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目的食管腺样囊性癌(adenoid cystic carcinoma,ACC)临床上罕见,文献多为个案报道或小样本病例,对本病缺乏系统了解。本研究回顾性分析22例食管ACC患者,分析其临床病理特征及诊疗策略,为临床提供参考。方法分析河北医科大学第四医院2004-01-01-2013-12-31行根治性手术切除,且术后病理确诊为食管ACC患者的临床病理资料,采用Kaplan-Meier法进行生存分析。结果食管ACC发病率男性多于女性,男女比例为4.5∶1。发病部位以胸中段最常见,为14例;其次是胸上段和胸下段,各4例。Ⅰ期19例,Ⅱ期2例,Ⅲ期1例。食管ACC的术前内镜诊断率较低,仅为27.3%(6/22),其中11例被误诊为鳞状细胞癌,4例误诊为腺癌,1例误诊为腺鳞癌。全组患者淋巴结转移率为9.1%(2/22),淋巴结转移度为0.9%(2/229),肺转移发生率为27.3%(6/22)。全组患者1、3、5年生存率分别为90.9%、81.3%和65.1%,术后综合治疗与单纯手术相比生存率差异均无统计学意义,χ~2=0.054,P=0.816。结论食管ACC发病男性多于女性,发病部位以胸中段最为多见,术前内镜诊断确诊率低。食管ACC总体预后较好,淋巴结转移并不常见,治疗失败以肺转移最为多见,术后是否需行综合治疗尚待进一步探索。
Purpose Adenoid cystic carcinoma (ACC) is rare in clinical practice. Most cases are reported as case reports or small sample cases, and lack of systematic understanding of this disease. This study retrospectively analyzed 22 cases of esophageal ACC patients, analysis of its clinical and pathological features and diagnosis and treatment strategies for clinical reference. Methods The clinical data of patients with esophageal ACC admitted to the Fourth Hospital of Hebei Medical University from January 2004 to December 13, 2013 were analyzed retrospectively. The Kaplan-Meier method was used for survival analysis. Results The incidence of esophageal ACC was higher in males than females, with a ratio of male to female of 4.5: 1. The most common part of the chest to the middle of the chest, 14 cases; followed by the upper thoracic and thoracic segments, each 4 cases. 19 cases in stage I, 2 cases in stage II and 1 case in stage III. The preoperative endoscopic diagnosis rate of esophageal ACC was low, only 27.3% (6/22), of which 11 cases were misdiagnosed as squamous cell carcinoma, 4 cases misdiagnosed as adenocarcinoma and 1 case misdiagnosed as adenosquamous carcinoma. The rate of lymph node metastasis in all patients was 9.1% (2/22), lymph node metastasis was 0.9% (2/229), and lung metastasis was 27.3% (6/22). The 1, 3, 5-year survival rates of all patients were 90.9%, 81.3% and 65.1% respectively. There was no significant difference in survival rates between the postoperative comprehensive treatment and the simple operation (χ ~ 2 = 0.054, P = 0.816). Conclusion There are more men than women in the incidence of esophageal ACC, the most common part of the disease is the middle part of the chest, and the diagnosis rate of endoscopic diagnosis is low. Esophageal ACC overall prognosis is good, lymph node metastasis is not common, the failure of treatment of lung metastasis is the most common, whether after comprehensive treatment remains to be further explored.