Orbital Lymphoma
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Disease Entity
Lymphoproliferative tumors of the ocular adnexa encompass a wide spectrum of lesions that range from reactive benign hyperplasia to malignant lymphoma . Ocular adnexal lymphoma (OAL) is a localized form of systemic lymphoma affecting the orbit, the lacrimal gland, the lids or the conjunctiva. It comprises 6-8% of orbital tumors, and 10-15% of adnexal lesions. OAL affect both genders, with a slight female predilection.
Disease
Ocular adnexal lymphoma (OAL) is considered primary if it involves the ocular adnexa alone and secondary if it is accompanied by a lymphoma of identical type at another site. OAL is defined as solitary if it involves one or both orbits only, extension if it involves contiguous sites such as the sinuses, and systemic if remote sites are involved. The majority of lesions in this area are non-Hodgkin lymphoma (NHL), 80% of which arises from B-lymphocytes, 14% from T cells and only 6% from natural killer cells. The most common primary OAL is the low-grade malignant extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (ENZL or MALT), but other types can occur: follicular lymphoma (FL), diffuse large B-cell lymphoma (DLBCL), mantle cell lymphoma (MCL) and lymphoplasmatic lymphoma (LPL) . Secondary OALs arise from systemic disease and are represented by intermediate or high-grade follicular lymphomas. The reported frequency of involvement of periocular sites is: conjunctiva, 20% to 33%; orbit, 46% to 74%; and eyelid, 5% to 20%.
Etiology
Tumors arise from germinal center cells (follicular lymphoma), mantel cells (mantle cell lymphoma) or memory B cells (extranodal marginal zone lymphoma) all of which have undergone antigen exposure. The infection/inflammation/mutation model (IMM) of lymphopatogenesis explains why the ocular adnexa is so often affected by lymphoma, occurring as a result of mistakes during normal lymphocyte response to infection or inflammation.
Risk Factors
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General Pathology
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Pathophysiology
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Primary prevention
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Diagnosis
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History
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Physical examination
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Signs
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Symptoms
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Clinical diagnosis
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Diagnostic procedures
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Laboratory test
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Differential diagnosis
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Management
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General treatment
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Medical therapy
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Medical follow up
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Surgery
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Surgical follow up
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Complications
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Prognosis
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Additional Resources
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References
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