Abstract
Purpose :
Systemic metastatic retinal lymphoma (SMRL), which originates in systemic organs, is extremely rare. It has been reported to exhibit clinical features similar to those of primary vitreoretinal lymphoma (PVRL) in the US. The purpose of this study was to elucidate the clinical features of SMRL in Japan.
Methods :
We retrospectively studied the clinical and pathological features of 6 Japanese patients with SMRL at the Kyusyu University hospital from 2002 to 2014. The clinical data were extracted for patients with SMRL: sex, age at diagnosis of eye involvement, eye involved, ocular symptoms, a primary lesion of lymphoma, interval between primary lesions to subsequent intraocular lesions, histological types, and the onset of central nervous system (CNS) involvement. In addition, we compared the prevalence of CNS involvement in patients between 6 SMRL and 26 PVRL.
Results :
The subjects were 2 male and 4 female patients with SMRL, with a mean age at first ophthalmic examination of 75.3 ± 3.90. The average interval between the onset of primary systemic lymphomas and subsequent ocular lesions was 56.7 ± 40.2 months. During the observation period, 33.3% patients had unilateral ocular involvement and the others had in both the eyes (66.7%). 1 of 2 male patient had testicular lymphoma. 2 of 4 women had the unilateral breast lymphoma. The other original sites were the chest wall, intestinal tract, and nasal sinus. Vitreous opacity was the most common initial ocular finding, observed in 83.3% (5/6) and 76.2% (20/26) in patients with SMRL and PVRL, followed by subretinal infiltration in 50.0% (3/6) and 69.2% (18/26). All patients with SMRL were diagnosed as diffuse large B-cell lymphomas (DLBCL) in our study, although the frequency of DLBCL was 50% in a report in the US (Cao X, et al., 2011). The prevalence of CNS dissemination in SMRL patients was 66.7% during the observation period, similar to that in PVRL patients was 78%.
Conclusions :
Ocular symptoms of SMRL were similar to those of PVRL. DLBCL is the most common subtypes in Japanese population with SMRL. The prevalence of CNS involvement and the progression to CNS is equal in patients with SMRL to PVRL, suggesting that examinations to detect intracranial lymphoma lesions, including CTs and MRIs, should be routinely performed in patients with SMRL as well as those with PVRL and PCNSL. Furthermore, we propose that the testis and breast are common sites of origin for SMRL.
This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.