: Neuroendocrine carcinoma of the breast is considered a rare entity, and for this reason there are no data from prospective clinical trials on its optimal management. Early stage tumors are usually treated with the same strategy used for the other types of invasive breast cancer. Anthracycline- and taxane-based regimens represent the most frequently administered chemotherapy in neoadjuvant and adjuvant setting, as well as for metastatic disease, although combinations of platinum compounds and etoposide have been widely used, in particular for small-cell histology and tumors with a high proliferation index. For metastatic disease, a multimodality therapeutic strategy can be considered on an individual basis, with chemotherapy, endocrine therapy, peptide receptor radionuclide therapy, radiation therapy, surgery, or a combination of the above. In the near future, a better knowledge of the biology of these tumors will hopefully provide new therapeutic targets for personalized treatment. In this review, we discuss the current evidence and the future perspectives on diagnosis and treatment of neuroendocrine carcinoma of the breast.
Implications for practice: Neuroendocrine carcinoma of the breast (NECB) is a distinct entity of breast cancer. Clinical features and morphology are not helpful to distinguish NECB from other subtypes of breast cancer; therefore, immunohistochemistry markers for neuroendocrine differentiation, mainly chromogranin and synaptophysin, should be routinely used to confirm the diagnosis, especially in cases of mucinous or solid papillary carcinoma in which the suspicion of NECB may be relevant. Adjuvant treatment should be offered according to the same recommendations given for the other types of invasive breast cancer. An accurate diagnosis of NECB is also important in the metastatic setting, in which a multimodality approach including specific therapies such as peptide receptor radionuclide therapy can be considered.
摘要
乳腺神经内分泌癌被认为是罕见疾病, 因此其最佳治疗缺乏相关的前瞻性临床试验数据。对于早期肿瘤, 常采用与其他类型的浸润性乳腺癌相同的治疗策略。以蒽环类和紫杉烷类为基础的方案是新辅助治疗和辅助治疗最常用的方案, 对于转移性肿瘤也是如此; 但在针对小细胞组织学和高增殖指数肿瘤进行治疗时, 已广泛使用联合铂类化合物和依托泊甙的方案。对于转移性疾病, 应基于个体化治疗原则考虑采用多学科治疗策略, 包括化疗、内分泌治疗、多肽受体放射性核素治疗、放射治疗、手术或联合使用以上方案。在不久的将来, 更深入地了解这些肿瘤的生物学将有望为个体化治疗提供更多治疗靶点。我们在本综述中讨论了乳腺神经内分泌癌诊断和治疗的现有证据和未来展望。The Oncologist 2016;21:28–32
对临床实践的提示: 乳腺神经内分泌癌 (NECB) 是一种独特的乳腺肿瘤。NECB 的临床特征和形态学均难以将其与其他乳腺癌亚型区分开来, 因此神经内分泌分化的免疫组化标记物 (主要是嗜铬粒蛋白和突触素) 应常规用于该病的确诊, 尤其是在怀疑可能与 NECB 相关的粘液性或实性乳头状癌中。辅助治疗应按照其他浸润性乳腺癌指南的建议实施。NECB 的准确诊断在转移性疾病中同样重要, 对于这类患者应考虑采用包括特异性治疗方案如多肽受体放射性核素治疗在内的多学科治疗方案。
Keywords: Carcinoid tumors; Diagnosis; Neuroendocrine breast carcinoma; Neuroendocrine differentiation; Small-cell breast cancer; Treatment.
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