Primary lymphoma of the lacrimal sac: an EORTC ophthalmic oncology task force study

Abstract

Aim: To define the clinical and histopathological characteristics of primary lacrimal sac lymphoma in a predominantly white population. Methods: Specimens of lacrimal sac lymphoma and follow up data were solicited from members of the Ophthalmic Oncology Task Force of the European Organization for Research and Treatment of Cancer (EORTC) and the European Ophthalmic Pathology Society (EOPS). Specimens were stained with haematoxylin and eosin and an immunohistochemical panel against leucocyte antigens was applied. Diagnosis was reached by consensus of five experienced pathologists according to the World Health Organization classification system. The histopathological findings were correlated with the clinical data. Results: Of 15 primary lacrimal sac lymphomas, five (33%) were diffuse large B cell lymphoma (DLBCL), five (33%) were extranodal marginal zone B cell lymphoma of mucosa associated lymphoid tissue (MALT lymphoma), three were classified as ''transitional MALT lymphoma,'' being in transition from MALT lymphoma to DLBCL, and two were unclassified B cell lymphomas. Nine of the patients were female, and the median age at the time of diagnosis was 71 years (range 45-95 years). The most frequent presenting symptoms were epiphora (85%), swelling in the region of the lacrimal sac (79%), and dacryocystitis (21%). All but one patient presented in stage I. Systemic spread occurred in three of nine patients (33%). The 5 year overall survival was 65%. Conclusions: DLBCL and MALT lymphoma are equally common in the lacrimal sac in contrast with the remaining periorbital and/or orbital region where MALT lymphoma predominates.

Chronic Lymphocytic Leukaemia/ Small-Cell Lymphocytic Lymphoma of the Lacrimal Sac: A Case Series

“…During DCR, an incisional biopsy of the lacrimal sac is essential for confirming CLL/SLL involvement, even in the absence of obvious swelling (as in 1 of our cases), and may guide adjuvant chemo- and/or radiotherapy treatment [1,2,3,4,6]. Treatment is usually a combination of surgery, irradiation, and/or chemotherapy (notably regimens involving chlorambucil), but no commonly agreed treatment regimen for periocular lymphoma exists because of the limited number of patients seen [2,3,7,10,11]. …”

“…Previous reporting would have been variable due to lack of global consensus regarding lymphoma classifications until the Revised European-American Classification of Lymphoid Neoplasms, the WHO classification, and later the EORTC ophthalmic oncology task force study defined the clinical and histopathological characteristics [1,2,3,9,10]. From the EORTC study of 15 primary lacrimal sac lymphoma cases, 33% of cases were classified as diffuse large B cell lymphoma (DLBCL; non-Hodgkin's lymphoma), 33% as extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), 20% as transitional MALT lymphoma with features between MALT lymphoma and DLBCL, and 13% as unclassified B cell lymphomas [10]. Of the other in the literature, it appears that DLBCL and MALT lymphomas occur with approximately equal frequency [1,2,3,5,7,9,10,11].…”

“…From the EORTC study of 15 primary lacrimal sac lymphoma cases, 33% of cases were classified as diffuse large B cell lymphoma (DLBCL; non-Hodgkin's lymphoma), 33% as extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), 20% as transitional MALT lymphoma with features between MALT lymphoma and DLBCL, and 13% as unclassified B cell lymphomas [10]. Of the other in the literature, it appears that DLBCL and MALT lymphomas occur with approximately equal frequency [1,2,3,5,7,9,10,11]. However, DLBCL has been more frequently described in association with systemic lymphoma [2,3,7].…”

Lacrimal sac mucocele

“…Primary lymphoma of the lacrimal sac is rare,5 with most reported cases representing secondary involvement from systemic lymphoproliferative malignancy. Lacrimal sac involvement can be a primary site or a metastatic lesion of a systemic lymphoma owing to the presence of rich plexus of lymphatics and blood vessels surrounding the lacrimal sac.…”

“…In cases with high clinical suspicion of a lacrimal sac tumour, an incisional biopsy (even under frozen section) followed by DCT and subsequent definitive therapy including radical resection, radiotherapy or chemotherapy is advocated 5 6. In cases with low clinical suspicion of a tumour, an open dacryocystorhinostomy (DCR) with biopsy of any abnormal tissue can be performed.…”