The eyelids, as well as most other ocular structures, can be involved with benign and malignant lymphoid tumors (1,2,3,4,5,6,7,8,9,10,11,12,13,14,15). The classification of extranodal lymphoid tumors in the ocular region is complex and confusing. The Revised European American Lymphoma classification is currently the most popular (4,5,11). Ophthalmic pathologists have traditionally classified lymphoid tumors into benign (lymphoid hyperplasia), intermediate, and malignant forms. Clinical differentiation of these forms is not usually possible, and biopsy and histopathologic assessment are necessary to make a specific diagnosis. Lymphoma can also be divided into Hodgkin’s and non-Hodgkin’s types and B-cell or T-cell types (cutaneous lymphoma; mycosis fungoides) depending on the type of lymphocyte that comprises most of the lesion.

Sezary syndrome is a variant of T-cell lymphoma that consists of a triad of erythroderma, leukemia, and large peripheral lymph nodes. It generally affects elderly men and is characterized by a more fulminating course. It often shows atypical mononuclear cells in the blood (Sezary cells). The skin lesions are identical clinically and histopathologically to typical mycosis fungoides and they can rarely involve the eyelids.

Eyelid lymphoid tumors tend to parallel those of the orbit in their degree of malignancy and their clinical behavior. Orbital lymphoid tumors are more common and are discussed in more detail in the Atlas of Orbital Tumors. Lymphoma can be confined to the eyelid, but is more often associated with systemic lymphoma. It is generally a disease of elderly patients, but it can occur in younger individuals, particularly those with AIDS. In contrast with conjunctival lymphoma, eyelid lymphoma tends to have a greater association with systemic lymphoma, particularly if it is bilateral (1,7).

B-cell lymphoma of the eyelid generally occurs as a smooth, rather firm subcutaneous mass. Ulceration of eyelid B-cell lymphoma is rare (10). Although it may be confined to the eyelid, it is more often continuous with anterior orbital disease.

In contrast, the less common T-cell lymphoma has a tendency to affect the skin more superficially and to exhibit papules, plaques, or ulceration (mycosis fungoides). The lesions can be solitary or multiple. It can often masquerade as other conditions before the diagnosis is realized (6). A severe form of this condition, called adult T-cell leukemia/lymphoma, has recently been recognized to exhibit simultaneous involvement of the eyelid, orbit, and uveal tract (9).

Lymphoma of the eyelids is identical histopathologically to lymphoma in other parts of the body. It is composed of abnormal lymphocytes that range from low grade to intermediate to clearly malignant. Immunohistochemical techniques and flow cytometry are necessary to categorize the specific type of lymphoma (1,8). Most eyelid lymphomas are of B-cell lineage.

When either B-cell or T-cell lymphoma are suspected in the eyelid, the affected patient should usually undergo a biopsy of the lesion and study of the cells with immunohistochemistry and flow cytometry to accurately categorize the lesion. It is important that the clinician communicate with the pathologist ahead of time to ensure that the tissue is handled properly. It is often wise to send the fresh material immediately to thepathologist rather than placing it in formaldehyde. If systemic evaluation reveals more widespread lymphoma, then chemotherapy is generally given to control the systemic disease and the eyelid lesion can be followed for regression. If the disease seems to be confined to the eyelid area, then radiotherapy can be considered. The dose of irradiation can vary from 2000 cGy for benign lymphoma to 4000 cGy for malignant lymphoma (1,2,3). The prognosis varies widely with the severity of the disease.