Vulvar Extramammary Paget Disease: Diagnostic Challenge and Surgical Management in a Case Report

Background: Vulvar extramammary Paget disease (EMPD) is a rare intraepithelial adenocarcinoma that typically affects postmenopausal women and often mimics benign inflammatory or infectious vulvar conditions, leading to delayed diagnosis. Optimal diagnosis and therapy remain challenging due to its nonspecific presentation and variable extent of disease. Case Presentation: We report a 58-year-old Lebanese postmenopausal woman with more than one year of persistent vulvar pruritus and an erythematous lesion refractory to topical antifungal and corticosteroid therapy. Clinical evaluation revealed a 5 cm vulvar lesion without lymphadenopathy. Vulvar biopsy confirmed extramammary Paget disease, and staging work-up excluded associated malignancies. Although histology suggested non-invasive disease, the lesion was extensive with ill-defined margins and was reviewed by a multidisciplinary tumor board. A key feature of this case is the discordance between non-invasive biopsy findings and the decision to proceed with radical surgery due to concern for occult invasion. The patient underwent radical vulvectomy with left superficial and deep inguinal lymph node dissection. Final histopathology confirmed disease confined to the epidermis with negative margins and no nodal involvement (0/8). Postoperative imaging at 6 months showed no recurrence or metastasis. Conclusions: This case highlights prolonged diagnostic delay despite multiple treatments and the management challenge posed by extensive clinical disease with non-invasive biopsy findings. It underscores the importance of considering EMPD in chronic refractory vulvar lesions. Management should be individualized in multidisciplinary settings. Given the short follow-up, long-term outcomes cannot be established, and careful surveillance remains essential due to the risk of late recurrence.