# Vulvar Adenocarcinoma With Pagetoid Spread: A Case Report

## TL;DR

This case report describes a rare instance of vulvar adenocarcinoma with pagetoid spread, where cancer cells spread in a pattern resembling Paget's disease.

## Contribution

The novelty lies in documenting a rare clinical case of vulvar adenocarcinoma with pagetoid spread without an identifiable primary lesion outside the vulva.

## Key findings

- The tumor was identified as secondary extramammary Paget's disease with pagetoid spread.
- Immunohistochemical markers confirmed the diagnosis and ruled out primary extramammary Paget's disease.
- Computed tomography showed lymph node metastasis associated with the vulvar adenocarcinoma.

## Abstract

In rare cases, cancer cells adjacent to the skin migrate intraepithelially and reach the epidermis, presenting a histological appearance similar to that of Paget's disease; this phenomenon is termed pagetoid spread. Herein, we report a case of vulvar adenocarcinoma with pagetoid spread, in which the primary lesion could not be identified outside the vulva.

An 84‐year‐old woman, presented with a 5‐cm tumor in the right vulva. Histopathological examination revealed atypical Paget cells. Immunohistochemical examination revealed that the cells were positive for CK20 and CK7 and negative for CDX2 and GCDFP15. Thus, it was determined that the tumor was not a primary extramammary Paget's disease, but a secondary extramammary Paget's disease with pagetoid spread. There were no masses in the anus, rectum, urinary tract, or vagina. Computed tomography scan revealed a right vulvar tumor and lymph node enlargement around the abdominal aorta, and the tumor was diagnosed as lymph node metastasis of vulvar adenocarcinoma associated with extramammary Paget's disease.

We report a rare case of vulvar adenocarcinoma with pagetoid spread.