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.

Iacobone, A., Guerrieri, M., Preti, E., Spolti, N., Radici, G., Peveri, G., et al. (2023). Tips and Tricks for Early Diagnosis of Cervico-Vaginal Involvement from Extramammary Paget’s Disease of the Vulva: A Referral Center Experience. DIAGNOSTICS, 13(3) [10.3390/diagnostics13030464].

Tips and Tricks for Early Diagnosis of Cervico-Vaginal Involvement from Extramammary Paget’s Disease of the Vulva: A Referral Center Experience

Bagnardi V.;
2023

Abstract

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.
Articolo in rivista - Articolo scientifico
atypical glandular cytology; cervico-vaginal involvement; colposcopic-guided biopsy; Paget cells; vulvar extramammary Paget’s disease (EMPD);
English
27-gen-2023
2023
13
3
464
none
Iacobone, A., Guerrieri, M., Preti, E., Spolti, N., Radici, G., Peveri, G., et al. (2023). Tips and Tricks for Early Diagnosis of Cervico-Vaginal Involvement from Extramammary Paget’s Disease of the Vulva: A Referral Center Experience. DIAGNOSTICS, 13(3) [10.3390/diagnostics13030464].
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10281/407600
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