Clinical Manifestations and Treatment Results in Accordance with Frontline Treatment Modalities for Ocular Adnexal Mucosa-Associated Lymphoid Tissue Lymphoma (OAML)

Abstract

Background: localized ocular adnexal mucosa-associated lymphoid tissue lymphoma (OAML) was effectively treated with frontline radiotherapy. however it is sometimes associated with radiation-related complications of ocular structures, as well as distant relapse or delayed relapse. Although there are no definitive optimal treatment approaches for these patients, we investigated the results in accordance with frontline treatment modalities for OAML.Patients and methods: 159 patients with biopsy-confirmed primary OAML between 2007 and 2015 were analyzed retrospectively. All the patients were sorted by risk-stratification with Ann-Arbor staging and the tumor, node, metastasis (TNM) staging system, and treatment modalities according to anatomic location, prognosis factors, which were supported by ophthalmologist.Results: first-line radiotherapy had overall survival(OS) of 100%, event-free survival(EFS) of 90%. However frontline-radiotherapy group has 100% of survival with 55% of moderate to severe dry eye syndrome, 37.4% of cataract incidence, 11% of cataract-related operations, and 1% (n=1) of radiation-related blindness. Although 5-year EFS is good in radiotherapy group (90% vs 64% in radiotherapy group vs chemotherapy, p<0.001), most of aggressive factors (young age at diagnosis, conjunctival lesions, Ann Arbor stage I, early T1N0M0 stage, and no distant extranodal metastasis including of bone marrow involvement) showed in radiotherapy group in comparison with chemotherapy group. In subgroup analysis of first-line chemotherapy, rituximab-containing group has more good response of event-free survival (p= 0.041) with tolerable hematologic toxicities compared to frontline radiotherapy group.Conclusion: OAML with localized to conjunctiva alone was treated with frontline-radiotherapy and non-conjuctival lesions treated with chemotherapy. Radiotherapy as first-line is good local control-therapeutic option in patients with localized to conjunctiva alone, but management of radiation-associated complication is concerned troubles. In advanced OAML including of extra-conjunctival lesions, systemic chemotherapy is best options. rituximab-based regimen could be feasible to obtain good disease-free survival and response rate.Table 1Patient's characteristics between frontline-chemotherapy and radiation group (n= 159)FactorsPrimary Chemotherapy group(n = 68)Primary Radiation group(n = 91)P-valueGender (male)37 (54%)28 (30.8%)0.003Age at diagnosis (median, year)50430.003involvement of both eyes at diagnosis22 (32.4%)23 (25.3%)0.75Conjuctival lesion at diagnosis22 (32.4%)76 (83.5%)<0.001Ki-67 on pathologic stain (median, %)540.545Ann-Arbor stage I23 (66.2%)3 (96.7%)<0.001LDH at diagnosis (mg/L)3553440.216Patients no. with T1N0M021 (30.9%)78 (85.7%)<0.001Bone marrow involvement7 (10.3%)0 (0%)<0.001Patients no. with N1~N3 staging10 (14.7%)0 (0%)<0.001Patients no. with T0N0M1~27 (10.3%)0 (0%)<0.001Patients treated with frontline chemotherapy were classified in more aggressive or advanced stage which compare to patients treated with primary radiotherapy statistically.Table 2Patient's characteristics between Rituximab containing group and non-rituximab in frontline chemotherapy subgroup (n=68)FactorsRitixumab-containing(n = 26)No Rituximab(n = 42)P-valueGender (male)0.942Age at diagnosis (median, year)52490.411involvement of both eyes at diagnosis0.057Conjuctival lesion at diagnosis0.087Ki-67 on pathologic stain (median, %)430.540Ann-Arbor stage I0.532LDH at diagnosis (mg/L)3533560.782Patients no. with T1N0M00.585Patients no. with N1~N3 staging0.659Patients no. with T0N0M1~20.695There are statistically no differences between patients treated with Rituximab-containing regimen and non-rixuximab in frontline chemotherapy subgroup. [Display omitted] [Display omitted] [Display omitted] DisclosuresNo relevant conflicts of interest to declare.