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Hindi Editorial, Int J Ophthalmic Pathol Vol: 13 Issue: 2
Orbital Lymphoma: A Comprehensive Overview
Mohamed Salah*
Department of Optometry, Misr International University, Egypt
- *Corresponding Author:
- Mohamed Salah
Department of Optometry, Misr International University, Egypt
E-mail: salah846@yahoo.com
Received: 01-Apr-2025, Manuscript No. iopj-25-169462; Editor assigned: 4-Apr-2025, Pre-QC No. iopj-25-169462 (PQ); Reviewed: 19-Apr-2025, iopj-25-169462; Revised: 26-Apr-2025, Manuscript No. iopj-25-169462 (R); Published: 30-Apr-2025, DOI: 10.4172/2324-8599.1000056
Citation: Mohamed S (2025) Orbital Lymphoma: A Comprehensive Overview. Int J Ophthalmic Pathol 13: 056
Introduction
Orbital lymphoma is a type of cancer that originates in the lymphoid tissue of the orbit—the bony cavity surrounding the eye. It accounts for approximately 10–15% of all orbital tumors and represents the most common primary orbital malignancy in adults. Most orbital lymphomas are classified as non-Hodgkin B-cell lymphomas, with extranodal marginal zone B-cell lymphoma (EMZL) of the mucosa-associated lymphoid tissue (MALT) being the most prevalent subtype.
Although orbital lymphoma is generally slow-growing and localized, it can significantly affect ocular function and appearance. Early diagnosis and appropriate management are crucial for preserving vision, ensuring local disease control, and preventing systemic spread [1,2].
Orbital lymphoma is a rare but significant malignancy involving the lymphoid tissue within the orbit, which is the bony cavity that contains the eye and its associated structures. It accounts for approximately 10–15% of all orbital tumors and is the most common form of primary orbital malignancy in adults. While it can affect individuals of all ages, it is most frequently diagnosed in middle-aged and older adults, with a slight predominance in females [3,4].
Most orbital lymphomas are non-Hodgkin B-cell lymphomas, with extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) being the most prevalent subtype. These tumors originate from lymphoid tissues that are naturally present or develop in response to chronic inflammation in the orbit, lacrimal gland, conjunctiva, or eyelid [5,6].
Orbital lymphoma often presents as a painless, slowly enlarging mass in the orbit, leading to symptoms such as proptosis (bulging of the eye), diplopia (double vision), eyelid swelling, or decreased ocular motility. Because of its subtle presentation and indolent nature, the disease can be mistaken for inflammatory or benign orbital disorders, which can delay diagnosis [7,8].
Imaging with MRI or CT scans typically reveals a well-defined, homogenous mass that conforms to the contours of the orbit without bone destruction. A tissue biopsy is essential for definitive diagnosis and classification. Histopathology, combined with immunohistochemistry and molecular studies, helps determine the lymphoma subtype and guides treatment planning [9].
Management strategies depend on the histological subtype and extent of disease. For localized, low-grade tumors (such as MALT lymphoma), radiation therapy is highly effective. High-grade or systemic lymphomas may require chemotherapy, immunotherapy, or a combination of both.
Although orbital lymphoma is generally associated with a favorable prognosis, especially in low-grade forms, long-term follow-up is necessary to monitor for recurrence or systemic progression. Early diagnosis and appropriate treatment are key to preserving vision and achieving optimal patient outcomes.
Discussion
Orbital lymphoma represents a unique subset of extranodal non-Hodgkin lymphomas, arising within the orbit or adjacent ocular adnexa such as the lacrimal gland, conjunctiva, or eyelids. Though relatively rare, it is the most common malignant orbital tumor in adults. Its clinical importance lies not only in its frequency but also in the challenges it poses in diagnosis and management [10].
One of the defining features of orbital lymphoma is its indolent, painless growth, which often results in delayed recognition. Patients typically present with proptosis, eyelid swelling, diplopia, or ocular discomfort, yet without the redness, warmth, or pain that characterizes inflammatory conditions. This subtlety often leads to confusion with orbital pseudotumor, thyroid eye disease, or benign lesions, underscoring the need for a high index of suspicion, especially in older adults.
Imaging studies, particularly MRI and CT, are helpful in identifying the presence of a well-defined, homogenous mass, but these modalities cannot distinguish lymphoma from other orbital lesions with certainty. Therefore, tissue biopsy remains the gold standard for diagnosis. Histological examination, along with immunohistochemistry and molecular markers, allows for classification into subtypes, most commonly extranodal marginal zone B-cell lymphoma (MALT), which tends to have a favorable prognosis.
The treatment approach is primarily dictated by the histologic subtype and stage of the disease. Radiotherapy is highly effective for localized, low-grade lymphomas, offering excellent local control with minimal side effects. For more aggressive types, such as diffuse large B-cell lymphoma (DLBCL), or in cases with systemic involvement, chemotherapy (e.g., R-CHOP) and immunotherapy with rituximab are standard. Some cases of MALT lymphoma, especially those linked to Chlamydia psittaci, may even respond to antibiotic therapy, though this remains geographically variable.
In conclusion, orbital lymphoma, while often slow-growing, requires timely and accurate diagnosis to prevent local tissue damage and preserve vision. With appropriate therapy, especially for low-grade variants, outcomes are generally favorable. Long-term follow-up is essential, as recurrence or transformation into more aggressive disease can occur even after initial successful treatment.
Diagnosis
Diagnosis involves a combination of clinical examination, imaging, and histopathological confirmation through biopsy.
Imaging
Orbital MRI is the imaging modality of choice, typically showing a well-defined, homogeneous, enhancing mass that molds to the globe and surrounding structures.
CT scan may be used to assess bone involvement or to guide biopsy.
Biopsy
Incisional or excisional biopsy is essential for definitive diagnosis.
Histopathological analysis, immunohistochemistry, and flow cytometry help determine the lymphoma subtype.
Staging Workup
Once diagnosed, systemic evaluation is performed to determine whether the disease is localized or part of a systemic lymphoma. This includes:
Whole-body PET-CT
Bone marrow biopsy
Blood tests including LDH, CBC, and liver/kidney function
Treatment
Treatment depends on the histological subtype, tumor location, and extent of disease (localized vs. systemic).
Radiation Therapy
Mainstay treatment for localized, low-grade orbital lymphomas (e.g., MALT lymphoma).
Radiation doses typically range between 24–30 Gy.
High local control rates (>90%) with minimal side effects.
Potential complications include dry eye, cataract formation, and retinopathy, especially with higher doses.
Chemotherapy
Used for high-grade lymphomas or systemic disease.
Regimens like R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) are standard for DLBCL.
May also be combined with radiation in select cases.
Immunotherapy
Rituximab, a monoclonal antibody against CD20, is effective in treating B-cell lymphomas and is often used as monotherapy or in combination.
Antibiotic Therapy
In some regions (notably Europe), antibiotic treatment targeting Chlamydia psittaci (e.g., doxycycline) has shown response in MALT lymphoma patients, though results are inconsistent and region-specific.
Conclusion
Orbital lymphoma is an important differential diagnosis in patients presenting with painless orbital masses or progressive proptosis. While most cases are low-grade and localized, high-grade subtypes can pose significant therapeutic challenges. Timely diagnosis through biopsy and imaging is essential for appropriate classification and treatment. Radiotherapy remains the cornerstone for localized disease, while systemic therapy is reserved for advanced or aggressive forms. With early intervention, most patients with orbital lymphoma can expect excellent outcomes and preserved vision.
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