Primary Extra Mammary Paget’s Disease of Vulva With Apocrine Adenocarcinoma Signet Ring Cell Differentiation and Distant Metastasis

摘要

Objective: Extramammary Paget's disease (EMPD) with invasive carcinoma and distant metastasis is extremely rare. In vulva EMPD associated apocrine carcinoma with signet ring cell differentiation has not been described in the literature so far. Its slow evolution, varied clinical presentation and histological appearances, lead to difficulty in diagnosis of this disease.