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Orbital lymphoma is a malignant lymphoid neoplasm involving the orbit, most commonly arising from extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma).

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It is the most common orbital malignancy in adults and typically presents as a painless, slowly progressive orbital mass.

Etiopathogenesis


Feature Details
Origin Arises from mature B-cells, especially MALT-type in the orbit
Risk factors – Chronic inflammation (e.g., Chlamydia psittaci infection, Sjögren's syndrome)
– Immunosuppression
– Autoimmune disorders
Systemic association May be primary (limited to orbit) or secondary to systemic lymphoma

Histological subtypes:

Subtype Frequency Notes
Extranodal marginal zone (MALT) lymphoma ~55–70% Indolent course
Follicular lymphoma ~20% Intermediate grade
Diffuse large B-cell lymphoma (DLBCL) ~10% Aggressive
Mantle cell lymphoma Rare Often systemic

Clinical features


Median age of presentation: ~60 years, slight female predominance

Feature Description
Painless orbital mass Most common presentation
Proptosis Unilateral or bilateral, slowly progressive
Eyelid swelling or fullness Without erythema or tenderness
Diplopia Due to mass effect on extraocular muscles
Visual disturbance From compressive optic neuropathy (less common)
No signs of acute inflammation Differentiates from IOI or orbital cellulitis

Radiology


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CT/MRI shows a homogeneous, well-marginated lesion, and definitive diagnosis requires biopsy.

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Common sites:

Imaging features:

Modality Imaging features
CT Soft tissue mass: Well-defined, homogeneous, mildly hyperdense relative to orbital fat
Molding to orbital contours: No bone destruction; conforms to globe, extraocular muscles
No calcification or necrosis (unlike metastasis or infection)
MR T1: Iso- to hypointense compared to muscle
T2: Iso- to mildly hyperintense; less hyperintense than most other orbital lesions
Post-contrast: Homogeneous enhancement, no necrosis or cystic areas
DWI: Restricted diffusion is often present (useful for biopsy planning)