Genomic Alterations as Potential Therapeutic Targets in Extramammary Paget's Disease of the Vulva.

To identify genomic alterations as potential therapeutic targets in extramammary Paget disease (EMPD) of the vulva. We identified all patients with primary vulvar EMPD who were treated at our institution and underwent paired tumor-normal massively parallel sequencing of 410-468 cancer-related genes (MSK-IMPACT assay). EMPD of the vulva samples sequenced from 2014 to 2019 were reviewed and somatic mutations identified, with specific focus on mutations of potential therapeutic targets. Clinical data were abstracted from electronic medical records. Microsatellite instability (MSI) was assessed by MSIscore. Tumors of 26 patients with EMPD underwent genomic sequencing. At diagnosis, all patients had noninvasive or microinvasive (< 1 mm) disease; invasive disease eventually developed in 2 patients. Primary treatment was surgery for 19 patients (73%) and imiquimod topical therapy for 7 (27%). Seven patients had ≥ 2 surgeries as part of clinical course (1 patient had 5 vulvar resections). Samples had a median of 2 coding mutations in the genes analyzed (range, 0-29). The most common mutations were in EMPD of the vulva has a chronic and relapsing course, often requiring multiple surgical resections. Effective topical treatments are lacking. We identified targetable mutations (

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© 2020 by American Society of Clinical Oncology.

Déclaration de conflit d'intérêts

The following represents disclosure information provided by authors of this manuscript. All relationships are considered compensated unless otherwise noted. Relationships are self-held unless noted. I = Immediate Family Member, Inst = My Institution. Relationships may not relate to the subject matter of this manuscript. For more information about ASCO's conflict of interest policy, please refer to www.asco.org/rwc or ascopubs.org/po/author-center. Open Payments is a public database containing information reported by companies about payments made to US-licensed physicians (Open Payments). Vance BroachOpen Payments Link: https://openpaymentsdata.cms.gov/physician/1162364Dennis S. ChiLeadership: C Surgeries Stock and Other Ownership Interests: Bovie Medical, Verthermia, Intuitive Surgical, TransEnterix Consulting or Advisory Role: Bovie Medical, Verthemia, Biom 'UpAnthony RossiHonoraria: Allergan, Evolus, Biofrontera, LAM Therapeutics, Cutera, Regeneron Research Funding: LEO Pharma (Inst), regen (Inst), Skin Cancer Foundation (Inst) Travel, Accommodations, Expenses: RegeneronTravis J. HollmanConsulting or Advisory Role: Sanofi/Aventis Research Funding: Bristol Myers SquibbAhmet ZehirHonoraria: IlluminaNadeem R. Abu-RustumHonoraria: Prime Oncology Research Funding: Stryker/Novadaq (Inst), Olympus (Inst), GRAIL (Inst) Travel, Accommodations, Expenses: Prime OncologyMario M. Leitao JrHonoraria: Intuitive Surgical Consulting or Advisory Role: Intuitive Surgical, Johnson & Johnson/Ethicon Research Funding: KCI Travel, Accommodations, Expenses: Intuitive Surgical No other potential conflicts of interest were reported.