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Case report
Follicular dendritic cell sarcoma and its response to immune checkpoint inhibitors nivolumab and ipilimumab
  1. Mee-young Lee1,
  2. Carolina Bernabe-Ramirez2,
  3. Daniel C Ramirez3,4 and
  4. Robert G Maki5
  1. 1Monter Cancer Center, Northwell Health, Lake Success, New York, USA
  2. 2Medical Oncology, Essen Medical, Long Island City, New York, USA
  3. 3Pathology, Northwell Health, Great Neck, New York, USA
  4. 4Pathology, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Hempstead, New York, USA
  5. 5Abramson Cancer Center, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA
  1. Correspondence to Professor Robert G Maki; bobmakimd{at}


Follicular dendritic cell sarcoma (FDCS) is a rare and unusual cancer that arises from sustentacular cells of the lymph node that present antigen to B cells, rather than lymphocytes themselves. While surgery for primary disease is still paramount in primary management, for unresectable, recurrent and metastatic tumours, FDCS is frequently treated with anthracycline-based lymphoma chemotherapy regimens. In recent years, it is clear that Programmed Cell Death 1 (PD1)-directed immune checkpoint inhibitors (ICIs) are active in Hodgkin lymphoma, but significantly less active in non-Hodgkin’s lymphoma. These data raised the question of whether FDCS respond to ICI therapy. We present two patients with FDCS who were treated with nivolumab and ipilimumab with evidence of tumour response. These cases also highlight the difficulty in arriving at a proper diagnosis, emphasising the need for expert review of pathology to optimise treatment for these and other patients with sarcoma.

  • cancer intervention
  • oncology
  • immunology

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  • Contributors RGM and M-yL were responsible for conception and design of the manuscript. All authors were involved in the reporting, acquisition of data and interpretation of data. All authors contributed to the writing of the manuscript and gave their approval of the final version of the text as prepared.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.