Lymphoma of the Lacrimal Gland — An International Multicenter Retrospective Study

• Published in: American journal of ophthalmology 2020-11, Vol.219, p.107-120
• Main Authors: Vest, Stine Dahl, Mikkelsen, Lauge Hjorth, Holm, Frederik, Rasmussen, Peter Kristian, Hindso, Tine Gadegaard, Knudsen, Marina K.H., Coupland, Sarah E., Esmaeli, Bita, Finger, Paul T., Graue, Gerardo F., Grossniklaus, Hans E., Honavar, Santosh G., Mulay, Kaustubh, Sjö, Lene D., Sniegowski, Matthew C., Vemuganti, Geeta K., Thuro, Bradley A., Heegaard, Steffen
• Format: Article
• Language: English
• Subjects: Adult; Aged; Aged, 80 and over; Antineoplastic Agents, Immunological - therapeutic use; Biopsy; Bone marrow; Cancer; Classification; Combined Modality Therapy; Disease-Free Survival; Eye diseases; Eye Neoplasms - epidemiology; Eye Neoplasms - pathology; Eye Neoplasms - therapy; Female; Humans; Internationality; Lacrimal Apparatus Diseases - epidemiology; Lacrimal Apparatus Diseases - pathology; Lacrimal Apparatus Diseases - therapy; Lymphatic system; Lymphoma; Lymphoma, B-Cell - epidemiology; Lymphoma, B-Cell - pathology; Lymphoma, B-Cell - therapy; Lymphoma, T-Cell - epidemiology; Lymphoma, T-Cell - pathology; Lymphoma, T-Cell - therapy; Male; Medical prognosis; Middle Aged; Monoclonal antibodies; Neoplasm Staging; Prognosis; Radiotherapy, Conformal; Retrospective Studies; Rituximab - therapeutic use; Survival Rate; Targeted cancer therapy; Tumors
• ISSN: 0002-9394; 1879-1891
• DOI: 10.1016/j.ajo.2020.06.015

To characterize the clinical features of subtype-specific lacrimal gland lymphoma and their effect on patient survival. Multicenter retrospective interventional case series. Patient data were collected from 6 international eye cancer centers from January 1, 1980, through December 31, 2017. All patients with histologically verified primary or secondary lymphoma of the lacrimal gland were included. Primary endpoints were overall survival (OS) and disease-specific survival (DSS). A total of 260 patients with lacrimal gland lymphoma were identified. The median age was 58 years and 52% of patients were men. Non-Hodgkin B-cell lymphomas constituted 99% (n = 258) and T-cell lymphomas constituted 1% (n = 2). The most frequent lymphoma subtypes were extranodal marginal zone B-cell lymphoma (EMZL) (n = 177, 68%), follicular lymphoma (FL) (n = 26, 10%), diffuse large B-cell lymphoma (DLBCL) (n = 25, 10%), and mantle cell lymphoma (MCL) (n = 17, 7%). Low-grade lymphomas (EMZL and FL) were most commonly treated with external beam radiotherapy (EBRT), whereas high-grade lymphomas (DLBCL and MCL) were treated with chemotherapy in combination with rituximab and/or EBRT. The prognosis was relatively good with a 5-year OS and DSS of 73.8% and 87.5%, respectively. Lymphoma subtype was a statistically significant predictor for DSS, with EMZL (5-year DSS: 93.4%) having the best prognosis and DLBCL (5-year DSS: 52.6%) having the poorest. This is the largest reported collection of data of subtype-specific lacrimal gland lymphoma. The subtype distribution of lacrimal gland lymphoma resembles that of the ocular adnexa. Prognosis is good and the histologic subtype is a significant predictor for disease-specific survival. •The distribution of lymphoma subtypes resembles that of the ocular adnexa.•The most frequent lymphoma subtypes are extranodal marginal zone B-cell lymphoma, follicular lymphoma, diffuse large B-cell lymphoma, and mantle cell lymphoma.•Lacrimal gland lymphoma has an overall 5-year disease-specific survival of 87.5%.•Lymphoma subtype is a significant predictor of disease-specific survival.