Division of Gynecologic Oncology, Mayo Clinic, Rochester, MN, USA.
Division of Gynecologic Oncology, The George Washington University, Washington, DC, USA.
Gynecol Oncol. 2020 Jun;157(3):649-655. doi: 10.1016/j.ygyno.2020.03.018. Epub 2020 Apr 5.
To assess the incidence, treatment, and outcomes in patients with invasive vulvar extramammary Paget's disease (EMPD) in a national cohort of patients.
Patients from the Surveillance, Epidemiology and End Results (SEER) database with diagnoses of vulvar EMPD from 1992 to 2016 were included. Demographic, treatment, and outcome data were analyzed.
A total of 1268 cases of invasive EMPD were identified. Of those, 69.6% had localized disease, 12.0% regional disease, 1.3% distant disease, and 17.1% were unstaged. The annual incidence of invasive vulvar EMPD was 0.36 per 100,000 person years: rates have increased >2-fold since 1992 (1992: 0.19 per 100,000 person years to 0.50 per 100,000 person years in 2016). Most patients underwent primary surgery (n = 1034; 81.5%). Five-year cancer specific survival (CSS) was 95.5% and was associated with stage. Compared to patients with localized disease, patients with distant metastases had dramatically worse CSS (HR: 85.8 (31.8-248) p < 0.0001). Synchronous cancers (diagnosed within one calendar year of EMPD diagnosis year) were observed in 35 cases (2.8%), and 195 patients (15.4%) developed a secondary malignancy (diagnosed >one year from year of EMPD diagnosis year). The most common synchronous breast, gastrointestinal tract, melanoma and the most common secondary cancers were breast, gastrointestinal tract and genitourinary tract.
The incidence of invasive vulvar EMPD has increased over time. CSS is excellent for localized disease, but those with metastatic disease are in need of novel therapies. Approximately 15% will develop a secondary malignancy, indicating that patients with invasive vulvar EMPD should undergo site specific preventative health screens during recurrence surveillance.
评估全国患者队列中侵袭性外阴外胚层乳腺样肿瘤（EMPD）患者的发病率、治疗方法和结局。
纳入 1992 年至 2016 年期间诊断为外阴 EMPD 的监测、流行病学和最终结果（SEER）数据库中的患者。分析了人口统计学、治疗和结局数据。
共发现 1268 例侵袭性 EMPD 病例。其中，69.6%为局限性疾病，12.0%为区域性疾病，1.3%为远处转移，17.1%为未分期。侵袭性外阴 EMPD 的年发病率为每 10 万人 0.36 例：自 1992 年以来，发病率增加了两倍以上（1992 年：每 10 万人 0.19 例，至 2016 年每 10 万人 0.50 例）。大多数患者接受了原发性手术（n=1034；81.5%）。5 年癌症特异性生存率（CSS）为 95.5%，与分期有关。与局限性疾病患者相比，远处转移患者的 CSS 明显更差（HR：85.8（31.8-248），p<0.0001）。1268 例患者中有 35 例（2.8%）观察到同步癌症（在 EMPD 诊断年的一个日历年内诊断出），195 例（15.4%）发生继发性恶性肿瘤（在 EMPD 诊断年后一年以上诊断出）。最常见的同步性乳腺癌、胃肠道癌、黑色素瘤和最常见的继发性癌症是乳腺癌、胃肠道癌和泌尿生殖道癌。
侵袭性外阴 EMPD 的发病率随着时间的推移而增加。CSS 对局限性疾病效果极佳，但转移性疾病患者需要新的治疗方法。大约 15%的患者会发生继发性恶性肿瘤，这表明侵袭性外阴 EMPD 患者在复发监测期间应进行特定部位的预防性健康筛查。
