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  • Multicenter Clinicopathologic Review of Lacrimal Gland Lymphoma

    By Lynda Seminara
    Selected By: Richard K. Parrish II, MD
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    Journal Highlights

    American Journal of Ophthalmology, November 2020

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    The most common malignant tumor of the lacrimal gland is non-Hodgkin lymphoma (NHL), but little is known about the clinicopathology of lacrimal gland lymphomas. Vest et al. reviewed features of subtype-specific lacrimal gland lymphomas and their prognoses. They found that the most common subtypes of NHL resembled those in the ocular adnexa (OA). Although they observed a strong relationship between lymphoma subtype and disease-specific survival (DSS), prognosis was relatively good overall.

    For this study, the authors analyzed biopsy and clinical data for patients with histologically verified primary or secondary lymphoma of the lacrimal gland. The main outcomes were overall survival (OS) and DSS.

    Among the 260 patients identified at six international eye cancer centers, the NHL pathology was B-cell in 258 (99%) and T-cell in two (1%). Extranodal marginal zone B-cell lymphoma (EMZL) was the most common subtype (n = 177; 68%), followed by follicular lymphoma (FL; n = 26; 10%), diffuse large B-cell lymphoma (DLBCL; n = 25; 10%), and mantle cell lympho­ma (MCL; n = 17; 7%).

    With regard to location, the percentage of patients with EMZL was higher in India, and the preponderance of MCL was greater in Denmark. Low-grade lymphomas (including EMZL and FL) were often managed with external beam radiotherapy (EBRT), whereas high-grade lymphomas (such as DLBCL and MCL) generally received chemotherapy plus rituximab and/or EBRT.

    The five-year OS and DSS rates were 73.8% and 87.5%, respectively, which support the findings of previous studies. Both rates varied greatly between sub­types (p < .001). EMZL had the highest five-year DSS rate (93.4%), and DLBCL had the lowest (52.6%). The difference in subtype-specific DDS between the treatment centers was not significant.

    The favorable survival rate for patients with EMZL supports earlier studies of the OA. However, the lym­phoma subtype distribution in the cur­rent study resembles that of the orbit and OA, rather than that of the salivary gland, as assumed previously.

    The original article can be found here.