Pictorial Review of Orbital Tumors and Tumor-like Lesions - PowerPoint Presentation

Slide1

Pictorial Review of Orbital Tumors and Tumor-like Lesions

Viet Nguyen, MDMaria Valencia, MDAchint Singh, MDWilson Altmeyer, MDCarlos Bazan III, MDBundhit Tantiwongkosi, MD

eEdE-155aSlide2

Disclosure Statement

The authors have no financial interest to disclose.Slide3

ObjectivesReview basic anatomy of the orbit

Illustrate characteristic imaging findings of orbital tumor and tumor-like lesions with respect to orbital compartmentsSlide4

Table of Contents

Introduction / AnatomyClick to proceed directly to section of choice.

Globe

Intraconal

Extraconal

Conal

Summary

MulticompartmentSlide5

IntroductionOrbital tumors and tumor-like lesions have a spectrum of imaging findings with

distinct outcome and treatment planningCompartmental approach of orbital lesions helps guide diagnostic considerationCT and MR play crucial role in diagnosis, treatment planning, follow up and surveillanceSlide6

Anatomy

Superior Oblique

Inferior Oblique

Superior Rectus

Inferior Rectus

Medial Rectus

Lateral Rectus

Optic Nerve

Ophthalmic Artery

Superior Ophthalmic Vein

Lacrimal Gland

Superior Orbital Fissure

Optic Canal

Levator

Palpebrae

Superioris

Conal

Intraconal

Globe

Extraconal

Click to highlight structure.Slide7

Anatomic Considerations

Orbital ForaminaContentsSuperior Orbital Fissure

Cranial nerve III, IV, V1, VISuperior ophthalmic veinMiddle meningeal artery, orbital branchSympathetic fibers

Optic Canal

Optic nerve

Ophthalmic artery

Sympathetic fibers

Infraorbital

Canal

Cranial

nerve V2

Infraorbital

artery & vein

Adapted from: Som

, Peter M., and Hugh D. Curtin. "Normal Orbital Anatomy." Head and Neck Imaging. St. Louis, MO: Mosby, 2011. 549-51.

http://commons.wikimedia.org/wiki/File:713_Bones_Forming_Orbit.jpg

http://commons.wikimedia.org/wiki/File:Schematic_diagram_of_the_human_eye_en.svgSlide8

Ocular Melanoma

S

agittal oblique MR images show

an avidly enhancing

,

high T1

/

low T2

signal

mass

in the posterior globe

extending to the adjacent sclera

& causing

partial retinal detachment (no enhancement)

.

E

nhancing

choroidal

mass with increased T1W MR signal; most common primary ocular tumor adult

Enhancement on MR distinguishes tumor from commonly associated retinal detachmentSlide9

Choroidal Melanoma

Coronal post contrast T1W MR image reveals

a focal linear area of enhancement

at the superior nasal aspect of the right globe, within the choroid layer. This was proven to be

choroidal

melanoma on histology. The tumor can be subtle in this case; therefore, correlation between imaging &

fundoscopic

findings are crucial.Slide10

Nodular Scleritis

Coronal & sagittal MR images of the orbits show an anterior right scleral lesion with

intermediate T1

/

low T2

signal intensity &

contrast enhancement

. This was a case of nodular

scleritis

mimicking a neoplasm in a patient with juvenile rheumatoid

arhritis

.

Thickening & enhancement

of the left

globe also represent

scleritis

. Slide11

Intraconal Orbital Hemangioma

MR imaging of the orbits reveals a well-defined, round

intraconal

mass displacing the optic nerve. The lesion shows

low T1,

high T2

signal intensity &

characteristic patchy enhancement

.

Well-defined, ovoid, enhancing mass with

patchy

enhancement

Most common adult orbital mass, typically

intraconal

Hemangiopericytoma

can have similar imaging appearance, but is rareSlide12

Optic Nerve Sheath Meningioma

Axial NECT image reveals a

calcified optic nerve sheath

menignioma

surrounding the

intraorbital

optic nerve resulting in

proptosis

.

Avid homogenous enhancement

is seen on axial MR T1W image. The

optic nerve

is normal in size but can have abnormal MR signal.

Enhancing mass encasing intraorbital

optic nerve with calcifications

Gradual onset of

proptosis

& decreased vision in middle-aged womenSlide13

Optic Nerve Glioma

Axial MR images of a patient with neurofibromatosis 1 show

T1

isointense

fusiform enlargement & kinking of the optic nerves. Note the

avid enhancement

of the right &

minimal enhancement

of the left optic nerve

gliomas

.

Fusiform optic nerve mass with variable involvement of posterior pathway

Variable enhancement

Bilateral

intraorbital

lesions are highly associated with NF1Slide14

CN III Schwannoma

Axial & coronal MR imaging shows a tubular,

intraconal

orbital mass with

low T1

/

high T2 signal

&

intense enhancement

in the superior orbital fissure extending posteriorly into the cavernous sinus. Note the

medial displacement of the tortuous CN II

.

