Cytology of Vulvar/Vaginal Paget Disease

OBJECTIVE: We describe a woman with a long-standing history of Paget disease involving the vulva and vagina, who was previously diagnosed with a high-grade squamous lesion, atypical glandular cells, and adenocarcinoma on various different yearly Pap tests. MATERIALS AND METHODS: This article is a case report of this patient and a review of the literature concerning the cytology of Paget disease. RESULTS: By reviewing Pap test cytology findings, Paget cells were identified as atypical cell groups with enlarged nuclei and occasional prominent nucleoli and further characterized by immunoreactivity to carcinoembryonic antigen and gross cystic disease fluid protein that distinguished them from high-grade squamous lesion. CONCLUSIONS: By enabling early diagnosis, regular surveillance, and identification of Paget cells by cytopathology, the Pap test becomes a valuable tool in identifying initial and recurrent Paget disease when it involves the vagina.