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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 clinicopathological features of patients with adenoid cystic carcinoma (ACC) so as to improve physicians’ understanding and diagnosis of the disease. Methods: The clinical data of 10 patients with breast adenocarcinoma were collected, their pathological features and immunohistochemical (immunohistochemical) performance were analyzed and analyzed. Results: All 10 breast adenocarcinomas were female, with an average age of (61 ± 14) years. The tumors in 6 patients were located in the left breast, 4 in the right breast, and more often in the upper quadrant. The main clinical manifestations were swollen growth mass; the largest diameter of the tumor was 1 to 4 cm. The pathological examination showed that the tumor cells arranged in a tubular shape - trabecular, sieve and solid type, the results of immunohistochemistry did not express estrogen receptor, progesterone receptor and proto-oncogene human epidermal growth factor receptor 2 (human epidermal growth factor receptor 2 and HER-2, while the expression of tyrosine kinase receptor (CD117) to varying degrees. The positive index of cell proliferation index (Ki-67) was 5% to 30%. Nine of 10 patients were followed up for a median follow-up of 4 to 110 months. One patient died of multiple organ failure and the other 8 patients survived well. Conclusion: Breast ACC is a rare malignant tumor with biologically inert behavior. Its pathological morphology is unique and needs to be differentiated from other benign and malignant breast lesions.