Clinical pathologic characteristics of extranodal follicular dendritic cell sarcoma
10.3760／cma.j.issn.0253?3766.2019.03.012
- VernacularTitle:结外滤泡树突细胞肉瘤的临床病理学特点
- Author:
Lifang CUI
1
;
Jixin ZHANG
;
Zhao LI
;
Ying GAO
;
Tao LU
;
Hongyu ZHAO
;
Dingrong ZHONG
Author Information
1. 首都医科大学附属北京世纪坛医院病理科 100038
- Keywords:
Follicular dendritic cell sarcoma;
PD?L1;
BRAF V600E;
Diagnosis
- From:
Chinese Journal of Oncology
2019;41(3):218-222
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the clinical pathologic characteristics of extranodal follicular dendritic cell sarcoma ( FDCS ). Methods We collected 7 cases of extranodal FDCS, HE staining, immunohistochemical study were performed. The V600E mutation of BRAF in 7 cases were detected by real?time PCR and EBER in situ hybridization was performed on 4 cases. Results Among the 7 cases of FDCS, 5 cases were male and 2 cases were female, the median age was 55 years old, including 4 cases of low?grade FDCS and 3 cases of high?grade FDCS. The tumor location of 2 cases was in mediastinum, the tumor locations of others were in nasopharynx, kidney, lung, rectum and liver, respectively. The results of immunohistochemistry showed that, the tumor cells were diffusely or focally positive for CD21, CD23, CD35, D2?40, EGFR and CXCL13, but negative for S?100, CD68, HMB45, SMA, Desmin, CD117, Dog?1, CD34, CD30, EMA and CK.Five cases were positive for PD?L1 and the its expression in high?grade FDCS were higher than that in low?grade FDCS.Two cases of low?grade FDCS were positive for BRAF V600E, but the BRAF V600E mutation weren′t detected in all of 7 cases. The result of EBER in?situ hybridization showed that only the nasopharynx FDCS was positive.The follow?up information of 5 patients were available (7～43 months), 4 patients died and 1 still alive with rectum metastasis.Conclusions FDCS is a rare malignant disease with relapse and metastatic tendency. The combined applications of the first?line antibodies including CD21, CD23, CD35 and second?line antibodies including D2?40, CXCL13, EGFR are helpful for its diagnosis and differential diagnosis. The high expression of PD?L1 implicates the potential benefit of FDCS patients acquired from immunotherapy.
