Stanford Women's Cancer Center, Division of Gynecologic Oncology, Stanford University, Stanford, CA, 94305, U.S.A.
Br J Dermatol. 2014 Mar;170(3):661-71. doi: 10.1111/bjd.12635.
Extramammary Paget disease (EMPD) is often associated with underlying or distant synchronous malignancies. The prognosis for affected patients is generally favourable; however, the risk of secondary malignancies is unknown.
The goal of the study was to analyse the incidence, prognosis and pattern of secondary malignancies for patients with invasive EMPD using data from the Surveillance, Epidemiology and End Results (SEER) Program.
We searched the SEER Program database for patients diagnosed with invasive EMPD between 1973 and 2008. Demographic data, outcome and secondary malignancies more than 1 year after the initial diagnosis of invasive EMPD were included in the analysis. We calculated the standardized incidence ratio (SIR) and estimated the excess absolute risk (EAR) per 10,000 person-years (PY).
There were 1439 patients who were diagnosed with invasive EMPD. Most patients (80.4%) had localized disease, while 17.1% had locoregional spread and 2.5% presented with distant disease. The SIR for secondary malignancies in patients with invasive EMPD was significantly elevated with an EAR of 97.4 additional malignancies per 10,000 PY. The excess risk was mostly due to a significantly increased incidence of colorectal and anal malignancies. The initial site of disease predicted the site of the secondary malignancies, with patients with colorectal, anal, vulvar and scrotal disease showing an increased risk of colorectal, anal, vulvar and scrotal malignancies, respectively.
Our study identified a long-term increased risk of developing secondary malignancies in patients with invasive EMPD that are mainly related to the site of origin of this disease. Patients with invasive EMPD require prolonged follow-up and screening for these malignancies.
乳房外 Paget 病（EMPD）常与潜在或远处同步恶性肿瘤相关。受影响患者的预后通常较好；然而，继发性恶性肿瘤的风险尚不清楚。
本研究的目的是使用监测、流行病学和最终结果（SEER）计划的数据分析浸润性 EMPD 患者继发性恶性肿瘤的发病率、预后和模式。
我们在 1973 年至 2008 年间从 SEER 计划数据库中搜索诊断为浸润性 EMPD 的患者。分析包括人口统计学数据、结局以及浸润性 EMPD 初始诊断 1 年后的继发性恶性肿瘤。我们计算了标准化发病比（SIR）并估计了每 10000 人年（PY）的超额绝对风险（EAR）。
共有 1439 例患者被诊断为浸润性 EMPD。大多数患者（80.4%）患有局限性疾病，17.1%有局部区域扩散，2.5%有远处疾病。浸润性 EMPD 患者继发性恶性肿瘤的 SIR 显著升高，EAR 为每 10000PY 增加 97.4 例额外恶性肿瘤。这种超额风险主要是由于结直肠和肛门恶性肿瘤的发病率显著增加所致。疾病的初始部位预测了继发性恶性肿瘤的部位，患有结直肠、肛门、外阴和阴囊疾病的患者分别有更高的结直肠、肛门、外阴和阴囊恶性肿瘤的风险。
我们的研究发现，浸润性 EMPD 患者发生继发性恶性肿瘤的长期风险增加，主要与该疾病的起源部位有关。浸润性 EMPD 患者需要长期随访和筛查这些恶性肿瘤。
