原发性乳腺神经内分泌肿瘤的临床特征及预后分析

林伟+朱有志+陈祥锦

[摘要] 目的 对原发性乳腺神经内分泌肿瘤患者的临床特征、治疗及随访资料进行分析，提供更多的管理这种特殊类型乳腺癌的依据。 方法 回顾性分析2007年3月—2017年3月共36例病理确诊为原发性乳腺神经内分泌肿瘤的病例资料，进行电话随访。 结果 36例患者中位年龄为 55 岁，30例行乳腺癌改良根治术，6例行乳腺癌单纯乳房切除+腋窝前哨淋巴结活检术，其中18例进行了术后化疗，3例接受放疗，有30例接受了内分泌治疗。有2例在随访期间死亡。 结论 原发性乳腺神经内分泌肿瘤的临床表现无特异性，应对其进行长期随访从而进一步总结出该类罕见疾病的诊治特点及预后。

[关键词] 乳腺神经内分泌肿瘤；临床特征；随访

[中图分类号] R5 [文献标识码] A [文章编号] 1674-0742（2017）12（c）-0074-03

[Abstract] Objective This paper tries to analyze the clinical characteristics， treatments and follow-up datas of patients with primary breast neuroendocrine carcinomas， providing more evidence for the management of this particular type of breast cancer. Methods The 36 medical records of primary breast neuroendocrine carcinomas diagnosed by pathology were analyzed from March 2007 to March 2017， and the clinical datas were followed up by telephone. Results The median age of 36 patients was 55 years old. 30 underwent modified radical mastectomy， and 6 underwent simple mastectomy plus sentinel lymph node biopsy， and 18 received adjuvant chemotherapy， 3 received radiotherapy， and 30 received endocrine therapy. There were 2 deaths during the follow-up period. Conclusion The clinical manifestations of primary breast neuroendocrine carcinomas are nonspecific and long-term follow-up should be performed to further summarize the diagnosis， treatments and prognosis of the rare disease.

[Key words] Breast neuroendocrine carcinomas； Clinical features； Follow-up

神经内分泌肿瘤（neuroendocrine neoplasm，NEN）是一组起源于肽能神经元和神经内分泌细胞的异质性肿瘤，它可发生于包括胃、肠、肺、乳腺等全身各个器官组织[1]。其中，原发性乳腺神经内分泌肿瘤（neuroendocrine breast carcinomas，NEBC）发病率约占全部乳腺恶性肿瘤的 1%[2]。1963 年，Feyrter等[3]首先描述了一种具有和胃肠道及肺神经内分泌肿瘤相似的形态学特征的乳腺癌，并以具有神经内分泌特征的浸润性乳腺癌来将其命名为[3]。世界卫生组织（World Health Organization，WHO）于 2003 年对原发性 NEBC 进行了定义[4]。2012 年，WHO对 NEBC 重新定义为伴神经内分泌分化的癌具有与胃肠道和肺神经内分泌肿瘤类似的形态学特征。所有肿瘤均不同程度地表达神经内分泌标记。将 NEBC 为 3 个亚型，包括：高分化的乳腺神经内分泌肿瘤，低分化的乳腺神经内分泌癌及小细胞癌，和伴有神经内分泌分化的浸润性乳腺癌[5]。……