Patients treated with disitamab vedotin had an 80% objective response rate (ORR) and a 100% disease control rate (DCR), with no adverse events above grade 3-4.<h4>Conclusion</h4>HER2 was frequently expressed in both primary tumors and metastatic LNs in EMPD patients, though heterogeneity was observed. This evidence concerns the gene ERBB2 and extramammary Paget disease.