ArticleCase reports in oncology
Treatment of Metastatic Extramammary Paget Disease with Combination Ipilimumab and Nivolumab: A Case Report.
Article in Case reports in oncology. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed.
- Carelizumab and rivoceranib for advanced extramammary Paget's disease: an investigator-initiated multicenter, single-arm, phase II trial.The oncologist · 2025Trial
- Treatment Strategies for Extramammary Paget Disease: A Narrative Review of Current Status and Future Directions.Cancers · 2026Review
- Extramammary Paget's Disease of the Suprapubic Region in a Male: A Novel Diagnostic Imaging Approach and Literature Review.Journal of clinical medicine · 2025Article
- PIK3CA inhibitor treatment for metastatic scrotal extramammary Paget's disease: a case report and literature review.AME case reports · 2025Article
- Whole genome sequencing of HER2-positive metastatic extramammary Paget's disease: a case report.Orphanet journal of rare diseases · 2024Article
- Article
- Tislelizumab plus chemotherapy in metastatic extramammary Paget disease after surgery: a case report.Frontiers in oncology · 2024Article
- Extramammary Paget Disease: a Therapeutic Challenge, for a Rare Entity.Current oncology reports · 2023Review
- Trousseau Syndrome in a Case of Extramammary Paget's Disease.Acta dermato-venereologica · 2023Article
- Extramammary Paget's disease: Updates in the workup and management.Asian journal of urology · 2022Review
- Case Report: Prolonged clinical benefit with sequential trastuzumab-containing treatments in a patient with advanced extramammary Paget disease of the groin.Frontiers in oncology · 2022Article
- Treatment of Metastatic Primary Extramammary Paget Disease With Combination Anlotinib and Tislelizumab: A Case Report and Review of the Literature.Frontiers in medicine · 2022Article
- Extramammary Paget's Disease: Diagnosis, Pathogenesis, and Treatment with Focus on Recent Developments.Current oncology (Toronto, Ont.) · 2021Review
Corrections and comments
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Authors and funding
18 authors.
Funding
Abstract
Metastatic primary cutaneous extramammary Paget disease (EMPD) is a rare clinical entity with a 5-year survival <10% and no standard therapy. We report the first case to our knowledge of metastatic EMPD with treatment response to checkpoint inhibitor immunotherapy. The patient had diffusely metastatic disease and previously progressed on cytotoxic chemotherapy and a molecularly targeted agent. Treatment with four cycles of ipilimumab 1 mg/kg plus nivolumab 3 mg/kg resulted in a durable partial response lasting 7 months. Analysis of metastatic tumor tissue failed to identify known predictors of treatment response to immune checkpoint inhibitors, such as high PD-L1 expression, high tumor mutation burden, or microsatellite instability. These findings support further investigation of immune checkpoint inhibition for the management of metastatic EMPD, which currently has an abysmal prognosis and no standard therapies.
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