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Title: [Clinical features of advanced adenoid cystic carcinoma in the nasal cavity and paranasal sinuses: analysis of 21 cases].
Author: Wang HG, Shen CX, Chen F, Li GX, Wang XQ, Wen Z.
Journal: Nan Fang Yi Ke Da Xue Xue Bao; 2017 Jun 20; 37(6):847-852. PubMed ID: 28669965.
Abstract:
OBJECTIVE: To evaluate the clinical characteristics, treatment and prognosis of advanced adenoid cystic carcinoma (ACC) in the nasal cavity and paranasal sinuses. METHODS: Twenty-one patients with advanced ACC in the nasal cavity and paranasal sinuses were treated in our department between February, 2007 and May, 2016. The clinical manifestations, T-stage, N-status, treatment, histological grade, recurrence and distant metastasis of the tumors were analyzed. Univariate survival analysis was performed with Kaplan-Meier method and Log-rank test, and the factors affecting the prognosis of the patients were explored using multivariate analysis with Cox proportional hazard model. RESULTS: Among the 21 patients, 10 (47.6%) had ACC containing less than 30% of solid tumor tissues and their overall survival rates at 1, 3, and 5 years were 100%, 100% and 70%, respectively; in the 11 cases (52.4%) with solid tumor tissues no less than 30%, the overall survival rates at 1, 3, and 5 years were 70%, 40% and 10%, respectively, showing significant differences between the two groups (P=0.02). The Log-rank test and survival analysis using the covariate variable model curve indicated a significant impact of the pathological classification on the patients' prognosis. The patients in T3 stage had slightly better prognosis than those in T4 stage; tumors originating from the maxillary sinus had a slightly better prognosis than those from the sphenoid sinus. Surgery combined with radiotherapy resulted in better outcomes of the patients than surgery or radiotherapy alone. Multiariable Cox regression model analysis showed that the pathological classification (P=0.045) and the disease course (P=0.028) were closely related with the prognosis of the patients. CONCLUSION: ACC in the nasal cavity and paranasal sinuses has a low incidence without specific symptoms. Its early diagnosis can be difficult, and most of the patients are in advanced stage upon diagnosis. We recommend comprehensive treatments combining surgery, postoperative radiotherapy and chemotherapy for these patients. The pathological classification, disease course, lesion site, clinical stage, treatment approache, compromise of the peripheral nerves, status at the edge of resection, and postoperative radiotherapy dose can all be factors affecting the prognosis of patients with advanced ACC. 目的: 探讨发生于鼻腔鼻窦晚期腺样囊性癌的临床特征、治疗方法、预后及影响因素。方法: 分析2007年2月~2016年5月入住我院的21例晚期鼻腔鼻窦腺样囊性癌患者的临床资料，包括临床表现、临床分期、病理、治疗方法、局部复发、远处转移及预后等，并结合文献进行诊疗特征分析。采用Kaplan-Meier法和Log-rank检验进行单因素生存分析, COX模型对影响预后的临床因素进行多因素分析。结果: 21例患者中，实体型占比小于30%的有10例（47.6%），1、3、5年总生存率分别为100%、100%、71%，实体型占比大于等于30%的有11例（52.4%），1、3、5年总生存率分别为70%、40%、10%，两者差异有统计学意义（P=0.02），且实体型占比大于等于30%的患者预后较差。Log-rank检验及全变量模型下协变量的生存曲线表明病理分型对预后具有显著影响，T3期预后略优于T4期，源发于上颌窦略优于蝶窦，手术及放化疗综合治疗优于单纯手术及单纯放化疗。多因素Cox回归模型分析结果显示，病理分型、病程与预后关系密切（P=0.045、0.028）。结论: 鼻腔鼻窦腺样囊性癌发病率较低，症状表现无特异性，部分患者病程较长，多发鼻窦内，尤其是上颌窦，早期诊断较困难，等到就诊时多为晚期。晚期患者以手术结合术后放化疗的综合治疗方式为主。病理分型、病程、发病部位、分期、治疗方式、周围神经侵袭，手术切缘阳性，术后放疗剂量不足60 Gy可能是影响晚期腺样囊性癌患者预后的因素。

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