Increased risk and pattern of secondary malignancies in patients with invasive extramammary Paget disease
Increased risk and pattern of secondary malignancies in patients with invasive extramammary Paget disease
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DOI：
10.1111/bjd.12635
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发表时间：
2014-03-01
影响因子：
10.3
通讯作者：
Dorigo, O.
中科院分区：
文献类型：
--
作者：
Karam, A.;Dorigo, O.
背景乳腺外佩吉特病（EMPD）常与潜在或远处的同步性恶性肿瘤相关。受影响患者的预后一般良好;然而，继发性恶性肿瘤的风险是unknown.ObjectivesThe研究的目的是分析侵袭性EMPD患者的发病率，预后和继发性恶性肿瘤的模式，使用来自监测，流行病学和最终结果（SEER）Program.MethodsWe搜索SEER程序数据库的患者诊断为侵袭性EMPD在1973年和2008年之间。人口统计学资料，结果和继发性恶性肿瘤超过1年后，初步诊断为侵袭性EMPD纳入分析。我们计算了标准化发病率比（SIR），并估计了每10 000人年（PY）的超额绝对风险（RR）。大多数患者（80.4%）有局限性疾病，而17.1%有局部区域扩散，2.5%有远处疾病。侵袭性EMPD患者继发性恶性肿瘤的SIR显著升高，每10 000 PY额外恶性肿瘤的SIR为97.4。过多的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。疾病的初始部位预测继发性恶性肿瘤的部位，结直肠，肛门，外阴和阴囊疾病患者显示结直肠，肛门，外阴和阴囊恶性肿瘤的风险增加，respectively.ConclusionsOur 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.侵袭性EMPD患者需要长期随访和筛查这些恶性肿瘤。
BackgroundExtramammary 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.ObjectivesThe 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.MethodsWe 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).ResultsThere 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.ConclusionsOur 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.