Objective: The aim of this study was to explore the clinicopathologic characteristics and prognostic factors of Paget's disease. Methods: 137 patients with Paget's disease of the breast who were diagnosed in Cancer Hospital Chinese Academy of Medical Sciences between January 2007 and May 2016 were identified and included in the study, including 134 females and 3 males. Results: The average age at onset of the disease was 51.8 years(range, 27-78 years). Among the 137 patients, 7 cases were Paget's disease without any other underlying malignancy, who were alive during the follow-up period. The other 130 patients were diagnosed with Paget's disease and underlying ductal carcinoma in situ (DCIS) or (and) with an invasive carcinoma. The positive expression rates of estrogen receptor (ER), progesterone receptor (PR) and human epidermal growth factor receptor 2 (HER-2) were 29.2%(40/137), 38.0%(52/137)and 78.1%(107/137), respectively. The two-year and five-year overall survival were 99.0%, 96.0%, and disease-free survival of two-year and five-year were 97.6%, 92.8%, respectively for all 137 patients. On survival analysis, longer course of disease, HER-2 positivity, tumor stage(T1-T4), and axillary lymph node metastasis were significantly associated with overall survival. Conclusions: Paget's disease of the breast is a slowly progressive malignancy with good prognosis. A longer course of disease, HER-2 positivity, tumor stage, axillary lymph node metastasis and distant metastasis were significantly associated with poor prognosis.
目的： 探讨乳腺派杰氏病的临床病理特征及预后影响因素。 方法： 回顾性分析2007年1月至2016年5月中国医学科学院肿瘤医院收治的137例乳腺派杰氏病患者的临床资料，其中男3例，女134例。采用Kaplan－Meier法和Log rank检验分析患者的预后及其影响因素。 结果： 137例患者的发病年龄为27~78岁，平均为51.8岁。137例患者中，仅7例为单纯乳腺派杰氏病患者，7例患者全部生存；派杰氏病合并原位癌和(或)浸润性癌130例。雌激素受体、孕激素受体和人表皮生长因子受体2(HER－2)的阳性率分别为29.2%(40/137)、38.0%(52/137)和78.1%(107/137)。全组患者的2、5年总生存率分别为99.0%和96.0%，2、5年无病生存率分别为97.6%和92.8%。生存分析显示，病程较长、腋窝淋巴结转移、HER－2阳性和肿瘤T分期较晚与乳腺派杰氏病患者的预后均有关(均P<0.05)。 结论： 乳腺派杰氏病病程较长，发展缓慢，患者的预后较好。病程较长、腋窝淋巴结转移、HER－2阳性、肿瘤T分期较晚和远处转移与患者的不良预后有关。.
Keywords: Paget′s disease, Mammary; Pathology, Clinical; Prognosis; Therapy.