Treatment of Bilateral Synchronous Ocular Adnexa Marginal Zone Lymphoma: Consortium for Improving Survival of Lymphoma (CISL) Study

BACKGROUND:

Ocular adnexal marginal zone lymphoma (OAML) represents 1-2% of all non-Hodgkin lymphomas and 8% of extranodal lymphomas. In Korea, OAML occupy about 25% of MZL. Both side synchronous involvement has been reported around 10~20% of OAML. Several treatment modalities- antibiotics, chemotherapy, chemo-immunotherapy, or radiotherapy can be used for the treatment of OAML. Especially radiotherapy in localized stage OAML is well controlled with low dose radiation and be able to be persisted its effects for a long survival duration. Despite the prominent local control of tumor, radiotherapy had the disadvantages of ophthalmologic toxic effects, such as moderate cutaneous or conjunctival reactions, as well as late complications such as constant cataract. Chemotherapy or chemo-immunotherapy with rituximab has been studied for the first line treatment of OAML. But there is no clinical treatment results about bilateral synchronous OAML (BS-OAML). Therefore, in this study we conducted a retrospective analysis to identify the clinical features and treatment outcomes of BS-OAML according to treatment modality, thus providing basic clinical data for prospective clinical trials.

METHODS:

Between Nov. 2001 and May. 2016, the patients with histologic diagnosis of BS-OAML from different institutions in Korea were included for analysis in this study. Metachronous bilateral involved OAML was excluded. Patients who treated with doxycycline or had watchful wait also excluded.

The following clinical data were obtained from the records: patient demographics, complete blood count, lactic dehydrogenase (LDH) level, International Prognostic Index (IPI), bone marrow (BM) findings, the presence of B symptoms, performance status, date of diagnosis, type of treatment, treatment response, date of relapse, date of last follow-up, vital status, and cause of death.

RESULTS

A total of 103 patients were enrolled at this analysis from 9 institutes in Korea. 42 males and 61 females were included as participants in this study. The median patient age was 43 years (range, 16-77 years). Except 1 patient evidenced good performance status (ECOG 0 or 1). B symptoms were not noted in all patients. 87.4% (90 of 103) were in the low-risk group according to the IPI.

Fourteen (13.6%) patients treated with chemo/chemo-immunotherapy (11 R-CVP, 2 CVP and 1 CHOP). Median number of treatment was 6 (range 6-8). There were 12 CRs (85.7%) and 2 PRs (14.3%). Almost of patients (86.4%) were received both ocular radiotherapy. Median radiation dose was 2650 rad (range 2000-4000 rad) to each eye. 71 CRs (79.8%) and 14 PRs (15.7%) were achieved.

Five year progression free survival (PFS) and overall survival (OS) rate were 90.6% and 96.7%. 5 year PFS rate of chemo/chemo-immunotherapy group and radiotherapy group were 75% and 92.3%, respectively. But statistical significance was not observed (P=0.088). Also, there was no difference in OS according chemotherapy or radiotherapy.

CONCLUSIONS:

BS-OAML was observed in relatively young female. They had good response and prognosis regardless of treatment modality. But radiotherapy had been observed tendency of prolonged PFS.