Borghi Chiara, Bogani Giorgio, Ditto Antonino, Martinelli Fabio, Signorelli Mauro, Chiappa Valentina, Scaffa Cono, Perotto Stefania, Leone Roberti Maggiore Umberto, Recalcati Dario, Lorusso Domenica, Raspagliesi Francesco
Int J Gynecol Cancer. 2018 Jan;28(1):176-182. doi: 10.1097/IGC.0000000000001131.
Extramammary Paget disease of the vulva (EPDV) is a rare occurrence with an indolent and relapsing course. Progression to invasion occurs in 4% to 19% of cases. The aim of this study is to report clinical-pathological features and outcomes of patients treated for invasive EPDV.
Data of consecutive patients treated between 2000 and 2017 for invasive EPDV were reviewed.
Among 79 patients with EPDV, 10 (12.7%) presented a microinvasive or invasive form at first diagnosis or during follow-up. All of them underwent upfront radical surgery; 7 (70%) received subsequent radiotherapy, chemotherapy, or both. The mortality rate was 40%. The recurrence rate after treatment for invasive forms was 60%, with a mean time to first recurrence of 20 (range, 5-36) months.
Our study confirms that invasive EPDV remains a rare gynecological neoplasm with a poor prognosis. Multicentre trials or well-organized prospective data collection could improve the knowledge about the management of invasive EPDV.
外阴部乳腺外佩吉特病（EPDV）较为罕见，病程呈惰性且易复发。4%至19%的病例会进展为浸润性病变。本研究旨在报告浸润性EPDV患者的临床病理特征及治疗结果。
回顾了2000年至2017年间接受治疗的连续性浸润性EPDV患者的数据。
在79例EPDV患者中，10例（12.7%）在初次诊断时或随访期间表现为微浸润或浸润性病变。所有患者均接受了 upfront 根治性手术；7例（70%）接受了后续放疗、化疗或两者联合治疗。死亡率为40%。浸润性病变治疗后的复发率为60%，首次复发的平均时间为20（范围5 - 36）个月。
我们的研究证实，浸润性EPDV仍然是一种罕见的妇科肿瘤，预后较差。多中心试验或组织良好的前瞻性数据收集可能会提高对浸润性EPDV治疗的认识。
