Follicular dendritic cell sarcoma and its response to immune checkpoint inhibitors nivolumab and ipilimumab

Publication Date

2020

Journal Title

BMJ Case Rep

Abstract

© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ. 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.

Volume Number

13

Issue Number

4

Pages

e234363

Document Type

Article

Status

Faculty, Northwell Researcher

Facility

School of Medicine; Northwell Health

Primary Department

Hematology/Medical Oncology

Additional Departments

Pathology and Laboratory Medicine

PMID

32327462

DOI

10.1136/bcr-2020-234363

For the public and Northwell Health campuses

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