原发性眼附属器淋巴瘤的临床特点及治疗分析

Clinical characteristics and treatment analysis of primary ocular adnexal lymphoma

目的 探讨原发性眼附属器淋巴瘤（POAL）的临床特点、治疗方法及疗效.方法 回顾性分析1999年6月至2009年6月经病理证实的26例POAL的临床资料,对其临床表现、影像学特点、病理类型及治疗进行分析.结果 临床特点多表现为缓慢生长的无痛性肿块、眼球突出和眼睑红肿;影像学检查多呈＂铸造＂状,50%发生在眼眶.病理分型：22例患者为B细胞来源淋巴瘤,其中13例为结外边缘区B细胞淋巴瘤-黏膜相关淋巴组织型（MALT）淋巴瘤,2例为NK/T细胞淋巴瘤.临床分期：Ⅰ～Ⅱ期占总数的76.9%.手术切除和放化疗为主要治疗手段.结论 POAL多为B细胞来源,MALT淋巴瘤最为常见,恶性度相对较低.临床表现无明显特征性,影像学检查有助于诊断,但难以区别良恶性质,易误诊漏诊.确诊依赖于组织病珲学检查.早期经手术切除后可行单独放疗,恶性度高、出现眶外侵犯者应联合化疗.免疫和分子靶向治疗为今后研究的方向.

Objective To study the clinical characteristics and analyze the treatment of primary ocular adnexal lymphoma （POAL）. Methods A retrospective review was performed based on the clinical records of 26 POAL cases, who were treated from June 1999 to June 2009. The clinical manifestations,imaging features, diagnosis approaches and treatment of them were analyzed. Results The main symptoms were a slowly growing painless mass, exophthalmia and red eyelid. Mostly, imaging finding shows ＂casting like＂. 50 % of POAL was at the orbit. 22 patients w ere B-cell origin [13 patients were extranodal marginal zone B-cell lymphoma-mucosal-associated lymphoid tissue （MALT） lymphoma], 2 patients were NK/T cell lymphoma. 76.9 % patients were in stage Ⅰ -Ⅱ. Surgery, radiotherapy and chemotherapy were most common treatment modalities for POAL. Conclusion Most POAL were low-grade B-cell lymphoma, with MALT by far the most common type. POAL was easy to be misdiagnosed due to non specific clinical manifestations.Imaging finding was helpful to diagnosis, but could not reliably distinguish between benign and malignant. The invasive biopsy is the main diagnosis approach. Radiotherapy had been the standard treatment for low-grade POAL. High-grade or infiltrating peri-orbit need combine chemotherapy. lmmunotherapy and molecular targeted therapy were the direction of future research.