Abstract

BackgroundExtranodal follicular dendritic cell sarcoma (FDCS) is a very rare malignancy with a variable clinical course. It is often not considered and has the potential to result in a misdiagnosis of other common sarcomas or sarcomatoid carcinomas. This is particularly true with the preoperative biopsy specimen, in which the tissue sample is often small.Case presentationA case of FDCS in a 63-year-old woman, arising in the urinary bladder, a previously unreported site, is described. The patient presented with the typical clinical symptoms of a bladder cancer, and the morphology of the tumor was similar to a lymphoepithelioma-like carcinoma, ultimately resulting in it being misdiagnosed. The patient received radical cystectomy, without further radiotherapy or chemotherapy. Two years after operation, a metastatic tumor to the lung was found. The mass of the right main bronchus lumen was frozen and resected through bronchoscopy, and radiotherapy was performed. The patient has lived with the tumor since then.ConclusionsThis paper presents the first FDCS occurring in the urinary bladder with metastasis to the lung and emphasizes potential diagnostic pitfalls.

Highlights

  • Extranodal follicular dendritic cell sarcoma (FDCS) is a very rare malignancy with a variable clinical course

  • This paper presents the first FDCS occurring in the urinary bladder with metastasis to the lung and emphasizes potential diagnostic pitfalls

  • Follicular dendritic cell sarcoma (FDCS) is a rare malignancy derived from follicular dendritic cells, which form a meshwork in lymphoid follicles and have the role of antigen capture and presentation [1, 2]

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Summary

Background

Follicular dendritic cell sarcoma (FDCS) is a rare malignancy derived from follicular dendritic cells, which form a meshwork in lymphoid follicles and have the role of antigen capture and presentation [1, 2]. The immunohistochemical staining showed results similar to the preoperative biopsy: the tumor cells were uniformly negative for CK(AE1/AE3) (Fig. 3b), CK7, CK20, P63 (while the remaining superficial urothelial cells were positive), focally positive for EMA, and positive for vimentin and P53. By further carefully observing the pathologic morphology, as there was the infiltration of mature small lymphocytes among the tumor tissue, a scattered distribution of multinucleated tumor giant cells, as well as focal cystitis glandularis around the tumor lesions, we considered the case to be a very rare FDCS, which had not yet been reported in the bladder. The immunohistochemical staining for the remaining preoperative biopsy tissues showed that the tumor cells were reactive to CD21, CD23, and CD35(Fig. 2f), supporting the diagnosis of FDCS of bladder.

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