Extramammary Paget’s disease of the vulva (EMPDv) is a rare neoplasia, with only intraepithelial spread in most of the cases, slowly evolving, with aspecific symptoms and high recurrence rate.
Extensive surgery is often necessary with repeated excision in case of recurrent disease. This can lead to significant genital disfigurement and impairment of the quality of life. Less invasive treatments (radiotherapy, immunotherapy, photodynamic therapy, laser ablation) have variable response and high recurrence rate. In case of invasive disease, poorly responsive to radio/chemotherapy, if a HER2 overexpression is detected, excisional surgery followed by specific target therapy is showing promising results. The key factor for the optimal management of EMPDv is the prompt diagnosis and treatment of lesions, in a multidisciplinary approach with proper therapies and a long-term follow-up, preserving the patient’s genital function and self-image.