Inflammatory pseudotumor-like follicular dendritic cell sarcoma: clinicopathological features and prognosis study of 6 casesChinese Full Text
XIANG Chun-xiang;WANG Jian-jun;WU Nan;YU Bo;XIA Chun;HE Yan;MA Jie;SHI Qun-li;Department of Pathology,Nanjing General Hospital of Nanjing Military Command;Department of Pathology,Wuhan Central Hospital;
Abstract: Purpose To explore the clinicopathological features of inflammatory pseudotumor-like follicular dendrtic cell sarcoma( IPT-like FDCS). Methods Six patients diagnosed with IPT-like FDCS were retrospectively observed and analyzed by means of clinicopathological data,immunohistochemical staining and in situ hybridization for Epstein-Barr virus( EBV)-encoded RNA( EBER).Results Among the 6 cases diagnosed as IPT-like FDCS,four cases were female,two cases were male,aged from 40 to 78 years( mean 62.3 years). The clinical symptoms were non-specific,which four cases were documented by physical examination,one had abdominal distension and emaciation,and one had low back pain with the tumor in the retroperitoneal. The neoplasms,with a mean size 7. 8 cm( range,3. 5 to 14 cm),occurred in the spleen( three cases),liver( two cases) and retroperitoneal( one case),which were usually slow-growing. The neoplasms usually had well-defined borders,composed of round,oval and spindle cells. The optical management of dysplasia were not obvious and they were arranged in whorls and a spiral or storiform growth pattern,mixed with inflammatory cells. Immunohistochemically,tumor cells were positive for CD21 /35,vimentin and Clusterin protein,with EBER in situ hybridization scattered positive. All cases went complete surgical excision of tumors and were followed up for 3 48 months,with 5 patients disease-free and 1 patient lost follow-up( disease-unknown). Conclusions IPT-like FDCS is a rare low-grade malignant tumor associated with EBV infection with a relatively indolent biological behavior,which is female predominated and mainly occurs in spleen and liver,and complete surgical excision may be the optimal approach to be performed.
- DOI:
10.13315/j.cnki.cjcep.2014.07.012
- Series:
- Subject:
- Classification Code:
R735.2
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