PMID- 25239799
OWN - NLM
STAT- MEDLINE
DCOM- 20141222
LR  - 20211021
IS  - 1432-2307 (Electronic)
IS  - 0945-6317 (Linking)
VI  - 465
IP  - 5
DP  - 2014 Nov
TI  - Perivascular epithelioid cell tumor (PEComa) with TFE3 gene rearrangement: 
      clinicopathological, immunohistochemical, and molecular features.
PG  - 607-13
LID - 10.1007/s00428-014-1655-x [doi]
AB  - Perivascular epithelioid cell tumors (PEComas) have been increasingly associated 
      with gene rearrangement of the transcription factor E3 (TFE3). We present three 
      cases of PEComa with a TFE3 gene abnormality detected by immunohistochemistry 
      (IHC) and fluorescence in situ hybridization (FISH). Their clinical features, 
      pathological morphology, and prognosis were investigated. Histologically, the 
      tumors in these three cases showed predominantly epithelioid cells arranged in 
      nests or sheets separated by a delicate vascular network, within two of the three 
      cases nuclear atypia, mitotic figures, and necrosis. All three cases showed 
      strong TFE3 and cathepsin K immunoreactivity and weak to strong reactivity for 
      HMB45. One case of PEComa with TFE3 gene fusion exhibited a benign course. The 
      other two cases of PEComa with both TFE3 translocation and X-chromosome polysomy 
      were histologically malignant and showed aggressive growth. In summary, unusual 
      cases of PEComa with TFE3 gene rearrangement might present malignant histological 
      features and aggressive clinical behavior. Our results add cases to the 
      literature and describe an association of polysomy with aggressive behavior.
FAU - