Objective To summarize the treatment outcomes of orbital adenoid cystic carcinoma and to evaluate prognostic factors. Method A retrospective case series study was performed on 75 patients with orbital adenoid cystic carcinoma treated from 1991 to 2006. Results The 2-and 5-year local recurrence rate of solid type orbital adenoid cystic carcinoma was significantly higher than that of the adeno-tubiform type [2-year, 85% (17/20) vs 23.53% (8/34), χ2= 19.14, P =0.000; 5-year, 100% (19/19) vs 64.52% (20/31), Fisher's exact test, P = 0.003]. The regional extension and distant metastasis of solid type were more than those of adeno-tubiform type. The 5-year local recurrence rate treated by postoperative radiation was lower than that treated with only surgical excision [70% (14/20) vs 92.86% (13/14) ;Fisher's exact test, P = 0.198]. The 5-year local recurrence rate in patients initially treated by orbital evisceration during the first time was lower than that of cases which evisceration procedure was used after the recurrence [25% (1/4) vs 75% (6/8), Fisher's exact test, P =0.222]. Tumors may extend into intracalvarium, nasal cavity and temporal fossa. They may spread to the lung, bone, liver and lymph node. The 5-year metastasis rate was 25.71% (9/35). Both of the lung and bone metastasis rates were 33.33% (3/9). The overall 5-year accumulative survival was 74.29% (26/35), mortality was 25.71% (9/35),and rate of survival without tumor recurrence was 37.14% (13/35). The 10-year disease free survival rate was 17.14% (6/35). Patients were most likely to die with intracranial extension. Surgical excision with postoperative radiation improved the 5-year survival rate to 80% (16/20). Conclusions Orbital adenoid cystic carcinoma is one of the most malignant tumors in the orbit. They have a high local recurrence rate and survival rate. Tumor histological types and the treatment procedure can influence the prognosis. Combined therapy may decrease the recurrence and increase the survival rate.%目的 分析总结眼眶腺样囊性癌的治疗方法及影响预后的因素,为改善预后提供帮助.方法 采用回顾性系列病例研究,分析1991年1月至2006年7月就诊的75例眼眶腺样囊性癌患者的手术记录、病理分型及随访记录.相关数据采用χ2检验和Fisher精确检验进行统计分析.结果 眼眶实体型腺样囊性癌的2年复发率为85%(17/20)、5年复发率为100%(19/19),而腺样.管状型则分别为23.53%(8/34)和64.52%(20/31),差异有统计学意义(2年,χ2=19.14,P=0.000;5年,Fisher精确检验,P=0.003).前者发生局部蔓延和远处转移例数亦多于后者.肿瘤切除术后放射治疗的5年复发率为70%(14/20),低于单纯手术切除的复发率92.86%(13/14)(Fisher精确检验,P=0.198).首次手术行眶内容物剜除术的5年复发率为25%(1/4),低于复发后再行眶内容物剜除术的病例为75%(6/8)(Fisher精确检验,P=0.222),γ刀、粒子刀、化疗及生物治疗的效果不能确定.局部蔓延主要是至颅内、副鼻窦和颞窝,远处转移可到达肺、骨、肝、耳前淋巴结.5年远处转移率为25.71%(9/35),肺转移和骨转移各占33.33%(3/9).5年生存率74.29%(26/35),死亡率25.71%(9/35),无瘤生存率37.14%(13/35),10年无瘤生存率17.14%(6/35).最常见的死亡原因是颅内蔓延.肿瘤切除联合放射治疗可以使5年生存率提高到80%(16/20).结论 腺样囊性癌是高度恶性的眼眶肿瘤,复发率和死亡率均较高,病理分型、治疗方法均影响预后.采取综合治疗方法,可以减少复发,提高生存率.
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