PMID- 34344077
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  - [Clinicopathological and molecular features of small round cell sarcoma of bone 
      and soft tissue: a study of 72 cases].
PG  - 919-923
LID - 10.3760/cma.j.cn112151-20201108-00833 [doi]
AB  - Objective: To investigate the clinicopathological, immunohistochemical and 
      molecular features of small round cell sarcoma (SRCS) of the bone and soft 
      tissue, and to compare the diagnostic value of different techniques. Methods: 
      Seventy-two cases of SRCS of the bone and soft tissue diagnosed at People's 
      Hospital, Peking University from January 2016 to March 2020 were recruited and 
      retrospectively analyzed for pathological morphology, immunophenotype and 
      fluorescence in situ hybridization (FISH) data. Next generation sequencing (NGS) 
      was performed on 13 difficult cases. Results: In the study cohort, the patients 
      ranged in age from 4-55 years, with a male predominance. The most Ewing's 
      sarcomas and osteosarcomas occurred in the bone, while CIC-rearranged sarcomas, 
      BCOR-rearranged sarcoma, synovial sarcoma, extraskeletal myxoid chondrosarcoma 
      and FUS-NFATc2 rearranged sarcoma occurred in soft tissue. Histologically, all 
      cases were composed predominantly of small round cells. Most cases were positive 
      for vimentin and CD99, and showed a variable reactivity for neurogenic markers. 
      Muscle marker and epithelial marker were negative for most cases. Combined with 
      clinical features, histopathologic findings, immunophenotype, FISH and NGS, we 
      diagnosed 46 Ewing sarcomas, 14 osteosarcomas, 3 CIC-rearranged sarcomas, 1 
      BCOR-rearranged sarcoma, 1 synovial sarcoma, 1 clear cell soft tissue sarcoma, 1 
      extraskeletal myxoid chondrosarcoma, 1 FUS-NFATc2 rearranged sarcoma, and 4 
      undifferentiated small round cell sarcomas. Conclusions: SRCS of bone and soft 
      tissue is a group of malignant mesenchymal tumors based on morphological 
      features. Most cases can be diagnosed with a combination of clinical 
      characteristics, morphological features and immunohistochemical phenotype, while 
      some cases require such further tests as FISH and NGS technologies, and NGS can 
      be useful in diagnosing and categorizing SRCS.
FAU - Yan, Y
AU  - Yan Y
AD  - Department of Pathology, People's Hospital, Peking University, Beijing 100044, 
      China.
FAU - Liu, L L
AU  - Liu LL
AD  - Department of Pathology, People's Hospital, Peking University, Beijing 100044, 
      China.
FAU - Kong, F Z
AU  - Kong FZ
AD  - Department of Pathology, People's Hospital, Peking University, Beijing 100044, 
      China.
FAU - Yan, T Q
AU  - Yan TQ
AD  - Department of Orthopaedic Oncology, People's Hospital, Peking University, Beijing 
      100044, China.
FAU - Shen, D H
AU  - Shen DH
AD  - Department of Pathology, People's Hospital, Peking University, Beijing 100044, 
      China.
LA  - chi
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
RN  - 0 (Biomarkers, Tumor)
RN  - 0 (Oncogene Proteins, Fusion)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Biomarkers, Tumor/genetics
MH  - Child
MH  - Child, Preschool
MH  - Female
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - Male
MH  - Middle Aged
MH  - Oncogene Proteins, Fusion/genetics
MH  - Retrospective Studies
MH  - *Sarcoma
MH  - *Sarcoma, Small Cell/diagnosis/genetics
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.cn112151-20201108-00833 [doi]
PST - ppublish
SO  - Zhonghua Bing Li Xue Za Zhi. 2021 Aug 8;50(8):919-923. doi: 
      10.3760/cma.j.cn112151-20201108-00833.