PMID- 33396985
OWN - NLM
STAT- MEDLINE
DCOM- 20210106
LR  - 20210106
IS  - 0529-5807 (Print)
IS  - 0529-5807 (Linking)
VI  - 50
IP  - 1
DP  - 2021 Jan 8
TI  - [Clinicopathological characteristics of pulmonary artery intimal sarcoma].
PG  - 38-43
LID - 10.3760/cma.j.cn112151-20200413-00313 [doi]
AB  - Objective: To describe the clinicopathological features of pulmonary artery 
      intimal sarcoma (PAIS), and to understand its molecular alterations. Methods: 
      Sixty cases of pulmonary artery endarterectomy performed at the China-Japan 
      Friendship Hospital, Beijing, China from January 2017 to January 2020 were 
      reviewed. Clinical data of 5 patients with pulmonary artery intimal sarcoma were 
      collected. Hematoxylin-eosin staining, immunohistochemistry staining and 
      fluorescence in situ hybridization (FISH) were performed to evaluate the 
      pathological features. RNA sequencing was conducted to assess the fusion gene 
      changes in PAIS. Results: The detection rate of PAIS was 8.3% (5/60), with the 
      median age of 49 years and a female predominance. Their clinical manifestations 
      were non-specific. Histopathological examination showed that the tumors were 
      composed of malignant spindle or epithelioid cells, with various degrees of 
      atypia. Focal heterologous osteosarcomatous or leiomyosarcomatous differentiation 
      was noted. The tumor cells could express PDGFRA, CDK4 and MDM2 with 
      co-amplification of MDM2, CDK4 and EGFR genes. RNA sequencing detected multiple 
      in-frame fusions in the tumors. Conclusions: PAIS is a rare, highly 
      heterogeneous, and poorly-or un-differentiated sarcoma accompanied by complex 
      changes of multiple genes.It has no known effective treatments, and thus has a 
      poor prognosis.
FAU - Wang, B
AU  - Wang B
AD  - Department of Pathology, China-Japan Friendship Hospital, Beijing 100029, China.
FAU - Zhang, T
AU  - Zhang T
AD  - Department of Pathology, China-Japan Friendship Hospital, Beijing 100029, China.
FAU - Liu, H Y
AU  - Liu HY
AD  - Department of Pathology, China-Japan Friendship Hospital, Beijing 100029, China.
FAU - Chen, R R
AU  - Chen RR
AD  - Geneplus-Beijing Ltd., Beijing 102206, China.
FAU - Zhang, X Y
AU  - Zhang XY
AD  - Department of Pulmonary and Critical Care Medicine, Center of Respiratory 
      Medicine, China-Japan Friendship Hospital, Beijing 100029, China.
FAU - Zhang, H L
AU  - Zhang HL
AD  - Department of Pathology, China-Japan Friendship Hospital, Beijing 100029, China.
FAU - Zhai, Z G
AU  - Zhai ZG
AD  - Department of Pulmonary and Critical Care Medicine, Center of Respiratory 
      Medicine, China-Japan Friendship Hospital, Beijing 100029, China.
FAU - Zhong, D R
AU  - Zhong DR
AD  - Department of Pathology, China-Japan Friendship Hospital, Beijing 100029, 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)
SB  - IM
MH  - Biomarkers, Tumor
MH  - China
MH  - Female
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - Middle Aged
MH  - Pulmonary Artery/surgery
MH  - *Sarcoma/genetics/surgery
MH  - *Vascular Neoplasms/genetics/surgery
OTO - NOTNLM
OT  - Gene amplification
OT  - Gene rearrangement
OT  - Hemangiosarcoma
OT  - Pulmonary artery
OT  - Tunica intima
EDAT- 2021/01/06 06:00
MHDA- 2021/01/07 06:00
CRDT- 2021/01/05 02:30
PHST- 2021/01/05 02:30 [entrez]
PHST- 2021/01/06 06:00 [pubmed]
PHST- 2021/01/07 06:00 [medline]
AID - 10.3760/cma.j.cn112151-20200413-00313 [doi]
PST - ppublish
SO  - Zhonghua Bing Li Xue Za Zhi. 2021 Jan 8;50(1):38-43. doi: 
      10.3760/cma.j.cn112151-20200413-00313.