Presentation number: eEdE-114
Orbital Lymphoproliferative
Disorders: The great Mimicker.
Purpose
• Present the varying imaging appearances of
orbital lymphoproliferative disorders from the
common to the uncommon and discuss the
patterns of orbital involvement that it may
assume
• Reinforce several CT/MRI findings that may
provide specificity in the proper inclusion of
lymphoproliferative disorders of the orbit in
the radiologists’ differential diagnosis
Approach
• Why:
– Most common primary orbital tumor in adults
• 10% of all orbital masses.
• 55% of malignant orbital tumors.
• Non-Hodgkin B-cell type lymphoma, specifically the
mucosa-associated lymphoid tissue (MALT) subtype, is
the most common primary orbital lymphoma
– Wide spectrum from benign (lymphoid
hyperplasia, IgG4-related ophthalmic disease,
etc.) to malignant lesions
– The Great Mimicker: Challenging diagnosis due
to broad clinical and radiological presentation
Approach
• How:
– CECT with coronal and sagittal reformations
 Common 1st study for orbital symptoms
– MR should be modality of choice for
evaluating location and extent of disease
• Pre- and post-gadolinium enhancement unless
contraindication
 Axial and coronal images: T1, T2, and post
contrast with fat suppression
Approach
• Where:
– Extraconal lesion*
 Lacrimal gland
– Intraconal lesion
– Extraocular muscle enlargement
– Optic Nerve/Sheath complex lesion
– Ocular mass
 Conjunctival
* Most frequent
Approach
• Where:
– Extraconal lesion*
 Lacrimal gland
Often is a Diffuse
process
May appear as a
Trans-spatial lesion
– Intraconal lesion
– Extraocular muscle enlargement
– Optic Nerve/Sheath complex lesion
– Ocular mass
 Conjunctival
* Most frequent
Findings
• Common pattern:
– Older patients, indolent, painless mass with proptosis
– Anterior extraconal region
– Unilateral, smooth, circumscribed mass that molds to
and encases orbital structures
– Isointense to muscle on T1-weighted sequences and
mildly hyperintense on T2-weighted sequences and
lower ADC (DWI) values than other orbital masses
(due to its high cellularity)
– Uniform enhancement
• It can be located anywhere in the orbit and be
found in various forms: Uncommon patterns
Findings
• Common pattern:
– Older patients, indolent, painless mass with proptosis.
– Anterior extraconal region.
– Unilateral, smooth, circumscribed mass that molds to
and encases orbital structures.
– Isointense to muscle in T1-weighted sequences.
Mildly hyperintense in T2-weighted sequences and
lower ADCs (DWI) values than other orbital masses
(due to its high cellularity).*
– Uniform enhancement.
• It can be located anywhere in the orbit and be
found in various forms: Uncommon patterns
Findings - Lymphoma
1. Extraconal lesion
‒ Homogeneous pliable enhancing mass
CECT of typical imaging features of follicular lymphoma in the extraconal
compartment, presenting as an homogenous enhancing infiltrative mass
in the right lateral extraconal orbit that extends to the temporal fossa
Findings
1. Extraconal lesion
– DDX: Infection/hematoma
– DDX: Cavernous venous malformation
– DDX: Dermoid and epidermoid
Frontozygomatic suture is classic location for orbital dermoid/epidermoid,
diffusion-restricted, fat-containing (dermoid) – NO ENHANCEMENT
Findings - Lymphoma
1. Extraconal lesion: Lacrimal Gland
– Involved in nearly 40% of cases.
– Lymphoproliferative disorders constitute up to
50% of nonepithelial lacrimal lesions
– Older patient age, presence of
lymphadenopathy elsewhere, and lack of
osseous remodeling are all characteristics
suggestive of lymphoma
– Lower ADC values may also be indicative of
lymphoma, as opposed to other lacrimal gland
diseases
Findings - Lymphoma
1. Extraconal lesion: Lacrimal Gland
Findings
1. Extraconal lesion: Lacrimal Gland
– DDX: Sarcoidosis
Diffuse enlargement and homogenous enhancement of lacrimal glands +/muscles bilaterally. Granulomatous diseases often have other ocular
manifestations such as uveitis, keratitis, and conjunctivitis)
Findings
1. Extraconal lesion: Lacrimal Gland
– DDX: Epithelial lesions - Pleomorphic
adenoma (Most common benign neoplasm of lacrimal gland.
