Russell Wesley, Herskovic Arnold, Gessert David, Cohen Jack A, Rubenstein Jonathan B, Gregory Stephanie A
Rush University Medical Center, Department of Radiation Oncology, Chicago, IL 60612, USA.
Clin Adv Hematol Oncol. 2013 Apr;11(4):209-14.
Ocular adnexal mucosal-associated lymphoid tissue lymphomas (MALTomas) are rare, and there are no phase III trials to guide treatment. Primary radiation therapy has been the typical management. This retrospective series reports the experience of a single institution and adds to the current literature.
Our electronic medical record system and available paper charts were used to identify patients with MALTomas of the lacrimal gland or sac, conjunctiva, and orbital structures, including extraocular muscles. In order to determine pathology, staging, treatment information, local and distant control, salvage treatments, and late toxicity, records were reviewed.
Sixteen patients with ocular adnexal MALTomas had local radiation between 1992 and 2011 for primary or recurrent disease. Fifty percent of patients had lymphoma in the conjunctiva, 25% had lymphoma in the lacrimal sac/gland, and 25% of patients had lymphoma in the posterior orbit. Stage IAE disease occurred in 75% of patients, 6% had stage IIAE disease, and 19% of patients had a positive bone marrow biopsy. One patient received chemotherapy as part of initial therapy. The median radiation dose was 30 Gy (25.5-36 Gy) delivered with electrons (31%) or photons (69%). After a mean follow-up of 62.8 months, 2 patients had residual/progressive disease, 2 had contralateral recurrence, and 1 patient had a distant failure, for local control of 87.5% and overall disease control of 68.75%. Recurrence/progression occurred at a median of 35.45 months. Two patients with residual/progressive disease and 1 patient with a contralateral recurrence were followed, successfully salvaged, and have no evidence of disease. Fourteen patients are still alive, and there were no disease-related/toxicity deaths. Seven patients developed cataracts in the treated eye, 2 patients had radiation retinopathy, 2 had permanent dry eye syndrome, and 1 patient had severe keratopathy requiring enucleation. Six patients (3.75%) had worsening visual acuity of unclear etiology.
Primary radiation therapy for ocular adnexal MALTomas with a median dose of 30 Gy led to excellent local control. Patients who did recur were successfully salvaged. Radiation was generally well tolerated, with expected cataractogenesis, given the dose required to achieve local control (with only 1 patient developing severe keratopathy after receiving the highest dose in this series).
眼附属器黏膜相关淋巴组织淋巴瘤（MALToma）较为罕见，尚无III期试验来指导治疗。原发性放射治疗一直是典型的治疗方法。本回顾性系列报告了单一机构的经验，并补充了当前的文献资料。
利用我们的电子病历系统和现有的纸质病历，识别泪腺或泪囊、结膜以及眼眶结构（包括眼外肌）发生MALToma的患者。为了确定病理、分期、治疗信息、局部和远处控制情况、挽救性治疗以及晚期毒性，对记录进行了审查。
1992年至2011年间，16例眼附属器MALToma患者因原发性或复发性疾病接受了局部放疗。50%的患者淋巴瘤位于结膜，25%位于泪囊/泪腺，25%位于眼眶后部。75%的患者为IAE期疾病，6%为IIAE期疾病，19%的患者骨髓活检呈阳性。1例患者接受了化疗作为初始治疗的一部分。中位放疗剂量为30 Gy（25.5 - 36 Gy），采用电子线（31%）或光子线（69%）照射。平均随访62.8个月后，2例患者有残留/进展性疾病，2例有对侧复发，1例有远处转移，局部控制率为87.5%，总体疾病控制率为68.75%。复发/进展的中位时间为35.45个月。对2例有残留/进展性疾病的患者和1例有对侧复发的患者进行了随访，成功挽救，且无疾病证据。14例患者仍存活，无疾病相关/毒性死亡。7例患者治疗眼发生白内障，2例有放射性视网膜病变，2例有永久性干眼综合征，1例有严重角膜病变需行眼球摘除术。6例患者（3.75%）视力下降，病因不明。
眼附属器MALToma的原发性放射治疗，中位剂量为30 Gy，可实现良好的局部控制。复发的患者成功得到挽救。放射治疗一般耐受性良好，鉴于实现局部控制所需的剂量，白内障形成是预期的（本系列中仅1例患者在接受最高剂量后发生严重角膜病变）。
