乳腺腺样囊性癌临床病理特征——附10例报道及文献复习

Clinicopathological characteristics of adenoid cystic carcinoma in breast: report of 10 cases and review of literature

目的：探讨乳腺腺样囊性癌（adenoid cystic carcinoma,ACC）患者的临床病理特征,以提高医师对该病的认识及诊断水平。方法：收集10例乳腺ACC患者的临床资料,分析其病理形态学特征及免疫组织化学（免疫组化）表现,并结合文献进行探讨。结果：10例乳腺ACC患者均为女性,平均年龄为（61±14）岁。6例患者的肿块位于左乳,4例位于右乳,且以外上象限多见,主要临床表现为膨胀性生长的肿块;肿瘤最大直径为1~4 cm。病理检查显示肿瘤细胞排列成管状-小梁状、筛状型和实体型,免疫组化标记结果均不表达雌激素受体、孕激素受体及原癌基因人类表皮生长因子受体2（human epidermal growth factor receptor 2,HER-2）,而不同程度表达酪氨酸激酶受体（CD117）;细胞增殖指数（Ki-67）的阳性指数为5%~30%。10例患者中有9例获得随访,随访中位时间为4~110个月,其中1例患者因多功能器官衰竭死亡,另8例患者生存状态良好。结论：乳腺ACC是一种少见的、生物学行为呈惰性的恶性肿瘤,其病理学形态独特,需与其他乳腺良、恶性病变鉴别。

Objective： To investigate the clinieopathological characteristics of adenoid cystic carcinoma （ACC） in breast for improving the recognition and diagnosis of ACC. Method： Ten female patients with ACC were enrolled. Clinical symptoms and pathological characteristics were reviewed, immunohistochemical phenotyping of biopsies of ACC lesions were detected, and the related data were analyzed in combination with relevant references. Results： Patients were female, with mean age of （61±14） years. Six cases had lesions in left-breast and four cases in right, mostly in the upper outer quadrant. The main clinical manifestation was expansive growth, and the ACC tumor size ranged from 1 to 4 cm, and histologically resembled glandular, tubular and solid tumor. The tumor cells were immunohistochemically negative for ER, PR, HER-2 expression and positive for CD117 expression. Proliferation marker Ki67 ranged from 5%-30%. The median follow-up of these 9 cases ranged from 4-110 months. One patient died of multiple organ failure, and the other eight patients were alive and well with no evidence of disease. Conclusions： ACC of breast is a rare subtype of breast cancer and exhibits an indolent clinical behavior. ACC has its own special histologic characteristics, and differentiation with other breast benign and malignant tumor is needed.