Clinical characteristics and risk factors of invasion in extramammary Paget's disease of the vulva
Clinical characteristics and risk factors of invasion in extramammary Paget's disease of the vulva
复制标题
乳外外阴Paget病的临床特点及侵袭危险因素
DOI：
10.1007/s00404-020-05803-z
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发表时间：
2020-09-29
影响因子：
2.6
通讯作者：
Ren, Yulan
中科院分区：
文献类型：
--
作者：
Liang, Shanhui;Wang, Juan;Ren, Yulan
目的探讨外阴乳房外Paget病(EPDV)复发和侵袭性疾病的危险因素。方法对2006年5月至2019年3月复旦大学上海肿瘤中心初诊为EPDV的患者进行回顾性分析。结果本院初诊EPDV患者38例。其中29例为上皮内型EPDV，8例为上皮内型EPDV伴间质侵犯，1例为外阴腺癌。总共有8例(21%)患者有12次复发。在这八名患者中，四名患者有一次复发，另外四名患者有两次复发。表皮内EPDV复发9次，侵袭性疾病表皮内EPDV复发3次。首次复发间隔62.1个月(9~146个月)，第二次复发间隔22个月(15~28个月)。初发EPDV侵袭性病变发生率为23.7%(9/38)，复发性EPDV侵袭性病变发生率为25%(3/12)。我们确定侵袭性疾病的存在与10年以上的病史有关(p=0.02)，与阳性切缘呈负相关(p=0.037)，而侵袭性疾病的存在与年龄(p=0.438)、复发(p=0.642)和病灶直径(p=0.08)没有统计学关系。结论有10年以上病史的EPDV与侵袭性疾病有关。建议进行密切和长期的随访，以确定哪些人需要进一步治疗。
Purpose This study aimed to evaluate the risk factors of recurrence and invasive disease in patients with extramammary Paget's disease of the vulva (EPDV). Methods We performed a retrospective analysis of patients who were initially diagnosed with EPDV in Fudan University Shanghai Cancer Center between May 2006 and March 2019. Results Thirty-eight patients were initially diagnosed with EPDV in our institution. Among them, 29 had intraepithelial EPDV, 8 had intraepithelial EPDV with stromal invasion, and 1 had an underlying vulvar adenocarcinoma. In total, 8 (21%) patients had 12 recurrences. Of these eight patients, four had one recurrence, while other four had two recurrences. Intraepidermal EPDV recurred nine times, while intraepidermal EPDV with invasive disease recurred thrice. The first and second recurrence intervals were 62.1 (9-146) months and 22 (15-28) months, respectively. The rate of invasive disease was 23.7% (9/38) for primary EPDV and 25% (3/12) for recurrent ones. We determined that the presence of invasive disease was associated with a history of more than 10 years (p = 0.02) and inversely correlated with positive margins (p = 0.037), However, invasive disease had no statistical relations with age (p = 0.438), recurrence (p = 0.642), and lesion diameter (p = 0.08). Conclusions EPDV with a history of more than 10 years was associated with invasive disease. Close and long-term follow-up are recommended to identify those who require further treatment.