33例原发鼻咽腺样囊性癌临床分析

Clinical analysis on nasopharyngeal adenoid cystic carcinoma

目的探讨分析鼻咽腺样囊性癌的临床诊治及预后。方法回顾分析2002年前近40年间收治的33例鼻咽腺样囊性癌患者，其中13例接受综合治疗（手术＋放疗9例；放疗＋手术4例），20例采用单纯放疗。从发病到确诊最短为1．0个月，最长为36．4个月，中位值为12个月。确诊前18例误诊，以中耳炎多见。临床症状基本与鼻咽鳞癌相似。颈部淋巴结转移少见（4例），颅神经受累多见（19例）。结果全组随访5．0-245．2个月，中位随访时间60．8个月。5、10年总生存率分别为66％、29％，无瘤生存率分别为41％、27％，局部控制率分别为61％、40％，无远处转移率均为62％。治疗失败20例，其中15例局部区域复发（13例原位复发，2例颈上淋巴结转移）。发生远处转移9例，其中肺转移5例，肺＋骨2例，肝1例，骨1例。单因素分析临床分期（Ⅰ＋Ⅱ期：Ⅲ＋Ⅳ期，P=0．009）影响预后，治疗模式（单纯放疗：综合治疗，P=0．598）5生存率相似（65％：67％）。结论鼻咽腺样囊性癌是一种局部侵袭性强、病程发展相对较慢、可带瘤长期存活的疾病。治疗上可选择单纯放疗，也可选择综合治疗（手术＋放疗或放疗＋手术）。

Objective To investigate the clinical features, treatment outcome and prognosis of patients with nasopharyngeal adenoid cystic carcinoma（ACC）. Methods From March 1963 to August 2002, 33 patients with ACC were retrospectively reviewed. Among them, 13 received multimodality therapy （S ＋ R in 9 and R ＋ S in 4） and the remaining 20 received radiotherapy alone. The interval between the first visit and clinical diagnosis was 12 months （ 1.0-36.4 months）. The misdiagnosis rate was 55 % （ 18/33 ）, with the most common error of otitis media. The clinical symptoms of ACC were similar to those of squamous carcinoma in nasopharynx,though the cervical lymph node metastasis was rare（ 12% ） and cranial nerve invasion was common （58%）. Results After a median follow-up of 60.8 months （5.0-245.2 months）, the 5-and 10-year overall survival rates,free-disease survival rates,local regional control rates and metastasis-free survival rates were 66% and 29% ,41% and 27% ,61% and 40% ,62% and 62% ,respectively. Among the 20 patients（61% ） with recurrence, 15 had local regional failure（ 13 in situ and 2 in cervical lymph node） ,and 9 had distant metastasis（5 in the lung,2 in the lung and bone,1 in the liver and 1 in the bone）. Univariate analysis showed that clinical stage was a prognostic factor（Ⅰ＋ⅡvsⅢ＋ Ⅳ,P=0.009）, while treatment modality（radiotherapy alone vs multimodality therapy） was not. Conclusions Nasopharyngeal ACC is a locally aggressive disease with a long course. Either radiotherapy alone or multimodality therapy（ S ＋ R/R ＋ S）is effective in the treatment.