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CC BY-NC-ND 4.0 · Indian J Med Paediatr Oncol
DOI: 10.1055/s-0042-1742319
Case Report with Review of Literature

Extra-Nodal Follicular Dendritic Cell Sarcoma with Unusual Morphological Features and PDL1 Expression

Manali Ranade
1   Department of Pathology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, Maharashtra, India
,
Aekta Shah
1   Department of Pathology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, Maharashtra, India
,
Subhash Yadav
1   Department of Pathology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, Maharashtra, India
,
Santosh Menon 
1   Department of Pathology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, Maharashtra, India
,
Sudeep Gupta 
2   Department of Medical Oncology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, Maharashtra, India
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Abstract

Follicular dendritic cell (FDC) sarcoma, a rare tumor of FDCs, poses a diagnostic challenge due to its myriad morphologic and architectural patterns. We present a unique case of extra-nodal FDC sarcoma occurring in a 28-year-old gentleman who presented with multiple pelvic, peritoneal and retroperitoneal soft tissue, and axial skeletal lesions. The tumor was composed of islands and trabeculae of epithelioid cells with interspersed lymphocytes against a background of abundant myxoid matrix. On immunohistochemistry, in addition to the CD21, the tumor was positive for GATA3, inhibin, and programmed death-ligand 1 (PDL1), which have been rarely reported previously in FDC sarcoma. The patient underwent surgical excision of the tumor and was later offered immunotherapy. He has been disease-free for 2 years following immunotherapy. Our case highlights the potential role of immune checkpoint inhibitors in treating FDC sarcoma, which show immuno-expression of PDL1.

Keywords

follicular dendritic cell sarcoma - PDL1 - immunotherapy

Declaration of Patient Consent

The authors certify that they have obtained all appropriate patient consent forms.


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Publication History

Article published online:
26 April 2022

© 2022. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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