乳腺分泌基质的癌影像学及临床病理特征观察

Radiographic and clinicopathologic observation of matrix-producing carcinoma of the breast

目的:探讨乳腺分泌基质的癌(matrix-producing carcinoma,MPC)影像学及临床病理特征。方法:回顾性分析12例乳腺MPC影像学表现、病理形态学特点、免疫表型、治疗及预后,并复习相关文献。结果:患者均为女性,年龄43~74岁,平均50岁。3例患者首诊时伴有同侧腋窝淋巴结转移。超声检查显示12例肿瘤均为低回声结节状病变伴后方回声增强效应,乳腺影像报告和数据系统(Breast Imaging Reporting and Data System,BI-RADS)分级范围3~4A级;MRI显示12例肿瘤T1WI呈环形增强,T2WI呈高信号,BI-RADS分级范围4C^5级。12例肿瘤均为单发,最大径1.5~9.0 cm,肉眼见灰白色结节状或多结节融合状肿块,界限相对较清,质地中等至硬,切面灰白、灰红,部分区域黏液半透明状;镜检显示浸润癌成分直接过度为黏液软骨样基质,无介于中间的梭形细胞肉瘤样化生区;结节边缘浸润癌成分均为浸润性导管癌。11例为雌激素受体(estrogen receptor,ER)(-)、孕激素受体(progesterone receptor,PR)(-)、人类表皮生长因子受体2(human epidermal growth factor receptor 2,HER-2)/neu(-)的三阴性乳腺癌(triple-negative breast cancer,TNBC);10例肿瘤细胞表达细胞角蛋白5/6(cytokeratin 5/6,CK5/6)和表皮生长因子受体(epidermal growth factor receptor,EGFR);所有患者肿瘤细胞均表达S-100。12例患者均获得完整随访资料,随访时间8~30个月。所有患者均生存至今,9例无瘤存活,1例术后16个月发生肺转移,1例术后8个月发生肺转移,1例术后11个月胸壁复发。结论:乳腺MPC是一类较罕见的特殊类型乳腺癌,其超声影像学特点与许多良性病变和肿瘤有所重叠,极易漏诊,而MRI诊断乳腺MPC有较高的灵敏度。乳腺MPC在病理形态学及免疫表型方面有显著的特征性表现。

Objective:To investigate the radiographic and clinicopathologic characteristics of matrix-producing carcinoma(MPC)of the breast.Methods:A total of12cases diagnosed as MPC of the breast were retrospectively analyzed for radiographic and clinicopathologic findings,immunophenotypes,treatment and prognosis,followed with review of the literatures.Results:The patients were all female,mean age50years(range43-74years).A relative well-circumscribed hypoechoic nodular lesion with posterior echo pattern was revealed by ultrasonography,with Breast Imaging Reporting and Data System(BI-RADS)categories3-4A.MRI revealed a low-intensity tumor with T1imaging and a high-intensity tumor with T2imaging,with BI-RADS categories4C-5.All patients were single mass.The size of tumors was1.5-9.0cm.Macroscopically,the tumor showed relatively well-circumscribed pale nodular lesion,with mucilaginous edge in some area.On microscopic evaluation,all the invasive components showed an abrupt transition to chondromyxoid matrix without an intervening spindle cell sarcomatoid component.The overt invasive carcinoma components were all invasive ductal carcinoma.On immnuohistochemical evaluation,11cases were triple-negative breast cancer(TNBC).Ten cases were positive for cytokeratin5/6(CK5/6)and epidermal growth factor receptor(EGFR).All cases were positive for S-100protein.Follow-up information of all cases were obtained.Follow-up time ranged8-30months.At the time of initial diagnosis,3patients had axillary lymph node metastasis.All patients were surviving.Nine patients were surviving with disease free,one patient had lung metastasis16months after surgery,one patient had lung metastasis8months after surgery,and one patient had recurrence in thoracic wall11months after surgery.Conclusion:MPC of the breast is an extremely rare variant carcinoma of the breast,and its ultrasonographic features were similar to many lesions or tumors,always leading to missed diagnosis.MRI had relatively higher sensitivity in the diagnosis of MPC of the breast.Thorough understanding of the pathological features of this disease and usage of immunochemical panel could make the patients benefit from the subsequent treatment.