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目的探讨乳腺小细胞神经内分泌癌的临床诊断和治疗。方法回顾性分析北京大学临床肿瘤学院2001年1月至2006年6月收治的3例乳腺小细胞神经内分泌癌的临床表现、病理、治疗及预后。结果3例乳腺小细胞神经内分泌癌均以自行发现乳腺肿物就诊。钼靶示病灶内无钙化。2例行保乳术,1例行改良根治术。病理检查具有小细胞癌的形态学特点,免疫组织化学染色结果突触素(Syn)(++)、嗜铬颗粒蛋白A(CgA)(++)3例,神经元特异性烯醇化酶(NSE)(+)1例,雌激素受体(ER)、孕激素受体(PR)和人表皮生长因子受体-2(HER-2)均为阴性。随访时间9~74个月,无淋巴结转移1例,术后无病存活74个月;淋巴结转移2例,术后1年内均出现全身多发转移,其中1例存活期15个月,1例术后9个月尚存活。结论乳腺小细胞神经内分泌癌十分罕见。无钙化,雌激素受体(ER)、孕激素受体(PR)和HER-2表达阴性可能是该病的临床特点。确诊需依靠病理形态学及免疫组织化学染色。目前治疗效果及预后差异较大,提示该病可能具有不同的亚型。
Objective To investigate the clinical diagnosis and treatment of breast small cell neuroendocrine carcinoma. Methods The clinical manifestations, pathology, treatment and prognosis of 3 cases of small cell neuroendocrine carcinoma of the breast cancer from January 2001 to June 2006 in Peking University were retrospectively analyzed. Results All three cases of small cell neuroendocrine carcinoma of breast showed self-discovered breast tumor. Molybdenum target lesions without calcification. 2 routine breast-conserving surgery, 1 case of modified radical mastectomy. The pathological examination showed the morphological features of small cell carcinoma. The immunohistochemical staining results showed synaptophysin (Syn) (++), chromogranin A (CgA) (++) in 3 cases, neuron specific enolase NSE) (+), negative for estrogen receptor (ER), progesterone receptor (PR) and human epidermal growth factor receptor-2 (HER-2) The follow-up time ranged from 9 to 74 months. There was no lymph node metastasis in 1 patient. No postoperative disease-free survival was found in 74 months. Lymph node metastasis in 2 patients showed systemic multiple metastasis within 1 year after operation. One case survived for 15 months and one patient Nine months after surviving. Conclusion Breast small cell neuroendocrine carcinoma is very rare. No calcification, estrogen receptor (ER), progesterone receptor (PR) and HER-2 negative expression may be the clinical features of the disease. Diagnosis depends on pathological morphology and immunohistochemical staining. The current treatment effect and prognosis vary greatly, suggesting that the disease may have different subtypes.