Enhancing, tubular soft tissue mass along the course of

CN III with

smooth bony

remodeling

Can

have cystic formationSlide15

Lymphoma

MR imaging of the orbits in a patient with known anaplastic large cell lymphoma of the head, face & neck shows

increased T2 signal

with

enhancement

involving the

intraorbital

left optic nerve & surrounding

intraconal

fat. There is restricted diffusion of the optic nerve on

DWI

&

ADC map

.

Avidly enhancing soft tissue that molds to & encases orbital structures

Prompts whole body staging & surveillance for systemic lymphoma

Important differential consideration for any orbital massSlide16

Leptomeningeal Carcinomatosis

Axial post contrast T1W MR image reveals

symmetric enhancement of optic nerve sheaths &

intraconal

fat.

This was a histologically proven case of

leptomeningeal

carcinomatosis

in a 44

yo

female patient with HIV & metastatic

leiomyosarcoma

.Slide17

Orbital Carcinoid Tumor

Multiplanar

contrast-enhanced MR T1W images show

avidly enhancing

intraconal

mass

displacing the optic nerve medially

. This was a case of orbital metastasis from primary abdominal carcinoid tumor.

Represent 4%-5% of all orbital metastasis

May show elevated urinary 5-HIAA without hepatic metastasisSlide18

Extraconal Cavernous Hemangioma

MR imaging of the orbits illustrates a well-defined, right lateral

extraconal

mass with

high T2

,

low T1

signal intensity &

characteristic patchy enhancement.

Well-defined, ovoid, enhancing mass with patchy enhancement

Most common adult orbital mass, typically

intraconal

Hemangiopericytoma

can have similar imaging appearance, but is rareSlide19

CN V1 Schwannoma

Coronal & axial MR images demonstrate an orbital mass with

heterogeneous

high T2

signal

&

intense enhancement

in the right

superomedial

extraconal

space that displaces the globe &

superior rectus muscle

inferolaterally

. This was proven to be schwannoma in the expected course of CN V1.Slide20

Acute Myelogenous Leukemia

Axial & coronal MR imaging of the orbits in a pediatric patient with AML demonstrates

extraconal

soft tissue masses with

low T1

/

low

T2

signal intensity &

homogenous enhancement

.

Homogenous enhancing soft tissue in patients with known systemic disease

Extramedullary

hematopoeisis

can have similar appearance in same at-risk patient populationSlide21

Orbital Lymphoplasmacytic Lymphoma

Coronal & axial MR images of the orbits reveal a

homogenously,

enhancing

mass

with

low T1

/

high T2

signal intensity in the right superior

extraconal

compartment at the level of the lacrimal gland resulting in inferior displacement of the globe. Focus of

low T2W signal intensity

represents lymphoid tissue.Slide22

Wegener Granulomatosis

Axial & coronal MR imaging of the orbits demonstrates a medial

intraconal

soft tissue mass with

low T1

/

low T2

signal intensity &

avid enhancement

. This was a proven case of

orbital extension

of Wegener

granulomatosis

.

Systemic necrotizing

vasculitis

, with renal & respiratory involvement

Paranasal

sinus & orbital involvement (most common

extrasinonasal

site) with bone destruction, commonly bilateral

Difficult to differentiate from lymphoma or

sarcoidosis

on imagingSlide23

Orbital Subperiosteal Abscess

Axial CECT imaging through the orbits reveals a

subperiosteal

abscess

extending into the medial

extraconal

orbit & causing

displacement of the medial rectus

. Note the

ethmoid

sinusitis

.

P

eriorbital

edema & mild proptosis

are also evident.

Lenticular, rim-enhancing fluid collection along the medial

extraconal

orbit, with adjacent

ethmoid

sinusitis

Orbital cellulitis &

proptosis

; may be 1

st

sign of acute to

subacute sinusitis in childrenRestricted diffusion on MR increases diagnostic confidenceSlide24

Intraorbital Abscess

Axial CECT image of the orbits reveals right

proptosis

& lateral

periorbital

edema with

a rim enhancing fluid collection in the right lateral

extraconal

space

. Axial & coronal MR images demonstrate a

rim enhancing fluid collection

with

high T2W signal intensity

abutting & displacing the right lateral rectus muscle. There is restricted diffusion on

DWI

&

ADC

map

. Slide25

Lacrimal Lymphoma

Axial & coronal MR images reveal an enlarged,

intensely enhancing lacrimal gland mass

with

low T1

/

high T2

signal intensity. This was found to be diffuse large B-cell lymphoma of the lacrimal gland.

Pliable mass arising from lacrimal gland.

Can be multiple masses & predominantly MALT type Slide26

Lacrimal Gland Pleomorphic Adenoma

Multiplanar

MR images reveal an enlarged lacrimal gland with

low T1

/

low T2

signal intensity.

Unilateral, well-marginated, oval lacrimal mass with characteristic scalloped bony remodeling of lacrimal fossa

Slow growing & absence of painSlide27

Lacrimal Adenoid Cystic Carcinoma

Axial & coronal MR images reveal an

avidly enhancing

,

T2

hyperintense

heterogeneous mass with lobulated, well-defined margins arising from the lacrimal gland. Biopsy demonstrated adenoid cystic carcinoma of the lacrimal gland.

