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目的:探讨乳腺神经内分泌癌(neuroendocrine breast cancer,NEBC)的临床特征和诊治经验。方法:对2004年1月—2010年12月北京协和医院乳腺外科连续收治的10例女性NEBC患者进行回顾性分析,患者的平均年龄为52岁,并进行了24～177个月的随访,中位随访时间为84个月。结果:临床上,NEBC多表现为乳腺肿物,偶为不可触及的乳腺病变(non-palpable breast lesion,NPBL)或乳头溢液。NEBC超声影像学多表现为不均质的低回声实性肿物,形态不规则,边界清晰;乳腺钼靶X线摄影检查结果与一般乳腺癌相比无明显差异。7例患者接受乳腺癌改良根治术,1例患者行保留乳头和乳晕的乳腺癌改良根治术,1例表现为乳头单孔溢液的患者接受了2次手术治疗,第1次为右乳病变导管及相关腺叶切除+超声引导下右乳肿物穿刺活检术,第2次手术为右乳单纯切除+前哨淋巴结活检术。NEBC的雌激素受体(estrogen receptor,ER)或孕激素受体(progesterone receptor,PR)阳性表达率较高,人类表皮生长因子受体2(human epidermal growth factor receptor 2,HER2)为(-)～(+),神经内分泌标志物嗜铬素A(chromgranin A,CgA)和突触素(synapsin,Syn)至少有一项表达为阳性。随访期间,2例患者出现远处转移,1例患者死亡。结论:NEBC不同于乳腺癌神经内分泌分化(breast cancer with neuroendocrine dierentiation,BC-NE)。国内NEBC患者年龄相对较轻,超声影像学具有特异性表现,手术治疗和综合治疗的疗效较好,尽管可发生复发或转移,但总体预后尚可。
Objective: To investigate the clinical features and diagnosis and treatment of neuroendocrine breast cancer (NEBC). Methods: A retrospective analysis was performed on 10 consecutive women with NEBC admitted to the Department of Breast Surgery, Peking Union Medical College Hospital from January 2004 to December 2010. The mean age of patients was 52 years and followed up for 24 to 177 months. The follow-up time was 84 months. Results: Clinically, NEBC mostly manifested as breast tumor, even non-palpable breast lesion (NPBL) or nipple discharge. NEBC ultrasound imaging showed heterogeneous low echo solid tumors, irregular shape, clear boundary; mammography mammography results compared with the general breast cancer no significant difference. Seven patients underwent modified radical mastectomy. One patient underwent modified radical mastectomy for breast cancer with preserved nipple and areola. One patient presented with nipple single well discharge underwent two surgeries and the first was right breast disease Duct and related lobectomy + ultrasound-guided right breast tumor biopsy, the second surgery for the right breast simple resection + sentinel lymph node biopsy. The positive expression rate of estrogen receptor (ER) or progesterone receptor (PR) in NEBC was higher than that in human epidermal growth factor receptor 2 (HER2) ~ (+), At least one of the neuroendocrine markers chromogranin A (CgA) and synapsin (Syn) was positive. During follow-up, two patients had distant metastases and one patient died. Conclusion: NEBC is different from breast cancer with neuroendocrine dierentiation (BC-NE). The age of NEBC patients in China is relatively mild, ultrasound imaging has a specific performance, surgical treatment and comprehensive treatment of better efficacy, despite the recurrence or metastasis, but the overall prognosis was acceptable.