Transcript Neuroimaging Clinics of North America

Pictorial Review of Orbital
Tumors and Tumor-like Lesions
eEdE-155a
Viet Nguyen, MD
Maria Valencia, MD
Achint Singh, MD
Wilson Altmeyer, MD
Carlos Bazan III, MD
Bundhit Tantiwongkosi, MD
Disclosure Statement
The authors have no financial interest to disclose.
Objectives
1) Review basic anatomy of the orbit
2) Illustrate characteristic imaging findings of orbital
tumor and tumor-like lesions with respect to orbital
compartments
Table of Contents
Introduction / Anatomy
Globe
Intraconal
Extraconal
Conal
Multicompartment
Summary
Click to proceed directly to section of choice.
Introduction
• Orbital tumors and tumor-like lesions have a spectrum
of imaging findings with distinct outcome and treatment
planning
• Compartmental approach of orbital lesions helps guide
diagnostic consideration
• CT and MR play crucial role in diagnosis, treatment
planning, follow up and surveillance
Anatomy
Click to highlight structure.
Extraconal
Lacrimal Gland
Superior Rectus
Inferior Rectus
Medial Rectus
Conal
Lateral Rectus
Superior Oblique
Inferior Oblique
Levator Palpebrae
Superioris
Intraconal
Optic Nerve
Ophthalmic Artery
Superior Ophthalmic Vein
Globe
Superior Orbital Fissure
Optic Canal
Anatomic Considerations
Orbital Foramina
Contents
Superior Orbital
Fissure
Cranial nerve III, IV, V1, VI
Superior ophthalmic vein
Middle meningeal artery, orbital branch
Sympathetic fibers
Optic Canal
Optic nerve
Ophthalmic artery
Sympathetic fibers
Infraorbital
Canal
Adapted from: Som, Peter M., and Hugh D. Curtin.
"Normal Orbital Anatomy." Head and Neck
Imaging. St. Louis, MO: Mosby, 2011. 549-51.
Cranial nerve V2
Infraorbital artery & vein
http://commons.wikimedia.org/wiki/File:Schematic_diagram_of_the_human_ey
e_en.svg
http://commons.wikimedia.org/wiki/File:713_Bones_Forming_Orbit.jpg
Ocular Melanoma
•
•
Enhancing choroidal mass with increased T1W MR signal; most common
primary ocular tumor adult
Enhancement on MR distinguishes tumor from commonly associated retinal
detachment
Sagittal 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).
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.
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.
Intraconal Orbital Hemangioma
•
•
•
Well-defined, ovoid, enhancing mass with patchy enhancement
Most common adult orbital mass, typically intraconal
Hemangiopericytoma can have similar imaging appearance, but is rare
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.
Optic Nerve Sheath Meningioma
•
•
Enhancing mass encasing intraorbital optic nerve with calcifications
Gradual onset of proptosis & decreased vision in middle-aged women
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.
Optic Nerve Glioma
•
•
•
Fusiform optic nerve mass with variable involvement of posterior pathway
Variable enhancement
Bilateral intraorbital lesions are highly associated with NF1
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.
CN III Schwannoma
•
•
Enhancing, tubular soft tissue mass along the course of CN III with smooth
bony remodeling
Can have cystic formation
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.
Lymphoma
•
•
•
Avidly enhancing soft tissue that molds to & encases orbital structures
Prompts whole body staging & surveillance for systemic lymphoma
Important differential consideration for any orbital mass
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.
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.
Orbital Carcinoid Tumor
•
•
Represent 4%-5% of all orbital metastasis
May show elevated urinary 5-HIAA without hepatic metastasis
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.
Extraconal Cavernous Hemangioma
•
•
•
Well-defined, ovoid, enhancing mass with patchy enhancement
Most common adult orbital mass, typically intraconal
Hemangiopericytoma can have similar imaging appearance, but is rare
MR imaging of the orbits illustrates a well-defined, right lateral extraconal mass
with high T2, low T1 signal intensity & characteristic patchy enhancement.
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.
Acute Myelogenous Leukemia
•
•
Homogenous enhancing soft tissue in patients with known systemic disease
Extramedullary hematopoeisis can have similar appearance in same at-risk
patient population
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.
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.
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 imaging
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.
Orbital Subperiosteal Abscess
•
•
•
Lenticular, rim-enhancing fluid collection
along the medial extraconal orbit, with
adjacent ethmoid sinusitis
Orbital cellulitis & proptosis; may be 1st sign
of acute to subacute sinusitis in children
Restricted diffusion on MR increases
diagnostic confidence
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.
Periorbital edema & mild proptosis are also
evident.
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.
Lacrimal Lymphoma
•
•
Pliable mass arising from lacrimal gland.
Can be multiple masses & predominantly MALT type
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.
Lacrimal Gland Pleomorphic Adenoma
•
•
Unilateral, well-marginated, oval lacrimal mass with characteristic scalloped
bony remodeling of lacrimal fossa
Slow growing & absence of pain
Multiplanar MR images reveal an enlarged lacrimal
gland with low T1/low T2 signal intensity.
Lacrimal Adenoid Cystic Carcinoma
•
•
Irregular mass in the lacrimal fossa with bony erosion
Difficult to distinguish from benign lacrimal processes without bone destruction
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.
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.
Dacrocystocele
•
•
•
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 meatus
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.
Dermoid Cyst
•
•
Well-circumscribed, nonenhancing unilocular cystic lesion containing lipid
Can have capsular calcification
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 contrastenhanced T1W fat suppression.
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.
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.
Idiopathic Inflammatory Pseudotumor
•
•
•
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 painful
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.
Lymphangioma
•
•
Ill-defined, lobulated lymphatic & venous malformation spanning multiple
compartments
Characteristic fluid-fluid levels, blood products, & variable irregular
enhancement
MR imaging of the orbits shows an irregular,
lobulated retrobulbar mass with characteristic fluidfluid levels & multi-compartment involvement. Low
T1/heterogenous high T2 signal intensity &
enhancement are seen.
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.
Summary
• It 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 consideration
• Correlation between imaging and physical findings are
paramount
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.
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