目的:探讨乳腺神经内分泌癌(neuroendocrinebreastcancer,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-palpablebreastlesion,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(estrogenreceptor,ER)或孕激素受体(progesteronereceptor,PR)阳性表达率较高,人类表皮生长因子受体2(humanepidermalgrowthfactorreceptor2,HER2)为(-)~(+),神经内分泌标志物嗜铬素A(chromgraninA,CgA)和突触素(synapsin,Syn)至少有-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(breastcancerwithneuroendocrinedifferentiation,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 characteristics of clinical manifestations, diagnosis and treatment of NEBC (neuroendocrine breast cancer). Methods: This was a retrospective study. Ten consecutive female patients with NEBC were admitted in Department of Breast Surgery, Peking Union Medical College Hospital between January 2004 and December 2010. The average age of the ten patients was 52 years, and the follow-up period was 24-1 77 months (median 84 months). Results: Clinically, most of the NEBC manifested as breast lump, sometimes as NPBL (non-palpable breast lesion) or nipple discharge. An ultrasound examination usually revealed heterogeneous echoic mass with irregular shape and clear boundary. Mammography showed no significantly different changes from other kinds of breast cancer. Nine patients received curative resection for NEBC, among whom, seven underwent modified radical mastectomy, one underwent nipple and areola-sparing modified radical mastectomy, one with nipple discharge underwent excision of duct and relevant Iobule and hook-wire-guided biopsy of breast lump, and later right mastectomy with sentinel lymph node biopsy. The expressions of hormone receptors like ER (estrogen receptor) /PR (progesterone receptor) were usually positive in NEBC, while theexpression of HER2 (human epidermal growth factor receptor 2) was usually negative (-) to positive (+). At least one neuroendocrine marker like Syn (synapsin) or CgA (chromgranin A) was positive. During the follow-up period, metastasis was found in two patients and one of them had passed away. Conclusion: NEBC is a clinical entity different from breast cancer with BC-NE (breast cancer with neuroendocrine differentiation). NEBC may affect younger patients in China, ultrasound examination is valuable for diagnosis, and surgical and comprehensive therapy may achieve satisfactory outcome with relatively good prognosis, although there might be cases of metastasis.