Lymphoma subtype is a predictor for disease-specific mortality

This retrospective case series describe the epidemiologic trends, clinical features and prognosis of patients with malignant lacrimal gland non-Hodgkin lymphoma.

Study design

Data was collected from 260 patients at 6 international eye cancer centers between 1980 and 2017. The median age was 58 years and 52% were men. All patients were histologically verified to have lymphoma of the lacrimal gland. Primary endpoints were overall and disease-specific survival.

Outcomes

Most patients (71%) were diagnosed with primary lymphoma of the lacrimal gland. The most frequent subtype was extranodal marginal zone B-cell (EMZL; 68%) followed by follicular (FL; 10%), diffuse large B-cell (DLBCL; 10%) and mantle cell (MCL; 7%). Five-year disease-specific survival was better in low-grade lymphomas, with EMZL being the best (93%) followed by FL (87%). Meanwhile, higher grade lymphomas tended to do worse, with DLBCL having a 5-year disease-specific survival of 53%.

Limitations

As the data dates back to 1980, some patients who did not survive then may survive now with newer therapies. In addition, the percentages of lymphomas at these tertiary care centers may not truly represent what occurs in communities. This is exemplified by the significant differences in frequency of tumor subtypes between the 6 centers.

Clinical significance

This study is consistent with previous ocular adnexal studies which reported that EMZL and FL have the best prognosis while DLBCL and MCL have the worst prognosis. While lower grade lymphomas of the orbit can often be treated with external beam radiotherapy, higher grade lymphomas often necessitate treatment with chemotherapy in combination with rituximab and/or radiation.