Irregular mass in the lacrimal fossa with bony erosion

Difficult to distinguish from benign lacrimal processes without bone destructionSlide28

IgG4 Related Disease

Bilateral chronic dacryoadenitis > submandibular sialadenitis >

parotiditis with variable orbital inflammatory pseudotumor

, myositis,

perineural

disease

Characteristically

hypointense

on T2WI due to high cellularity

&

fibrosis

Axial NECT image of the orbits reveal

enlarged lacrimal glands

. They demonstrate intermediate

T1

/

T2

signal intensity &

avid enhancement

.Slide29

Dacrocystocele

Axial NECT images of a 22-day-old newborn reveal a

cystic lesion

in the medial orbit at the location of the lacrimal sac. Note the

dilated nasolacrimal sac

.

Well-circumscribed cyst arising from the lacrimal sac, typically self-limited

25% bilateral with variable intranasal extension

Bilateral cysts may cause nasal obstruction at the level of inferior meatusSlide30

Dermoid Cyst

Axial imaging of the orbits reveals an ovoid, well-defined, right lateral

extraconal

mass causing medial displacement of the lateral rectus. This mass demonstrates

fatty attenuation (-20 HU)

,

hyperintensity

on T1W

, &

hypointensity

on contrast-enhanced T1W fat suppression

.

Well-circumscribed,

nonenhancing

unilocular

cystic lesion containing lipid

Can have capsular calcification Slide31

Extraconal Dermoid Cyst/Lipoma

Axial & coronal NECT images of the orbits reveal a

well-defined mass with fatty attenuation

along the lateral aspect of the left globe, anterior to the attachment of the lateral rectus muscle. Note the

punctate calcification

at the medial aspect of the mass, abutting the globe. Slide32

Extraocular Muscle Metastasis

Coronal & axial CECT images show an

enlarged, enhancing left lateral rectus muscle

with adjacent

conal

fat stranding. There is mild left

proptosis

. Axial & coronal MR images reveals

isotense

T1

/

hyperintense

T2

, &

avidly enhancing

lateral rectus muscle. The patient was a 31

yo

female with metastatic breast cancer & left orbital cellulitis not responding to antibiotics.Slide33

Idiopathic Inflammatory Pseudotumor

Axial & coronal MR imaging through the orbits shows a poorly-defined

T1

hypointense

/

T2

hyperintense

mass

with

heterogeneous enhancement

encasing the

medial rectus

with involvement of multiple compartments. There is mild

proptosis

& lateral displacement of the globe.

Ill-defined

masslike

enhancing soft tissue involving any compartment of the orbit; 25% bilateral

Diagnosis of exclusion; biopsy for atypical onset, poor response or recurrence

Typically painfulSlide34

Lymphangioma

MR imaging of the orbits shows an irregular, lobulated

retrobulbar

mass with characteristic

fluid-fluid levels

& multi-compartment involvement.

Low T1

/

heterogenous

high T2

signal intensity &

enhancement

are seen.

Ill-defined, lobulated lymphatic & venous malformation spanning multiple compartments

Characteristic fluid-fluid levels, blood products, & variable irregular enhancementSlide35

Metastatic Breast Cancer

Axial & coronal MR images show

T1

hypointense

,

enhancing metastatic breast cancer

filling multiple compartments of the bilateral orbits. Note the prominent

right

enophthalmos

secondary to classic

schirrous

changes. Slide36

SummaryIt is crucial for radiologists to be familiar with the imaging features of orbital masses. Some have characteristic findings, e.g. optic nerve

glioma, optic nerve sheath meningioma, cavernous hemangioma, etc. Compartmental approach to assessing orbital lesions helps guide diagnostic considerationCorrelation between imaging and physical findings are paramountSlide37

References

Ansari, Sameer A., and Mahmood F. Mafee. "Orbital cavernous hemangioma: role of imaging." Neuroimaging clinics of North America 15.1 (2005): 137-158. Chung, Ellen M., et al. "From the Archives of the AFIP Pediatric Orbit Tumors and Tumorlike

Lesions: Osseous Lesions of the Orbit 1." Radiographics 28.4 (2008): 1193-1214. Demirci, Hakan, et al

. "Orbital tumors in the older adult population

.“

Ophthalmology

 109.2 (2002): 243-248

.

Kapur

,

Rashmi

, et al.

"Orbital schwannoma and neurofibroma: role of imaging." Neuroimaging Clinics of North America 15.1 (2005): 159-174.Smoker, Wendy RK, et al.

"Vascular Lesions of the Orbit: More than Meets the Eye 1." Radiographics 28.1 (2008): 185-204.Tailor, Tina D., et al. "Orbital neoplasms in adults: clinical, radiologic, and pathologic review." Radiographics 33.6 (2013): 1739-1758.

Uduma, Felix Uduma, and Kamga Titalom. "Intra-orbital malignant melanoma: role of MR imaging (a case report and literature review)." Global journal of health science 4.1 (2011): p253.Slide38

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