[Clinical and pathological analysis of ocular adnexal diffuse large B-cell lymphoma]

Objective: To summarize the clinical and pathological characteristics of diffuse large B-cell lymphoma of ocular adnexal (OA-DLBCL). Methods: A retrospective case series study. Twenty-three cases of OA-DLBCL were collected in Tianjin Eye Hospital from January 2005 to December 2018. The clinical manifestations and imaging examination results were analyzed. The pathological characteristics and immune subtypes were analyzed with hematoxylin and eosin staining and immunohistochemistry. Eighteen patients completed the follow-up. Overall survival (OS) time was from the date of diagnosis to the date of death or the final follow-up (April 30, 2020). Clinical stages were analyzed by the Ann Arbor classification; Kaplan-Meier plots were used to visualize survival outcomes. The different risk groups were compared using Log-rank test. Results: There were 13 males and 10 females. The median age was 65 years (range, 43 to 82 years). Twenty-two patients had unilateral lymphoma (14 left eyes, 8 right eyes), and one patient had bilateral lymphoma. The OA-DLBCL was in the orbit in 14 patients, in the lacrimal gland in two patients, in both the lacrimal gland and the orbit in three patients, in both the lacrimal sac and the orbit in one patient, in the conjunctiva in one patient, in both the conjunctiva and the orbit in one patient, and in the skin of eyelids in one patient. Imaging examinations showed the tumors were of irregular soft tissue density. MRI showed the tumors were close to the extraocular muscles or ectocinerea. Centroblastic morphology was present in 21 specimens, and 2 specimens had immunoblastic morphology. Six patients were of germinal center B cell-like (GCB) type, and 17 were of non-GCB type. There were 2 patients with double expression of cellular-myelocytomatosis viral oncogene and B-cell lymphoma 2. The follow-up time ranged from 25 to 156 months, and the median follow-up time was 48 months. Five of them had primary OA-DLBCL, and 13 patients had secondary OA-DLBCL. Five patients were at the Ann Arbor clinical stage ⅠE, one was at stage ⅢE, and 12 were at stage ⅣE. During the follow-up, 8 patients survived, and 10 died. The 1-, 3-, and 5-year OS rates were 88.9%, 71.4% and 41.7%, respectively. Log-rank analysis indicated that the Ann Arbor clinical stage and age were related to the OS of OA-DLBCL (χ²=7.448, 8.804; both P<0.01). The gender, tumor size, molecular typing, Ki-67 index, and bone invasion were not related to the OS of OA-DLBCL (all P>0.05). Conclusions: OA-DLBCL mainly occurrs in the elderly population, unilaterally, and in the orbit. Most molecular types are non-GCB subtypes. The Ann Arbor clinical stage and age are associated with prognosis. (Chin J Ophthalmol, 2021, 57: 366-371).