Department of Gynecological Oncology, Fudan University Shanghai Cancer Center, Fudan University, No. 270 Dong'an Road, Xuhui District, Shanghai, 200032, China.
Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, 200032, China.
Arch Gynecol Obstet. 2021 Feb;303(2):541-546. doi: 10.1007/s00404-020-05803-z. Epub 2020 Sep 29.
This study aimed to evaluate the risk factors of recurrence and invasive disease in patients with extramammary Paget's disease of the vulva (EPDV).
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.
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).
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.
本研究旨在评估外阴派杰氏病（EPDV）患者复发和侵袭性疾病的危险因素。
我们对 2006 年 5 月至 2019 年 3 月期间在复旦大学附属肿瘤医院初诊为 EPDV 的患者进行了回顾性分析。
我们医院共初诊 38 例 EPDV 患者，其中 29 例为上皮内 EPDV，8 例为上皮内 EPDV 伴间质浸润，1 例为外阴腺癌。共有 8 例（21%）患者出现 12 次复发，其中 4 例复发 1 次，4 例复发 2 次。复发均为上皮内 EPDV，复发 9 次，上皮内 EPDV 伴侵袭性疾病复发 3 次。首次复发和第二次复发的间隔分别为 62.1（9-146）个月和 22（15-28）个月。EPDV 患者的原发性和复发性侵袭性疾病发生率分别为 23.7%（9/38）和 25%（3/12）。我们发现，存在侵袭性疾病与病史超过 10 年有关（p=0.02），与切缘阳性呈负相关（p=0.037），但与年龄（p=0.438）、复发（p=0.642）和病变直径（p=0.08）无关。
病史超过 10 年与侵袭性疾病相关，建议密切和长期随访，以确定需要进一步治疗的患者。
