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 and/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

Clinical and imaging data cannot be used to make the diagnosis of OAL with certainty. Diagnosis is made based on a combination of histopathologic, immunophenotypic, and molecular genetic studies, therefore, open biopsy should be obtained. If conjunctival or eyelid OAL is identified clinically, MRI or CT of the orbit is indicated for staging. If there is palpable cervical adenopathy, imaging of this region is performed as well. As more than half of patients have systemic lymphoma at the time of orbital diagnosis or will develop the disease, is important to run a complete systemic evaluation at diagnosis and at regular follow ups. The role of positron-emission tomography (PET) scanning in lymphoma staging and more particularly in staging of OAL is not established. PET has greater sensitivity in detecting abnormalities than does CT or MRI, but the significance of minimal lesions is not known.

History

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Physical examination

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Signs

In the conjunctiva: the typical lesion is salmon or flesh-pink color. In the orbit, eyelid or lacrimal gland: when palpable, the masses are firm.

Symptoms

Many lesions are asymptomatic but patients can complaint of exophthalmos, pain or diplopia, as well of conjunctival, eyelid, orbital or lacrimal gland mass.

Clinical diagnosis

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Diagnostic procedures

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Laboratory test

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Differential diagnosis

It includes benign lymphoproliferative lesions, epithelial tumors, inflammatory and infectious lesions, among others.

Management

Radiation: is been the most frequently used modality for treating OAL, because many patients present with localized disease. Electron or photon irradiation can be used depending on the site, extent of disease and the tumor grade or type. A wide variation of doses has been recommended, ranging from lows of 15–20 Gy up to 40 Gy. Typical doses are 28 to 36 Gy for low-grade OAL and 30 to 40 Gy for high-grade disease. Recurrences of low-grade types are often treatable with local modalities.

Chemotherapy: With solitary OAL, systemic chemotherapy is not indicated, except with diffuse large B-cell lymphoma (DLBCL). Chemotherapy for OAL when it is part of stage II or greater disease has included the use of standard regimens for systemic lymphoma.

Immunotherapy - The local use of IFN-a for OAL is not yet established. - Antilymphocyte antibodies represent the newest form of lymphoma treatment. Using antibodies to CD20 (rituximab), destruction of B cells can take place based on the induction of apoptosis, complement-mediated cytolysis, and antibody-dependent cytoxicity.

In microbial-associated MALT lymphomas, antimicrobial treatment can lead to remission. Further studies are needed to verify this infectious association and those with other possible inciting pathogens.

General treatment

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Medical therapy

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Medical follow up

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Surgery

Excision is reserved for localized lesions of the conjunctiva and orbit. Cryotherapy has been used infrequently for OAL, with varying success. Typically, it is used in patients with conjunctival lymphoma to reduce tumor bulging or those unable to receive other treatment modalities.

Surgical follow up

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Complications

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Prognosis

The site of presentation of OAL has been associated with prognosis with 20% of conjunctival, 35% of orbital, and 67% of eyelid OALs developing systemic lymphoma after 4 years. Among the common OAL tumor types, EMZL, FL, and LPL are considered low grade or indolent, whereas DLBCL and MCL are considered high grade. The mortality ranges for EMZL is 0-20%, for FL 20-37%, for MCL 38-100% and for LPL 14-100%. The mean time to relapse was over 5 years, suggesting that longer follow-up than typically recommended is needed.

Additional Resources

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References

Bardenstein DS. Ocular Adnexal Lymphoma: Classification, Clinical Disease, and Molecular Biology. Ophthalmology clinics of North America. 2005; vol:18(1):187-197.

Bardenstein DS. Orbital and adnexal lymphoma. In: Singh AD, Damato BE, Peér J, Murphree AL, Perry JD, ed. Clinical Ophthalmic Oncology. Philadelphia. Saunders-Elsevier. 2007:565-570.

Bernardini FP, Bazzan M. Lymphoproliferative disease of the orbit. Current opinion in ophthalmology. 2007; vol 18(5):398 -401

Rootman J, White VA, Connors JM, Gascoyne RD. Lymphoproliferative, leukemic, and Histiocytic Lesions of the Orbit. In: Rootman J, ed. Diseases of the orbit: a multidisciplinary approach. Philadelphia, 2nd ed. Lippincott Williams & Wilkins; 2003:385-416


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