原发性乳腺神经内分泌肿瘤（Br-NEN）很少见。近年来，该分类不断更新，使得对已发布数据的解释变得具有挑战性。目前尚不清楚神经内分泌分化是否与较差的预后相关以及应采用哪些治疗方法。对 2009 年至 2022 年在克拉科夫玛丽亚·斯克洛多夫斯卡·居里国家肿瘤研究所分院接受治疗的乳腺癌患者的数据库进行了探索，以寻找 Br- NEN。收集并分析患者的医学和病理数据。我们纳入了 22 名在诊断时患有 Br-NEN 且无转移的女性患者。中位年龄为64岁（范围：28-88），其中18例激素受体阳性，全部为HER-2阴性，中位Ki67为27%（10-100%）。诊断时的中位肿瘤大小为29.5mm（7-75mm），9名患者为N阳性。 5 例存在 DCIS。只有 1 例嗜铬粒蛋白和突触素染色呈阴性，但 4 例数据缺失。 9 名患者接受了辅助化疗，主要以蒽环类和紫杉类药物为主，16 名患者接受了辅助激素治疗，15 名患者接受了术后放疗。所有患者均接受了根治性手术，但其中两名患者接受了次优肿瘤切除术。一名患者局部复发，三名患者发生转移，均累及肺部，但这些患者仍然活着。中位随访时间为 96 个月 (8-153)。 2例患者死亡，随访时间>4年未复发。我们的结果与收集 Br-NEN 临床数据的 12 个病例系列进行了比较，患者人数中位数为 10.5（范围：3-142）。Br-NEN 代表一组异质疾病，缺乏前瞻性研究或临床试验的数据。目前尚无针对 Br-NEN 的既定治疗标准。我们的患者队列表现出良好的预后，这可能归因于与其他报道的病例系列相比较低的肿瘤分期和 Ki67 指数。我们建议根治性手术和术后放疗的实施类似于无特殊类型乳腺癌的标准治疗。 ESMO 也提倡在全身治疗中采用这种方法，但我们建议考虑对具有高 Ki67 的低分化 Br-NEN 患者进行铂类化疗。版权所有 © 2024 Püsküllüoğlu、Grela-Wojewoda、Ambicka、Pacholczak-Madej、Pietruszka、Mucha-Małecka 、Rudzińska、Ziobro、Ryś 和 Mituś。

Primary neuroendocrine neoplasms of the breast (Br-NENs) are rare. The classification has been updated in recent years making interpretation of the data published challenging. It is unclear whether neuroendocrine differentiation is associated with poorer prognosis and what treatment approaches should be applied.The database for breast cancer patients treated between 2009 and 2022 at the Maria Sklodowska-Curie National Research Institute of Oncology Branch Krakow was explored to search for Br-NENs. Patients' medical and pathological data were collected and analyzed.We included 22 females with Br-NEN without metastases at the time of diagnosis. The median age was 64 years (range: 28-88), Of the cases, 18 were hormone receptor positive, all were HER-2 negative, the median Ki67 was 27% (10-100%). The median tumor size at the time of diagnosis was 29.5mm (7-75mm), 9 patients were N-positive. DCIS was present in 5 cases. Only one case was negative for chromogranin and synaptophysin staining, but data were missing for 4 cases. Nine patients received adjuvant chemotherapy, mainly based on anthracyclines and taxanes, while 16 received adjuvant hormonal therapy and 15 received postoperative radiotherapy. Radical surgery was performed in all patients, but two underwent suboptimal tumorectomy. One patient had local recurrence, three experienced metastatic disease, all involving the lungs, but these patients are still alive. The median follow-up was 96 months (8-153). Two patients died, with a follow up time of no recurrence >4 years. Our results were compared to twelve case series collecting clinical data on Br-NENs, with median patient number of 10.5 (range: 3-142).Br-NENs represent a heterogenous group of diseases, lacking data from prospective studies or clinical trials. There are no established treatment standards tailored for Br-NENs. Our patients' cohort exhibited a favorable prognosis, potentially attributed to lower tumor stage and Ki67 index compared to other reported case series. We suggest that radical surgery and postoperative radiotherapy be administered akin to standard treatment for breast cancer of no special type. ESMO also advocates for this approach in systemic treatment, although we recommend considering platinum-based chemotherapy for patients with poorly differentiated Br-NENs exhibiting high Ki67.Copyright © 2024 Püsküllüoğlu, Grela-Wojewoda, Ambicka, Pacholczak-Madej, Pietruszka, Mucha-Małecka, Rudzińska, Ziobro, Ryś and Mituś.