Departments of Pathology, Unit 85, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX, 77030, USA.
Department of Pathology, West China Hospital, Sichuan University, Chengdu, China.
Breast Cancer Res Treat. 2023 Nov;202(1):23-32. doi: 10.1007/s10549-023-07052-5. Epub 2023 Aug 11.
Neuroendocrine neoplasms (NENs) of the breast are rare and not well-studied. NEN are subcategorized as well-differentiated neuroendocrine tumor (NET) and poorly differentiated neuroendocrine carcinoma (NEC). The objectives of the current study were to review the clinicopathologic features of NENs, therapeutic efficacy of current systemic therapy and clinical outcomes of NEN of the breast.
Between 2004 and 2015, 420 NET, 205 NEC, 146 Adenocarcinoma with NE differentiation (ACNED) and 1,479,520 of invasive carcinoma, not otherwise specified (IC-NOS) of the breast were identified in the National Caner Database. Overall survival was compared among groups using Kaplan-Meier method and Log-rank test. Multivariate analyses were performed to identify prognostic factors.
After adjusting for other prognostic factors, both NET and NEC of the breast showed significantly worse OS than IC-NOS (HR (95% CI) = 1.41 (1.17, 1.72), p = 0.005 and HR (95% CI) = 2.11 (1.67, 2.67), p < 0.001, respectively). Both NET and NEC benefited from endocrine therapy if the tumors were hormonal receptor positive (median OS for treated with vs without: 125 vs 57 months in NET, not reached vs 29 months in NEC). NEC also benefited from chemotherapy (median OS for treated with vs without: 42 vs 34 months), but not NET.
NEN is a unique pathologic and clinical entity, which has worse clinical outcome compared to IC-NOS of the breast. Current therapeutics used in the treatment of IC-NOS improve, but do not fully mitigate, the poorer prognosis of NEN patients. More effective therapy for patients with this unique tumor type are needed.
乳腺神经内分泌肿瘤（NEN）较为罕见，研究也尚不充分。神经内分泌肿瘤分为分化良好的神经内分泌肿瘤（NET）和分化差的神经内分泌癌（NEC）。本研究旨在回顾 NEN 的临床病理特征、当前全身治疗的疗效以及乳腺 NEN 的临床结局。
在 2004 年至 2015 年间，国家癌症数据库中鉴定出 420 例 NET、205 例 NEC、146 例腺鳞癌伴神经内分泌分化（ACNED）和 1479520 例非特指浸润性癌（IC-NOS）。采用 Kaplan-Meier 法和 Log-rank 检验比较各组的总生存期。采用多因素分析确定预后因素。
在调整其他预后因素后，乳腺 NET 和 NEC 的 OS 均显著差于 IC-NOS（HR（95%CI）=1.41（1.17，1.72），p=0.005 和 HR（95%CI）=2.11（1.67，2.67），p<0.001）。如果肿瘤为激素受体阳性，NET 和 NEC 均从内分泌治疗中获益（治疗与未治疗的中位 OS：NET 为 125 比 57 个月，NEC 为未达到比 29 个月）。NEC 也从化疗中获益（治疗与未治疗的中位 OS：42 比 34 个月），但 NET 没有获益。
NEN 是一种独特的病理和临床实体，其临床结局较乳腺 IC-NOS 差。目前用于治疗 IC-NOS 的治疗方法虽然有所改善，但并不能完全缓解 NEN 患者的不良预后。需要为这种独特肿瘤类型的患者提供更有效的治疗方法。
