**1. Introduction**

Extramammary Paget's disease (EMPD) of the vulva is a rare vulvar neoplasia with an unclear pathophysiology that usually occurs in the apocrine gland-rich skin of postmenopausal Caucasian women [1]. Vulvar EMPD predominantly manifests as an intraepithelial tumor (primary EMPD) but can also appear with stromal invasion or in association with an underlying lower genital tract or distant adenocarcinoma (secondary EMPD) [2].

The clinical presentation is various and includes erythematous, scaly or eczematous plaque on the vulva and perineum with occasional erosions or ulcerations, hypopigmentation and nodules. Itching and burning pain are the most common symptoms. Due to the overlap of signs and symptoms with other vulvar diseases, the diagnosis is confirmed by

**Citation:** Iacobone, A.D.; Guerrieri, M.E.; Preti, E.P.; Spolti, N.; Radici, G.; Peveri, G.; Bagnardi, V.; Tosti, G.; Maggioni, A.; Bottari, F.; et al. Tips and Tricks for Early Diagnosis of Cervico-Vaginal Involvement from Extramammary Paget's Disease of the Vulva: A Referral Center Experience. *Diagnostics* **2023**, *13*, 464. https://doi.org/10.3390/ diagnostics13030464

Academic Editor: Edward J. Pavlik

Received: 20 October 2022 Revised: 24 January 2023 Accepted: 25 January 2023 Published: 27 January 2023

**Copyright:** © 2023 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https:// creativecommons.org/licenses/by/ 4.0/).

histological assessment on punch or excision biopsy. It is also well known that EMPD often appears as multifocal and/or with the histological extent of the disease far beyond the visible macroscopic lesion [3]. Moreover, despite surgical excision, local recurrence has been reported in up to 73% of cases and negative resection margins cannot ensure relapse-free survival [4]. On the contrary, Matsuo et al. recently showed that positive surgical margins are significantly associated with an increased risk of local but not distant recurrence [5]. Nevertheless, alternative therapeutic regimes have been advocated over time, including laser excision and ablation, topical therapy with imiquimod, photodynamic therapy and radiotherapy, since multiple surgical instances for recurrences lead to the destruction of vulvar anatomy with psychosocial consequences.

The cervical and vaginal localization of EMPD has only been described in the case reports as an extremely rare extension of recurrent vulvar EMPD and was firstly described in 1988 by Costello et al. [6].

While investigations based on age and anatomical site to distinguish between primary and secondary EMPD are well established, little is known about how to early diagnose cervico-vaginal (CV) localization of EMPD.

The objective of the present study was to investigate the incidence risk of CV involvement from EMPD of the vulva in women referred to a tertiary cancer center and to identify the pathognomonic clinical and pathological features of this rare evolution of vulvar EMPD.
