Comprehensive Cancer Centre The Netherlands, Department of Research and Registration, Utrecht, The Netherlands.
Eur J Surg Oncol. 2012 Mar;38(3):214-21. doi: 10.1016/j.ejso.2011.12.008. Epub 2012 Jan 14.
The aim of this study was to determine the incidence and survival of Extramammary Paget's disease (EMPD) and to describe the possible increased risk of tumours after EMPD. All invasive cases diagnosed between 1990 and 2002 were selected from the RARECARE database. Incidence was expressed in European standardized rates. Relative survival was calculated for the period 1995-1999, with a follow-up until 31st December 2003. Standardized incidence ratios of second primary tumours were calculated to reveal possible increased risk after EMPD. European age standardized Incidence of EMPD within Europe is 0.6 per 1000,000 person years. Five-year relative survival for invasive EMPD was 91.2% (95%CI; 83.5-95.4), 8.6 percent of the EMPD patients developed other malignancies. The highest increased risk of developing a second primary tumour was found in the first year of follow-up (SIR:2.0 95%CI; 1.3-2.9), living in the South European region (SIR:2.3 95%CI; 1.5-3.5) or being female (SIR:1.5 95%CI; 1.1-1.9). Female genital organs displayed greatest increased risk of developing a second primary tumour after EMPD (SIR:15,1 95%CI; 0.38-84.23). Due to the increased risk of a second primary tumour after EMPD a thorough search for other tumours during their follow-up is recommended.
本研究旨在确定乳腺外派杰氏病（EMPD）的发病率和生存率，并描述 EMPD 后可能增加的肿瘤风险。从 RARECARE 数据库中选择了 1990 年至 2002 年间诊断的所有浸润性病例。发病率以欧洲标准化率表示。1995-1999 年期间计算相对生存率，随访至 2003 年 12 月 31 日。计算第二原发肿瘤的标准化发病比，以揭示 EMPD 后可能增加的风险。欧洲 EMPD 的年龄标准化发病率为每 100 万人中有 0.6 人。浸润性 EMPD 的 5 年相对生存率为 91.2%（95%CI；83.5-95.4），8.6%的 EMPD 患者发生了其他恶性肿瘤。在随访的第一年发现第二原发肿瘤的风险最高（SIR：2.0 95%CI；1.3-2.9），居住在南欧地区（SIR：2.3 95%CI；1.5-3.5）或女性（SIR：1.5 95%CI；1.1-1.9）。女性生殖器官发生第二原发肿瘤的风险最高（SIR：15.1 95%CI；0.38-84.23）。由于 EMPD 后发生第二原发肿瘤的风险增加，建议在随访期间彻底检查其他肿瘤。
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