乳腺腺样囊性癌临床病理特点及预后分析

Adenoid cystic carcinoma of the breast:clinical features and prognosis of 22 cases

目的探讨乳腺腺样囊性癌（adenoid cystic carcinoma,ACC）的临床特点及预后情况。方法回顾性分析新疆医科大学附属肿瘤医院1991-03-01-2014-05-01经病理确诊为ACC的22例患者临床病理资料并对其随访。22例患者均为女性,年龄36-79岁,中位年龄54岁。结果 22例ACC患者中10例患者行乳腺癌改良根治术,4例患者行乳腺癌根治术,8例患者行乳腺单纯切除术。B超检查显示,22例ACC患者均表现为有完整声晕包绕的低回声、混杂的声团,13例患者在B超检查中诊断为乳腺癌,其中6例边界清晰者伴有动脉血流信号,边界欠清晰者7例,超声的检出率为59.1%（13/22）。病理显示,13例呈筛状型,7例呈梁状-管状型结构,2例呈实体巢团型结构;均未发现淋巴结转移及神经受侵。免疫组化结果显示,22例患者中雌激素受体（estrogen receptor,ER）和孕激素受体（progesterone receptor,PR）均为阴性;18例患者人表皮生长因子受体2（human epidermal growth factor receptor-2,HER-2）表达为阴性,4例表达情况不详。随访至2014-05-01,21例患者无病生存,1例患者于术后168个月死于心脏病。结论 ACC发病率极低,与其他病理类型乳腺癌的病理特点、手术范围及预后不同,肿瘤进展慢,很少出现腋窝淋巴结转移及远处转移,预后较好,治疗上主张行乳腺单纯切除术或肿块切除术。

OBJECTIVE To explore the clinical features and prognosis for adenoid cystic carcinoma（ACC）of the breast.METHODS A retrospective analysis of 22 cases pathologically diagnosed breast adenoid cystic carcinoma was carried out in Tumor Hospital Affiliated to Xinjiang Medical University from March 1 1991 to May 1 2014.The clinical features and the follow-up data were analyzed too.RESULTS The median age of 22 ACC patients was 54 years old,10 patients underwent modified radical mastectomy,4patients underwent radical mastectomy,8patients underwent breast simple excision.Pathological foundings demonstrated no lymph node metastases and nerve involvement.Immunohistochemistry staining showed that the estrogen receptor（ER）and progesterone receptor（PR）were both negative in 22 patients.Human epidermal growth factor receptor-2（HER-2）expression was negative in 18 cases,and unknown in 4cases.Twenty-one patients survived disease-free,one patient died of heart attack 168 months after the surgery.CONCLUSIONS The incidence of adenoid cystic carcinoma of the breast is very low.The pathological features,the extent of surgery and prognosis are different from other types of breast cancer by slow tumor progression,rarely axillary lymph node and distant metastasis.The prognosis is better than that of other breast cancers.Breast simple excision or lumpectomy is reconmended for the treatment.