PMID- 34344069 OWN - NLM STAT- MEDLINE DCOM- 20210805 LR - 20210805 IS - 0529-5807 (Print) IS - 0529-5807 (Linking) VI - 50 IP - 8 DP - 2021 Aug 8 TI - [Mesenchymal chondrosarcoma in central nervous system: a clinicopathological analysis]. PG - 870-875 LID - 10.3760/cma.j.cn112151-20210302-00176 [doi] AB - Objective: To investigate the clinicopathological features of central nervous system (CNS) mesenchymal chondrosarcoma (MCS). Methods: Nine cases of CNS MCS were collected at the First Affiliated Hospital of Fujian Medical University from September 2010 to September 2020. The clinical,imaging,histopathological and immunohistochemical features were reviewed. NCOA2 gene rearrangement was evaluated by fluorescence in situ hybridization (FISH). Results: There were three male and six female patients, with age range of 1 to 59 years (median 31 years). Six cases were intracranial and three cases were intraspinal, and the tumors showed dural attachment. They were often diagnosed as meningioma basing on preoperative imaging. Microscopically, the tumors showed a characteristic biphasic histologic pattern composed of undifferentiated mesenchymal small cells and well-differentiated hyaline cartilage islands. The small cells area were positive for SOX9 (9/9), CD99 (8/9), and without BRG1 and INI1 deletion. The cartilaginous component expressed SOX9 (9/9) and S-100 protein (8/9). NCOA2 gene break apart signal was identified in five cases (5/5). Eight patients were followed up for 4-124 months. Three patients (3/8) had recurrences within one year and two patients died of the tumor. Conclusions: CNS MCS is an extremely rare malignant neoplasm with a propensity to dural involvement. Preoperative imaging has low diagnostic accuracy. CNS MCS should be differentiated from other CNS small round cell tumors and chondrosarcoma. FISH detection of NCOA2 gene rearrangement will assist the diagnosis of MCS. FAU - Huang, Q AU - Huang Q AD - Department of Pathology, the First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, China. FAU - Chen, Y P AU - Chen YP AD - Department of Pathology, the First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, China. FAU - Song, F L AU - Song FL AD - Department of Pathology, the First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, China. FAU - Zheng, L M AU - Zheng LM AD - Department of Pathology, the First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, China. FAU - Liu, X Y AU - Liu XY AD - Department of Pathology, the First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, China. FAU - Zhang, S AU - Zhang S AD - Department of Pathology, the First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, China. FAU - Wang, X F AU - Wang XF AD - Department of Pathology, the First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, China. LA - chi GR - 2018J01155/Natural Science Foundation of Fujian Province/ GR - FBJG20170072/Funds of Fujian Provincial Research Project on the Reform of Undergraduate Education and Teaching/ PT - Journal Article PL - China TA - Zhonghua Bing Li Xue Za Zhi JT - Zhonghua bing li xue za zhi = Chinese journal of pathology JID - 0005331 SB - IM MH - Adolescent MH - Adult MH - *Bone Neoplasms/diagnostic imaging/genetics MH - Central Nervous System MH - Child MH - Child, Preschool MH - *Chondrosarcoma/diagnostic imaging/genetics/surgery MH - *Chondrosarcoma, Mesenchymal/diagnostic imaging/genetics/surgery MH - Female MH - Humans MH - In Situ Hybridization, Fluorescence MH - Infant MH - Male MH - Middle Aged MH - Young Adult EDAT- 2021/08/04 06:00 MHDA- 2021/08/06 06:00 CRDT- 2021/08/03 23:10 PHST- 2021/08/03 23:10 [entrez] PHST- 2021/08/04 06:00 [pubmed] PHST- 2021/08/06 06:00 [medline] AID - 10.3760/cma.j.cn112151-20210302-00176 [doi] PST - ppublish SO - Zhonghua Bing Li Xue Za Zhi. 2021 Aug 8;50(8):870-875. doi: 10.3760/cma.j.cn112151-20210302-00176.