Cervicovaginal cytologic features in patients with Vulvar Paget disease

INTRODUCTION: Extramammary Paget disease (EMPD) is an uncommon intraepithelial neoplasm which typically involves the vulva or perineal skin. Documented involvement of the cervix or vagina is infrequent, but Paget disease involving cervical cytology (Pap test) has been rarely reported. We examined Pap tests from patients with known EMPD to better understand the frequency of involvement as well as to characterize the distinctive cytologic features. MATERIALS AND METHODS: Cases were identified via natural language search of our pathology LIS for biopsy/resection diagnoses of vulvar or vaginal EMPD at our institution over a 10-year period. Patient records were searched for concurrent or near metachronous Pap tests. Pap tests were retrospectively reviewed to identify cytologic features suspicious for involvement by Paget disease. RESULTS: We identified 98 surgical pathology cases of EMPD, affecting 40 patients, with 67 Pap tests taken concurrently or after initial diagnosis. After our review, 10 of 67 (10/67, 15%) of Pap tests showed possible involvement by EMPD. Suspicious features included: hyperchromatic nuclei lacking visible nucleoli, increased nuclear to cytoplasmic ratios, and anisonucleosis. CONCLUSIONS: Cervicovaginal involvement by EMPD is likely underappreciated on cytology. In our experience, EMPD likely has a constellation of cytomorphologic features that warrant further evaluation in future studies.