Case Report: HER2-positive vulvar Paget disease achieving long-term control with trastuzumab-based systemic therapy...

Vulvar Paget's disease with secondary invasive adenocarcinoma can be challenging to manage, particularly in inoperable or recurrent situations. We report this rare case to raise clinical awareness, accumulate diagnostic and treatment experience, and offer guidance for managing similar cases in the future.

We present a 79-year-old woman with large invasive vulvar Paget's disease (HER2 3+).

As surgery was not feasible, she received trastuzumab plus capecitabine, achieving marked tumor shrinkage. Nine months later, local recurrence with bone metastasis was treated with the same regimen followed by sequential radiotherapy (60 Gy), resulting in partial remission. With trastuzumab maintenance, disease control has been sustained for 17 months.

HER2-targeted therapy combined with chemotherapy and radiotherapy is a promising treatment strategy for HER2-positive, inoperable or recurrent VPD.