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

– Intraconal lesion– Extraocular muscle enlargement– Optic Nerve/Sheath complex lesion– Ocular mass

Conjunctival

* Most frequent

Often is a Diffuse process

May appear as a Trans-spatial lesion

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

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

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)

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)

Findings

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...

6. Diffuse and ill-defined lesion- Nearly half of

lymphoproliferativelesions are diffuse and

ill defined at imaging

Findings - Lymphoma

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

Findings7. 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

Bibliographic sources• Shields et al 2004. Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with

orbital tumors and simulating lesions: The 2002 Montgomery Lecture, part 1. ophthalmology 2004;111:997–1008.

• K. Haradome, H. Haradome, Y. Usui, S. Ueda, T.C. Kwee, K. Saito, K. Tokuuye, J. Matsubayashi, T. Nagao, and H. Goto. Orbital Lymphoproliferative Disorders (OLPDs): Value of MR Imaging for Differentiating Orbital Lymphoma from Benign OPLDs. AJNR Am J Neuroradiol. 2014 Oct;35(10):1976-82.

• Tailor TD, Gupta D, Dalley RW, Keene CD, Anzai Y.Orbital Neoplasms in Adults: Clinical, Radiologic, and Pathologic Review. Radiographics. 2013 Oct;33(6):1739-58.

• Demirci H, Shields CL, Karatza EC, Shields JA. Orbital lymphoproliferative tumors: analysis of clinical features and systemic involvement in 160 cases. Ophthalmology 2008;115(9):1626–1631, 1631.e1–e3.

• Sepahdari AR, Aakalu VK, Setabutr P, Shiehmorteza M, Naheedy JH, Mafee MF. Indeterminate orbital masses: restricted diffusion at MR imaging with echo-planar diffusion-weighted imaging predicts malignancy. Radiology. 2010 Aug;256(2):554-64.

• Politi LS, Forghani R, Godi C, et al. Ocular adnexal lymphoma: diffusion-weighted MR imaging for differential diagnosis and therapeutic monitor- ing. Radiology 2010;256(2):565–574.

• Jung WS, Ahn KJ, Park MR, et al. The radiological spectrum of orbital pathologies that involve the lacrimal gland and the lacrimal fossa. Korean J Radiol 2007;8(4):336–342.