PMID- 32846779
OWN - NLM
STAT- MEDLINE
DCOM- 20200910
LR  - 20221005
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Print)
IS  - 0025-7974 (Linking)
VI  - 99
IP  - 34
DP  - 2020 Aug 21
TI  - Clinical features of 18 perivascular epithelioid cell tumor cases.
PG  - e21659
LID - 10.1097/MD.0000000000021659 [doi]
LID - e21659
AB  - To investigate the biological behavior and clinical characteristics of 
      perivascular epithelioid cell tumor (PEComa).Eighteen PEComa patients admitted to 
      Zhongshan Hospital and the Central Hospital of Xuhui District in China from 
      January 2006 to October 2018 were included. All patients were diagnosed based on 
      pathological findings and treated with surgical resection or medication.Among the 
      18 patients, 1 underwent lymph node biopsy for multiple enlarged lymph nodes and 
      17 underwent mass resection. The median disease-free survival was 22 months after 
      the first resection and over 12 months following a second resection. Treatment 
      with mechanistic target of rapamycin (mTOR) inhibitors was effective for patients 
      with unresectable or metastatic lesions. The median progression-free survival was 
      approximately 13 months.Surgery is the predominant treatment approach for PEComa 
      and patients can benefit from multiple operations. mTOR inhibitors are considered 
      for patients with multiple lesions or intolerance to surgery. Anti-angiogenetic 
      drugs can be selected when mTOR inhibitors fail to control the illness.
FAU - Jia, Rongfei
AU  - Jia R
AD  - Department of Oncology, Central Hospital of Xuhui District.
FAU - Jiang, Ling
AU  - Jiang L
AD  - Department of Oncology, Central Hospital of Xuhui District.
FAU - Zhou, Yuhong
AU  - Zhou Y
AD  - Department of Oncology.
FAU - Wang, Yan
AU  - Wang Y
AD  - Department of Oncology.
FAU - Guo, Xi
AU  - Guo X
AD  - Department of Oncology.
FAU - Ji, Yuan
AU  - Ji Y
AD  - Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai, China.
FAU - Ni, Xiang
AU  - Ni X
AD  - Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai, China.
FAU - Yang, Xiaoyan
AU  - Yang X
AD  - Department of Oncology, Central Hospital of Xuhui District.
LA  - eng
PT  - Journal Article
PT  - Multicenter Study
PT  - Observational Study
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
SB  - IM
MH  - Adult
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Perivascular Epithelioid Cell Neoplasms/*diagnosis/therapy
MH  - Young Adult
PMC - PMC7447485
COIS- Competing interests: The authors declare that they have no competing interests. 
      The authors have no funding and conflicts of interest to disclose.
EDAT- 2020/08/28 06:00
MHDA- 2020/09/12 06:00
PMCR- 2020/08/21
CRDT- 2020/08/28 06:00
PHST- 2020/08/28 06:00 [entrez]
PHST- 2020/08/28 06:00 [pubmed]
PHST- 2020/09/12 06:00 [medline]
PHST- 2020/08/21 00:00 [pmc-release]
AID - 00005792-202008210-00033 [pii]
AID - MD-D-19-10220 [pii]
AID - 10.1097/MD.0000000000021659 [doi]
PST - ppublish
SO  - Medicine (Baltimore). 2020 Aug 21;99(34):e21659. doi: 
      10.1097/MD.0000000000021659.