Treatment of Metastatic Extramammary Paget Disease with Combination Ipilimumab and Nivolumab: A Case Report

Case Reports in Oncology

Gopa Iyer¹  Vanessa Figueroa¹  Jonathan E. Rosenberg¹  Dean F. Bajorin¹  Komal Jhaveri¹  Samuel A. Funt¹  Mario E. Lacouture¹  Anthony M. Rossi¹  Anne Capozzi¹  Darren R. Feldman¹  Brendan John Guercio¹  Yelena Y. Janjigian¹  Wajih Zaheer Kidwai²  David N. Assis³  Travis J. Hollmann⁴  Klaus J. Busam⁴  Ying-Bei Chen⁴  Soleen Ghafoor⁵ 

[1] Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY, USA;Department of Medicine, Yale Cancer Center/Smilow Cancer Hospital, New Haven, CT, USA;Department of Medicine, Yale School of Medicine, New Haven, CT, USA;Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA;Institute of Diagnostic and Interventional Radiology, University Hospital Zurich, Zurich, Switzerland;

关键词: extramammary paget disease;    immunotherapy;    nivolumab;    ipilimumab;    immune checkpoint blockade;   

DOI  :  10.1159/000514345

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown