periods of
Adjacent bony destruction occurs in 40% (Fig. 13). Globe dis- growth and dormancy,97,98 some may regress com-
tortion85 and extension to the paranasal sinuses82,86 may also pletely.99,100 In an analysis of affected orbital locations, 48%
be seen. Regional lymph nodes are involved later in the dis- involved the orbital optic nerve, 24% the orbital and intracra-
ease course. Calcification is rare in untreated tumors. nial optic nerve, 10% intracranial optic nerve and chiasm, and
MR typically shows bright T2 signal, distinguishing rhab- just chiasm in up to 5%.101 Surgery is typically reserved for
domyosarcoma from other tumors such as chloroma (granu- cases where there is significant mass effect.
locytic sarcoma), lymphoma and metastatic neuroblastoma
(Fig. 14). MR may also better delineate the true extent of dis- Imaging findings
ease if surgical resection is planned.87 On occasion, a pyo- MR is optimal for imaging optic nerve glioma.102 The clas-
genic abscess may have a subacute presentation that sic finding of optic nerve glioma is sharply circumscribed fusi-
mimics a necrotic rhabdomyosarcoma clinically and by imag- form thickening and tortuosity of the optic nerve. Optic
ing.88 In such cases, MRI with DWI is critical in distinguishing gliomas are typically T2 hyperintense, and usually show some

Figure 14. A 3 year-old boy with embryonal rhabdomyosarcoma. A. Axial T2 shows a well-defined, hyperintense mass involving the retrobulbar and
extraconal soft tissues, without destruction of the bone or distortion of adjacent structures. B. Axial fat-suppressed T1 with contrast shows intense,
homogeneous enhancement. This mass has certain features that are common to capillary hemangioma. However, continued growth of the mass after the
first year of life, absence of flow voids, and absence of discrete lobules with T2 hypointense septations, are features that suggest an alternative diagnosis.
Orbital masses: CT and MRI of common vascular lesions, benign 381

Figure 15. Optic glioma. A. Axial fat-suppressed T1 with contrast shows a solidly enhancing dumbbell-shaped mass extending through the right optic
canal (arrow). B. Coronal fat-suppressed T1 with contrast shows diffuse enhancement of the substance of the right optic nerve (arrow), a finding that
confirms optic glioma rather than optic nerve sheath meningioma. Meningioma forms an enhancing mass that surrounds and constricts the optic nerve.

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