Human epidermal growth factor receptor 2 (HER2) is tyrosine kinase receptor encoded by the ERBB2 gene [1]. ERBB2 amplification results in overexpression, receptor dimerization and persistent activation of downstream pathways and tumor growth [1]. ERBB2 amplification is commonly encountered among patients with breast cancer, and gastroesophageal malignancies [2,3]. Previous studies have also identified a high incidence of ERBB2 amplification among patients with uterine serous carcinoma [4,5]. For patients with metastatic or recurrent uterine serous carcinoma, a randomized phase II demonstrated survival benefit from the addition of transtuzumab, a monoclonal antibody against HER2 receptor, to standard of care chemotherapy [6]. However, ERBB2 amplification has been also reported by smaller studies in patients with other high-grade endometrial cancers as well as those with mucinous ovarian cancer [4,7]. Several new agents targeting tumors with ERBB2 amplification are currently under investigation underlying the importance of understanding the molecular landscape of ERBB2 amplification among patients with gynecologic malignancies [[8], [9], [10], [11], [12], [13], [14], [15], [16], [17], [18]].

In the present study we sought to investigate the prevalence of ERBB2 gene amplification among patients with gynecologic malignancies and explore the genomic profile of ERBB2 amplified tumors using a large multicenter dataset. In addition, we discuss emerging strategies to target ERBB2/HER2 amplification that can provide new therapeutic avenues for these patients.