To study the clinicopathologic features of a rare disease of pagetoid urothelial intraepithelial neoplasia (PUIN) in the vulva.Materials and Methods
We reviewed a case of PUIN in a Chinese woman with a long history of bladder urothelial carcinoma.Results
The patient presented with vulvar pruritus for more than 1 month. Gynecologic examination showed a red, thickened, eczematoid lesion in the bilateral labia minora and a palpable 4-cm mass between the middle part of the vagina and the urethral meatus. Microscopically, the neoplastic cells with clear or pale eosinophilic cytoplasm were distributed throughout the squamous epithelium, with a predilection for the middle and basal portion in the vulva. Acantholysis-related papillary formation and pagetoid spread pattern to the normal squamous epithelium were also present. Invasive carcinoma was found underneath the unremarkable squamous epithelium in the vaginal biopsy. Immunohistochemistry demonstrated that these cells were negative for mucin stain, carcinoembryonic antigen, and 34βE12 and were strongly positive for cytokeratins 5/6, 7, 18, 19, and 20.Conclusions
This rare entity of PUIN was associated with metastatic urothelial carcinoma and should be discriminated from vulvar Paget disease and pagetoid squamous cell carcinoma in situ.