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CC BY-NC-ND 4.0 · Indian J Med Paediatr Oncol 2013; 34(04): 317-319
DOI: 10.4103/0971-5851.125255
CASE REPORT

Follicular dendritic cell sarcoma with paraneoplatic pemphigus: Rare case and a brief review of literature

Saphalta Baghmar
Department of Medical Oncology, Dr. BRA IRCH, AIIMS, New Delhi, India
,
Sanjeev Kumar
Department of Radiology, AIIMS, New Delhi, India
,
S Dutta Gupta
Department of Pathology, AIIMS, New Delhi, India
,
Vinod Raina
Department of Medical Oncology, Dr. BRA IRCH, AIIMS, New Delhi, India
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Abstract

Paraneoplastic pemphigus (PNP) is often a fatal autoimmune bullous disease characterized by severe stomatitis, polymorphous skin eruptions, and underlying neoplasms. We describe a patient with PNP associated with follicular dendritic cell sarcoma (FDCS), a rare neoplasm originating from follicular dendritic cells, which are non-lymphoid, non-phagocytic accessory cells of the lymphoid system and play an integral role in regulation of the germinal center reaction and present antigens to B-cells. The presence of rich vascularity around the tumor and few hyalanized vascular follicles found in histopathological examination gives the clue that the tumor might have developed from Castleman′s disease (CD). As for the mechanisms by which CD induces PNP, it has been proposed that autoantibodies secreted from the Castleman′s tumor play pivotal role. This hypothesis seems to be supported by the present case, in which CD may have triggered both the FDCS and the PNP.

Keywords

Castleman′s disease - follicular dendritic cell sarcoma - paraneoplatic pemphigus


Publication History

Article published online:
19 July 2021

© 2013. 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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