Iacobone Anna Daniela, Guerrieri Maria Elena, Preti Eleonora Petra, Spolti Noemi, Radici Gianluigi, Peveri Giulia, Bagnardi Vincenzo, Tosti Giulio, Maggioni Angelo, Bottari Fabio, Scacchi Chiara, Ghioni Mariacristina
Preventive Gynecology Unit, European Institute of Oncology IRCCS, 20141 Milan, Italy.
Department of Biomedical Sciences, University of Sassari, 07100 Sassari, Italy.
Diagnostics (Basel). 2023 Jan 27;13(3):464. doi: 10.3390/diagnostics13030464.
Cervico-vaginal (CV) localization of extra-mammary Paget's disease (EMPD) of the vulva is extremely rare. In order to investigate the incidence risk and the pathognomonic clinical and pathological features of this condition, a retrospective analysis was conducted including 94 women treated for vulvar EMPD at the European Institute of Oncology, Milan, Italy, from October 1997 to May 2020. Overall nine patients developed CV involvement from EMPD, with a cumulative incidence of 2.5% (95% CI: 0.5-8.0%) at 5 years, 6.5% (95% CI: 1.9-15.1%) at 10 years and 14.0% (95% CI: 4.8-27.8%) at 15 years, respectively. All cases except one were firstly detected by abnormal glandular cytology. None reported vaginal bleeding or other suspicious symptoms. The colposcopic findings were heterogeneous and could sometimes be misdiagnosed. Cervical and/or vaginal biopsies were always performed for histopathological diagnosis by identification of Paget cells in the epithelium or stroma. Most patients developed invasive EMPD (5/9) of the cervix and/or vagina and underwent hysterectomy with partial or total colpectomy. CV involvement from EMPD should not be underestimated in women with a long-standing history of vulvar Paget's disease. Liquid-based cytology with immunocytochemistry represents a valuable tool for early diagnosis and should be routinely performed during the required lifelong follow-up.
外阴部乳房外佩吉特病（EMPD）的宫颈 - 阴道（CV）定位极为罕见。为了调查这种情况的发病风险以及特征性的临床和病理特征，我们进行了一项回顾性分析，纳入了1997年10月至2020年5月期间在意大利米兰欧洲肿瘤研究所接受外阴EMPD治疗的94名女性。总体而言，9名患者出现了EMPD累及CV，5年时的累积发病率为2.5%（95%CI：0.5 - 8.0%），10年时为6.5%（95%CI：1.9 - 15.1%），15年时为14.0%（95%CI：4.8 - 27.8%）。除1例之外，所有病例均首先通过异常腺细胞检查发现。无一例报告有阴道出血或其他可疑症状。阴道镜检查结果各异，有时可能会被误诊。总是通过在上皮或基质中识别佩吉特细胞进行宫颈和/或阴道活检以进行组织病理学诊断。大多数患者发展为宫颈和/或阴道浸润性EMPD（5/9），并接受了子宫切除术及部分或全阴道切除术。对于有长期外阴佩吉特病病史的女性，不应低估EMPD累及CV的情况。液基细胞学联合免疫细胞化学是早期诊断的有价值工具，应在所需的终身随访期间常规进行。
