Han Bo-Yue, Xu Xiao-Li, Zhu Xiu-Zhi, Han Xiang-Chen, Hu Xin, Ling Hong
Department of Breast Surgery, Fudan University Shanghai Cancer Center, Fudan University, Shanghai, 200032, People's Republic of China.
Key Laboratory of Breast Cancer in Shanghai, Fudan University Shanghai Cancer Center, Fudan University, Shanghai, 200032, People's Republic of China.
Cancer Manag Res. 2022 Jan 18;14:237-247. doi: 10.2147/CMAR.S338788. eCollection 2022.
Mammary Paget's disease (PD) is a rare type of breast cancer. Most cases of PD are presented with underlying ductal carcinoma in situ (DCIS) or invasive breast carcinoma (IDC). This study aimed to investigate the clinicopathological characteristics and survival outcomes of PD patients.
A total of 406 patients diagnosed with PD with IDC/DCIS at Fudan University Shanghai Cancer Center (FUSCC) were recruited as the PD group, 1218 patients diagnosed with IDC/DCIS alone during the same period were selected as the non-PD group, and the clinicopathological results of these two groups were compared. The Surveillance, Epidemiology, and End Results (SEER) database was used to investigate the clinicopathological features between PD and non-PD patients for validation.
Compared with the non-PD group, the PD group was much more likely to have larger (≥2 cm: 43.1% vs 35.5%, P < 0.001), less hormone receptor (HR)-positive (68.5% vs 26.6%, P < 0.001), more human epidermal growth factor receptor-2 (HER-2)-positive (70.7% vs 27.5%, P < 0.001) and higher Ki-67 proportion (51.5% vs 42.5%, P < 0.001) tumors. The HER-2 overexpression subtype accounted for the largest proportion in the PD-IDC group and the lowest proportion in the non-PD-IDC group (54% vs 8%, P < 0.01). Moreover, the PD group had significantly worse disease-free survival (DFS) than the non-PD group (5-year DFS: 91.8% vs 97.3%, P = 0.001), and the SEER database showed a similar trend. Univariate and multivariate Cox regression analyses demonstrated that PD was an independent poor-risk factor. Our matched study showed that the PD group had worse survival than the non-PD group after excluding age, HR, HER-2, tumor size and lymph node status.
PD with IDC/DCIS is associated with more aggressive tumor characteristics and worse survival outcomes. More than half of PD breast cancers are HER-2 overexpression subtype. PD is an independent poor-risk factor for breast cancer survival.
乳腺佩吉特病（PD）是一种罕见的乳腺癌类型。大多数PD病例伴有潜在的原位导管癌（DCIS）或浸润性乳腺癌（IDC）。本研究旨在探讨PD患者的临床病理特征和生存结局。
复旦大学附属肿瘤医院共招募了406例诊断为伴有IDC/DCIS的PD患者作为PD组，同期选取1218例仅诊断为IDC/DCIS的患者作为非PD组，比较两组的临床病理结果。利用监测、流行病学和最终结果（SEER）数据库调查PD和非PD患者之间的临床病理特征以进行验证。
与非PD组相比，PD组更易出现更大（≥2 cm：43.1% 对35.5%，P < 0.001）、激素受体（HR）阳性比例更低（68.5% 对26.6%，P < 0.001）、人表皮生长因子受体2（HER-2）阳性比例更高（70.7% 对27.5%，P < 0.001）以及Ki-67比例更高（51.5% 对42.5%，P < 0.001）的肿瘤。HER-2过表达亚型在PD-IDC组中占比最大，在非PD-IDC组中占比最低（54% 对8%，P < 0.01）。此外，PD组的无病生存期（DFS）明显差于非PD组（5年DFS：91.8% 对97.3%，P = 0.001），SEER数据库显示了类似趋势。单因素和多因素Cox回归分析表明，PD是一个独立的不良风险因素。我们的配对研究表明，在排除年龄、HR、HER-2、肿瘤大小和淋巴结状态后，PD组的生存率低于非PD组。
伴有IDC/DCIS的PD与更具侵袭性的肿瘤特征和更差的生存结局相关。超过一半的PD乳腺癌为HER-2过表达亚型。PD是乳腺癌生存的一个独立不良风险因素。
