TP53 Mutation-driven Stratified Mucin-producing Carcinoma Coexisting With Squamous Cell Carcinoma of the Vulva: A Case Study.

ABSTRACT

Associated with high-risk human papillomavirus infection, invasive stratified mucin-producing carcinoma is a recently characterized adenocarcinoma of the cervix. It often occurs in association with adjacent stratified mucin-producing intraepithelial lesion. Differentiated vulvar intraepithelial neoplasia and related invasive squamous cell carcinoma often arise in background vulvar lichen sclerosus with TP53 mutation as the underlying molecular signature. We present a unique case of vulvar invasive stratified mucin-producing carcinoma-like component coexisting with invasive squamous cell carcinoma in a 64-year-old woman. Both neoplastic components were proven TP53 -driven processes arising in the background of differentiated vulvar intraepithelial neoplasia and lichen sclerosus. The invasive stratified mucin-producing carcinoma-like component behaved aggressively in this case, evidenced by the presence of lymphovascular invasion and inguinal lymph node metastasis.