Ann Ital Chir. 2020;91:23-26.
Breast neuroendocrine carcinomas constitute approximately 0.3-0.5% of all breast cancers. In this study, we aimed to evaluate the data of patients diagnosed with primary breast neuroendocrine carcinoma.
Patients with more than 50% neuroendocrine differentiation identified in the histopathological examination between January 2010 and January 2015 and who had no other focus on imaging were evaluated retrospectively from the hospital registry system. Patients with secondary neuroendocrine tumor of the breast and male patients were excluded from the study. All patients gave informed consent. Patients were staged according to TNM classification.
During the study period, 425 patients were operated for breast cancer. Eleven patients were included in the study. The mean age of the patients was 68 (range 49-86). Immunohistochemical examinations revealed positive staining with neuron-specific enolase, synaptophysin and chromogranin in all patients. Ten patients had strong positive estrogen and progesterone receptors and receptor status was not specified in one patient. Distant organ metastasis was detected in 1 patient during the follow-up period, no local recurrence and mortality were seen in any patient.
The most widely used specific markers of neuroendocrine differentiation are chromogranin and synaptophysin. There is no standard treatment protocol for primary breast neuroendocrine tumors. Most of the treatments reported in the literature and in this study are breast sparing surgery or mastectomy, followed by anthracycline and taxanebased chemotherapy and/or hormonotherapy, similar to the treatment of ductal carcinoma. The distinction of primary metastases in breast neuroendocrine tumors is important, so the presence of neuroendocrine tumors should be investigated in other organs. In this case the treatment is changed. The issue of how neuroendocrine differentiation affects clinical outcome is yet to be debated.
Chromogranin, Neuroendocrine tumor, Synaptophysin.
乳腺神经内分泌癌约占所有乳腺癌的 0.3-0.5%。本研究旨在评估诊断为原发性乳腺神经内分泌癌患者的数据。
从医院登记系统中回顾性评估 2010 年 1 月至 2015 年 1 月期间组织病理学检查中发现超过 50%神经内分泌分化的患者，且影像学无其他病灶。排除乳腺继发性神经内分泌肿瘤和男性患者。所有患者均签署知情同意书。患者根据 TNM 分期。
在研究期间，有 425 例患者接受了乳腺癌手术。11 例患者纳入研究。患者的平均年龄为 68 岁（范围 49-86 岁）。免疫组织化学检查显示所有患者神经元特异性烯醇化酶、突触素和嗜铬粒蛋白染色均为阳性。10 例患者强阳性雌激素和孕激素受体，1 例患者受体状态未明确。在随访期间，1 例患者发现远处器官转移，无局部复发和死亡。
神经内分泌分化最广泛使用的特异性标志物是嗜铬粒蛋白和突触素。原发性乳腺神经内分泌肿瘤尚无标准治疗方案。文献和本研究中报道的大多数治疗方法是保乳手术或乳房切除术，然后是蒽环类和紫杉类为基础的化疗和/或激素治疗，类似于导管癌的治疗。乳腺神经内分泌肿瘤中原发转移的鉴别很重要，因此应在其他器官中检查神经内分泌肿瘤的存在。在这种情况下，治疗方法会发生改变。神经内分泌分化如何影响临床结果的问题仍有待讨论。
嗜铬粒蛋白、神经内分泌肿瘤、突触素。
