目的：探讨临床少见、目前尚无标准治疗方案的晚期泪腺癌的临床表现及治疗方法，以提高对晚期泪腺癌诊治的认识。方法：回顾性分析中国医学科学院肿瘤医院2007年1月至2016年3月收治的6例晚期泪腺癌患者临床资料。结果：6例患者均为男性（33～66岁），中低分化腺癌4例、腺样囊性癌2例。泪腺肿瘤平均最长径为(3.3±0.5) cm，4例侵犯眶壁骨质。在泪腺癌病灶完全切除及放疗后，6例患者均出现远处转移，其中5例骨转移、4例肺转移、2例远处淋巴结转移、皮肤和肝转移各1例。6例患者均接受化疗，5例患者一线采用紫杉醇联合顺铂方案，1例患者一线采用顺铂联合替吉奥方案；治疗后仅1例获得缓解，2例稳定，3例进展。其中1例三线化疗失败的患者应用阿帕替尼治疗取得部分缓解，无进展生存期达6.1个月，不良反应包括高血压、手足综合征、腹泻和咯血，对症治疗后均好转。结论：分化较差和局部侵犯范围广的泪腺癌患者易发生远处转移，骨和肺为常见转移部位。紫杉醇联合顺铂方案化疗疗效欠佳，阿帕替尼用于晚期泪腺癌的治疗值得进一步研究。

Objective:Advanced lacrimal gland carcinomais rare andthere is rate and there is no standard treatment regimen. To analyze the clinical presentation and treatment of advanced lacrimal gland carcinoma, in order to improve the understanding of this rare disease. Methods: Six patients with advanced lacrimal gland carcinoma were treated in our hospital between October 2007 and March 2016. The clinical and follow-up data were analyzed retrospectively. Results: All the patients were male (33-66 years old), including four cases of moderately or poorly differentiated adenocarcinoma and two cases of adenoid cystic carcinoma. The averagelongest diameter of lacrimal gland tumorwas (3.3±0.5) cm, and in four cases the tumor infiltrated the bone of orbital wall. After complete resection of lacrimal gland lesions, distant metastasis appeard in all 6 cases. Bone metastasis was observed in five cases, lung in four,distant lymph node in 2, and skin and liver in 1 case, respectively. Chemotherapy was documented in 6 patients, from whom 5 received cisplantin plus paclitaxel regimen as the first-line chemotherapy and partial response was observed in 1, stable disease in 2 and progression disease in 3. One patient failed threelines of chemotherapy regimens was treated with apatinib as the fourth line therapy and achieved a partial response with a progression-free survival of 6.1 months. The adverse events related with apatinib were hand-foot syndrome, hypertension, diarrheaand hemoptysis, which were all manageable. Conclusion: Poor differentiation and wide local invasion are the risk factors for distant metastases in lachrymal gland carcinoma patients. Bone and lung were common sites of metastases. For patients with metastatic disease, chemotherapy has a limited role, but apatinib may represent an effective therapeutic option and deserves further study.