Attempts should be made to distinguish lacrimal lymphoma from
pleomorphic adenoma, as biopsy of the latter is generally
avoided because of potential risk of tumor seeding)
Imaging: May remodel bone, T2 hyperintense, more heterogeneous with
homogenous enhancement
Findings
1. Extraconal lesion: Lacrimal Gland
– DDX: Dacryoadenitis (Involvement of orbital septum and
soft tissue thickening and enhancement, local periosteal
reaction)
Findings
2. Intraconal lesion
– DDX: Cavernous hemangioma (venous
malformation) (Most common intraconal mass in adults)
Well-defined, ovoid, may remodel bone, T2 hyperintense, patchy
enhancement that fills in over time)
Findings
2. Intraconal lesion
– DDX: Lymphatic malformation
(Hamartomatous
malformation)
Lobulated and multicystic, fluid-fluid levels, minimal rim enhancement
Findings - Lymphoma
3. Extraocular muscle enlargement
– Diffuse, homogenous enhancement
– Hypointense T2 signal (increased
nuclear/cytoplasmic ratio)
Findings
3. Extraocular muscle enlargement
– DDX: Idiopathic orbital inflammatory
pseudotumor (Acute onset, pain, unilateral, tubular
configuration of affected muscle, tendinous insertion
involvement, adjacent fat stranding)
Findings
3. Extraocular muscle enlargement
– DDX: Thyroid orbitopathy
Bilateral, sparing of
tendinous insertions, expanded retrobulbar fat ( star), low
density within EOM bellies in late disease (arrow)
Findings - Lymphoma
4. Optic Nerve/Sheath complex lesion
‒ Pliable tumor that conforms to normal
structures
Findings
4. Optic Nerve/Sheath complex lesion
- DDX: Meningioma
Homogenous enhancing mass surrounding and distinct from optic nerve
(normal appearance of nerve and surrounding CSF, arrow). Calcifications (30-50%),
"Tram-track" appearance, perioptic cyst behind optic nerve head
Findings
4. Optic Nerve/Sheath complex lesion
– DDX: Glioma
Children, NF1, kinking/buckling common (arrow),
may involve nerve, chiasm, tract, or central pathways, variable
enhancement
Findings - Lymphoma
5. Ocular mass: Conjunctival
– Conjunctiva-associated lymphoid tissue (CALT)
–Usually with reddish decoloration of the conjunctiva (“salmon
patch”), due to its vascularization. Indolent course
Conjunctival lymphoma situated in the lower-medial quadrant of the right eye
–DDX: Benign ocular surface tumors, malignant tumors
(squamous cell carcinoma and amelanotic melanoma),
epi/scleritis, ectopic lacrimal gland, foreign body...
Findings - Lymphoma
6. Diffuse and ill-defined lesion
- Nearly half of lymphoproliferative
lesions are diffuse and ill defined at
imaging
Example: IgG4- related disease
Findings
6. Diffuse and ill-defined lesion
– DDX: Pseudotumor
Pain , 75% retrobulbar, infiltration or
thickening of EOMs, isointense to orbital fat in T2-WI
Diffuse involvement and enhancement of involved structures
Findings
6. Diffuse and ill-defined lesion
– DDX: Cellulitis
Pain, peri/intraorbital soft tissue infiltration
with mass effect and enhancement (arrow), sinus as most
frequent source of infection, peds>adults, +/- dehiscence of
lamina papyracea
Findings - Lymphoma
7. Trans-spatial mass
– Infiltrative appearance and bone destruction
associated with unfavorable histology of
lymphoproliferative condition
Trans-spatial multiple myeloma
Findings - Lymphoma
7. Trans-spatial mass
Post-lung transplant EBV+ diffuse
large B-cell lymphoma
Findings
7. Trans-spatial mass
– DDX: Orbital metastases
Not rare! Breast most
common, melanoma often metastasizes to orbit. Carcinoid
tumor may involve EOMs
Prostate cancer metastasis to left orbit
Findings
7. Trans-spatial mass
– DDX: Rhabdomyosarcoma
Rare in adults, variable
contrast enhancement, +/- bone remodelation/destruction.
Conclusion
• Orbital lymphoproliferative disease can
assume many appearances and be
everywhere in the orbit
• Makes almost all differential diagnosis lists
of orbital disease
• Some imaging findings can be helpful in
the differential diagnosis
– Uniformity of MR appearance, hypointense T2,
restricted diffusion
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