Lymphoma of the Lacrimal Gland — An International Multicenter Retrospective Study

Abstract

To characterize the clinical features of subtype-specific lacrimal gland lymphoma and their effect on patient survival.DESIGN: Multicenter retrospective interventional case series.METHODS: 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).RESULTS: 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 Bcell 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 highgrade 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.CONCLUSIONS: 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 Biology of Ocular Adnexal Marginal Zone Lymphomas

“…For the lacrimal gland, the annual incidence is about 0.7/million individuals; 99% of lymphomas in this location are B-cell lymphomas, with 68% of them EMZLs. The lacrimal sac is reported to be involved in 2% of patients [ 16 , 17 ]. Secondary involvement of the ocular adnexa with a systemic lymphoma is described in only 10–32% of all OAL cases [ 18 ].…”

Ocular adnexal lymphoma: Subtype‐specific clinical and genetic features

“…Classification of OAL is, thereby, complex and continuously evolving to best incorporate these features. OALs belong almost exclusively to the non‐Hodgkin group of lymphomas, are predominantly of B‐cell type and can be further subdivided into four major subtypes: extranodal marginal zone lymphoma (EMZL), follicular lymphoma (FL), mantle cell lymphoma (MCL) and large B‐cell lymphoma (LBCL) (Sjo, 2009; Olsen et al, 2019; Vest et al, 2020). LBCLs are some of the most diverse lesions and are subclassified further, with the most frequent type being diffuse large B‐cell lymphoma (DLBCL) (Kluin et al, 2017).…”

“…OALs are almost exclusively NHLs (Margo & Mulla 1998; Holm et al 2021). A large majority of the reported cases are of B‐cell type (>95%), with the most common lymphoma subtype being EMZL (37%–68%), followed by FL (10%–23%), LBCL (10%–15%) and MCL (7%–8%) (Svendsen et al 2017; Olsen et al 2019; Vest et al 2020).…”

“…Lacrimal sac tumours frequently present with epiphora due to obstruction by the tumour (Venkitaraman & George 2007; Neerukonda et al 2022). Orbital tumours and tumours of the lacrimal gland frequently entail exophthalmos, limited eye movement and displacement of the globe (Olsen et al 2019; Vest et al 2020). In addition, vision loss has been described in orbital OALs in cases where invasion or compression of the optic nerve is present (Lamba et al 2016).…”

“…Conjunctival lesions, on the contrary, are often biopsied first, with imaging of the ocular adnexal region performed subsequently to determine the involvement of other structures in the region. A complete workup to determine the extent of the lymphoma includes a CT or an MRI scan of the ocular adnexa, a PET/CT of the entire body and a bone‐marrow biopsy (Aronow & Singh 2014; Vest et al 2020).…”