New treatment standards for vulvar cancer 2020.

PURPOSE OF REVIEW: To highlight the recent advances regarding molecular mechanisms and therapeutic strategies in vulvar squamous cell carcinoma (VSCC), a rare but continuously rising disease. RECENT FINDINGS: Clinical research focuses on deescalation especially with regard to surgery. Recurrence patterns have been analyzed to further understand the course of disease showing a persistent risk for local recurrence even several years after the initial diagnosis. The main focuses of recent translational research are the distinct molecular mechanisms behind human papillomavirus (HPV)-positive and -negative VSCC. Next-generation sequencing analyses have highlighted TP53 as central driver mutation in HPV-negative disease. For HPV-independent VSCC, an impaired prognosis with limited disease-free and overall survival has been reported from a large multicenter analysis. Although no targeted agent has been granted approval, the impact of immunotherapy in vulvar cancer has been investigated in basket trials. Therapy response, however, was limited. SUMMARY: Further clinical research should focus on deciphering the molecular mechanisms of tumor development further. Detailed understanding of the molecular landscape will help to find novel therapy targets, fight the disease in advanced stages and thereby improve the quality of life for affected patients.