Treatment of metastatic extramammary Paget disease with combination ipilimumab and nivolumab: A case report Journal Article


Authors: Guercio, B. J.; Iyer, G.; Kidwai, W. Z.; Lacouture, M. E.; Ghafoor, S.; Rossi, A. M.; Assis, D. N.; Chen, Y. B.; Busam, K. J.; Janjigian, Y. Y.; Jhaveri, K.; Feldman, D. R.; Capozzi, A.; Figueroa, V.; Bajorin, D. F.; Rosenberg, J. E.; Hollmann, T. J.; Funt, S. A.
Article Title: Treatment of metastatic extramammary Paget disease with combination ipilimumab and nivolumab: A case report
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. © 2021 The Author(s). Published by S. Karger AG, Basel.
Keywords: ipilimumab; immunotherapy; immune checkpoint blockade; nivolumab; extramammary paget disease
Journal Title: Case Reports in Oncology
Volume: 14
Issue: 1
ISSN: 1662-6575
Publisher: S. Karger AG  
Date Published: 2021-01-01
Start Page: 430
End Page: 438
Language: English
DOI: 10.1159/000514345
PROVIDER: scopus
PMCID: PMC7983595
PUBMED: 33790764
DOI/URL:
Notes: Article -- Export Date: 1 June 2021 -- Source: Scopus
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MSK Authors
  1. Mario E Lacouture
    458 Lacouture
  2. Dean Bajorin
    663 Bajorin
  3. Darren Richard Feldman
    348 Feldman
  4. Yelena Yuriy Janjigian
    403 Janjigian
  5. Gopakumar Vasudeva Iyer
    358 Iyer
  6. Yingbei Chen
    402 Chen
  7. Komal Lachhman Jhaveri
    223 Jhaveri
  8. Klaus J Busam
    698 Busam
  9. Jonathan Eric Rosenberg
    524 Rosenberg
  10. Anthony Rossi
    243 Rossi
  11. Travis Jason Hollmann
    127 Hollmann
  12. Samuel Aaron Funt
    143 Funt
  13. Brendan John Guercio
    16 Guercio