Vulvar Extramammary Paget Disease Detected by Cytology for Cervical Cancer Screening: A Case Report and Literature Review

22 October 2021: Articles

Challenging differential diagnosis, Rare disease

Kotaro Takahashi ^A* , Akira Kikuchi ^B , Ayano Horiuchi ^D , Ryo Tamura ^D , Kazufumi Haino ^D , Masayuki Yamaguchi ^D , Takashi Kawasaki ^B

DOI: 10.12659/AJCR.933655

Am J Case Rep 2021; 22:e933655

Table 1. Reported cases of primary or secondary extramammary Paget disease with abnormal cervical cytology.

Author	Patient age, y	Primary Lesion	Treatment history of primary lesion	Clinical presentation	Cervical cytology	Cervical histology
Costello et al []³	78	PEMPD	Yes	Itching of vulva and vagina	Adenocarcinoma	EMPD
Arnould et al []⁴	72	UC	Yes	Cervical cancer screening	Malignant urothelial cells	EMPD
Hendsch et al []⁵	53	UC	No	Cervical cancer screening	Adenocarcinoma	No cervical neoplasm in hysterectomy specimen
Gu et al []⁶	78	PEMPD	Yes	Cervical cancer screening	Adenocarcinoma	EMPD
Gu et al []⁶	87	PEMPD	No	Cervical cancer screening	SCC	EMPD
Reyes et al []⁷	58	UC	Yes	Cervical cancer screening	ASC-H	EMPD; She was initially diagnosed with HSIL/CIN3
Westacott et al []⁸	81	PEMPD	No	Vulvar eczema and itching	Abnormal cells	EMPD
Clayton et al []⁹	73	PEMPD	Yes	Postmenopausal bleeding	Abnormal cells	EMPD
Davis et al []¹⁰	66	PEMPD	Yes	Cervical cancer screening	SCC	EMPD
van der Linden et al []¹¹	66	CRC	No	Postmenopausal bleeding	Adenocarcinoma	EMPD
Koyanagi et al []¹²	68	UC	Yes	Postmenopausal bleeding	Adenocarcinoma	No cervical neoplasm in conization specimen
This case	77	UC	Yes	Cervical cancer screening	Adenocarcinoma	Atypical glandular cells
ASC-H – atypical squamous cells-cannot exclude high-grade squamous intraepithelial lesion; CRC – colorectal carcinoma; EMPD – extramammary Paget disease; HSIL/CIN3 – high-grade squamous intraepithelial lesion/cervical intraepithelial neoplasia 3; PEMPD – primary extramammary Paget disease; SCC – squamous cell carcinoma; UC – urothelial carcinoma.