摘要目的 探讨腺样囊性癌高级别转化的临床病理特点.方法 收集首都医科大学附属北京同仁医院病理科2005年1月至2011年12月间4例腺样囊性癌高级别转化病例的临床及病理资料,采用免疫组织化学EnVision法标记平滑肌肌动蛋白(SMA)、p63、p53、Ki-67；用荧光原位杂交(FISH)方法检测c-myc基因.结果 4例患者中男性3例、女性1例,平均年龄55.5岁.2例分别于术后29个月,17个月死亡；1例术后23个月发生多处转移带瘤存活(随访26个月)；1例存活(随访3个月).高级别转化的组织病理学表现为低分化腺癌或未分化癌形态.异型性明显的肿瘤细胞连接成片,面积超过一个低倍视野；核质比明显增高；核仁及核分裂象易见(8～25/10 HPF).2例出现粉刺样坏死,3例出现多灶钙化.免疫组织化学染色显示转化区域SMA和p63阳性肌上皮细胞数日明显减少或消失,p53强阳性,Ki-67阳性指数增高.4例均未出现c-myc基因扩增或分离.结论 腺样囊性癌高级别转化组织病理学改变有显著特征,肌上皮减少,甚至消失,生物学行为差.c-myc基因突变可能不具有关键作用.

abstractsObjective To study the clinicopathologic features and possible molecular mechanisms of adenoid cystic carcinoma with high-grade transformation.Methods Four cases of adenoid cystic carcinoma with high-grade transformation were enrolled into the study.Immunohistochemical study for smooth muscle actin,p63,p53 and Ki-67 was carried out.C-myc gene status was analyzed by fluorescence in-situ hybridization.Results There were altogether 3 males and 1 female.The mean age of the patients was 55.5 years.Two patients died 17 months and 29 months after operation,respectively.One patient had distant metastasis 23 months after operation and was still alive at 26-month follow up.The remaining patient remained tumor free at 3-month follow up.High-grade transformation in adenoid cystic carcinoma presented either as poorly differentiated adenocarcinoma or undifferentiated carcinoma.Histologic examination showed sheets of pleomorphic tumor cells occupying more than one low-power field.The high-grade carcinoma cells showed increased nuclear-cytoplasmic ratio,prominent eosinophilic nucleoli and active mitosis (ranging from 8 to 25 per high-power field).Comedo necrosis was observed in 2 cases and multiple loci of calcifications in 3 cases.Immunohistochemical study demonstrated loss of myoepithelial differentiation,overexpression of p53 and high proliferative index by Ki-67.No c-myc translocation or copy-number changes were observed.Conclusions High-grade transformation in adenoid cystic carcinoma is rare.The histopathologic features are rather distinctive and the biologic behavior is aggressive.C-myc gene mutation does not seem to play a key role in the pathogenesis.