Extra-Nodal Follicular Dendritic Cell Sarcoma with Unusual Morphological Features and PDL1 Expression
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 retroperit...
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Thieme Medical and Scientific Publishers Pvt. Ltd.
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| Series: | Indian Journal of Medical and Paediatric Oncology |
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| Online Access: | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0042-1742319 |
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| author | Manali Ranade Aekta Shah Subhash Yadav Santosh Menon Sudeep Gupta |
| author_facet | Manali Ranade Aekta Shah Subhash Yadav Santosh Menon Sudeep Gupta |
| author_sort | Manali Ranade |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-654a714b2bd3401a90fcceffc09cce89 |
| institution | DOAJ |
| issn | 0971-5851 0975-2129 |
| language | English |
| publisher | Thieme Medical and Scientific Publishers Pvt. Ltd. |
| record_format | Article |
| series | Indian Journal of Medical and Paediatric Oncology |
| spelling | doaj-art-654a714b2bd3401a90fcceffc09cce892025-08-20T03:21:06ZengThieme Medical and Scientific Publishers Pvt. Ltd.Indian Journal of Medical and Paediatric Oncology0971-58510975-212910.1055/s-0042-1742319Extra-Nodal Follicular Dendritic Cell Sarcoma with Unusual Morphological Features and PDL1 ExpressionManali Ranade0Aekta Shah1Subhash Yadav2Santosh Menon3https://orcid.org/0000-0002-4140-0671Sudeep Gupta4https://orcid.org/0000-0002-6742-6378Department of Pathology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, Maharashtra, IndiaDepartment of Pathology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, Maharashtra, IndiaDepartment of Pathology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, Maharashtra, IndiaDepartment of Pathology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, Maharashtra, IndiaDepartment of Medical Oncology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, Maharashtra, IndiaFollicular 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.http://www.thieme-connect.de/DOI/DOI?10.1055/s-0042-1742319follicular dendritic cell sarcomaPDL1immunotherapy |
| spellingShingle | Manali Ranade Aekta Shah Subhash Yadav Santosh Menon Sudeep Gupta Extra-Nodal Follicular Dendritic Cell Sarcoma with Unusual Morphological Features and PDL1 Expression Indian Journal of Medical and Paediatric Oncology follicular dendritic cell sarcoma PDL1 immunotherapy |
| title | Extra-Nodal Follicular Dendritic Cell Sarcoma with Unusual Morphological Features and PDL1 Expression |
| title_full | Extra-Nodal Follicular Dendritic Cell Sarcoma with Unusual Morphological Features and PDL1 Expression |
| title_fullStr | Extra-Nodal Follicular Dendritic Cell Sarcoma with Unusual Morphological Features and PDL1 Expression |
| title_full_unstemmed | Extra-Nodal Follicular Dendritic Cell Sarcoma with Unusual Morphological Features and PDL1 Expression |
| title_short | Extra-Nodal Follicular Dendritic Cell Sarcoma with Unusual Morphological Features and PDL1 Expression |
| title_sort | extra nodal follicular dendritic cell sarcoma with unusual morphological features and pdl1 expression |
| topic | follicular dendritic cell sarcoma PDL1 immunotherapy |
| url | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0042-1742319 |
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