[Treatment and prognosis of primary ocular adnexal extranodal marginal zone mucosa-associated lymphoid tissue lymphoma: a report from a single center].

Objective: This study aimed to see how different initial treatment regimens affected the long-term prognosis of patients with extranodal marginal zone mucosa-associated lymphoid tissue lymphoma confining to the ocular adnexal (OAML) . Methods: Between April 2008 and April 2019, 109 patients with initial mucosa-associated lymphoid tissue confining to ocular adnexal were evaluated and followed-up, and the prognosis of various initial treatment regimens were examined. Results: A total of 36 patients underwent complete surgical resection of the lesions, and 73 patients had residual lesions after surgery, of which 37 patients chose watchful waiting, and 36 patients chose treatment. The treatment regimen included local radiotherapy and systemic treatment (chemotherapy, immunochemotherapy, the combination of radiotherapy and chemotherapy, etc.) , and no serious toxic and side effects were observed in patients receiving systemic treatment. The median follow-up time was 61 (10-142) months. The 5-year and 10-year progression-free survival (PFS) of monocular involvement patients were 78.2% and 76.0% . The 5-year and 10-year PFS rates of patients with binocular involvement were 64.4% and 23.5%. There was significant diference in PFS between patients with monocular and binocular involvement (P=0.010) . Patients who received additional treatment had higher PFS than those patients in the watchful waiting group (P=0.046) . The 5-year PFS was 71.4% and 90.1% among patients in the watchful waiting group and those who received additional treatment, whereas the 10-year PFS was 63.5% and 75.1% , respectively. Patients with OAML were still a risk of disease progression after 5 years. Conclusions: Patients with binocular involvement OAML at the start of the disease had a poor prognosis, but treatment could reduce the risk of recurrence/progression. Systemic therapy is one of the first-line treatment options for patients with OAML, who require long-term monitoring.目的： 探讨局限于眼附属器黏膜相关淋巴组织结外边缘区淋巴瘤（OAML）患者应用不同初始治疗方案对长期预后的影响。 方法： 对2008年4月至2019年4月首都医科大学附属北京同仁医院血液科收治的109例初发OAML患者进行疗效评估及随访，对初始治疗方案与预后进行分析。 结果： 36例患者经手术完全切除病灶，73例术后存在残留病灶，其中37例选择观察等待，36例选择治疗，治疗方案包括局部放疗和系统性治疗（化疗、免疫化疗、放化疗联合等），选择系统性治疗患者未发生严重不良反应。中位随访时间61（10~142）个月，单眼受累患者5年、10年无进展生存（PFS）率分别为78.2%和76.0%，双眼受累患者5年、10年PFS率分别为64.4%和23.5%，单眼、双眼受累患者PFS的差异有统计学意义（P＝0.010）。残留病灶观察组患者和残留病灶治疗组患者5年PFS率分别为71.4%和90.1%，10年PFS率分别为63.5%和75.1%，两组患者PFS率的差异有统计学意义（P＝0.046）。OAML患者超过5年仍有疾病进展风险。 结论： 双眼受累OAML患者预后差，治疗可以降低复发或进展风险。系统性治疗可作为OAML患者的一线治疗选择之一。OAML需要长期随访。.