Background: Pagetoid spread of adenocarcinoma in situ of the cervix into vaginal epithelium is a rare phenomenon, mimicking Paget's disease of the vulva.
Case: We report a case of in situ squamous cell and in situ adenocarcinoma of the cervix in a 44-year-old woman who underwent total abdominal hysterectomy, bilateral salpingo-oophorectomy, and upper vaginectomy. Pathologic evaluation showed pagetoid endocervical adenocarcinoma in situ spreading into the vagina with positive margins. Malignant cells were positive for mucin, P16, CK-7, and carcinoembryonic antigen.
Conclusion: The asymptomatic occult finding of pagetoid changes in the vagina should raise the suspicion of vaginal extension of pagetoid genital or urothelial carcinoma in situ; however, it can be mistaken as vaginal dysplasia or primary Paget's disease. Immunohistochemical profiling may help in the differential diagnosis.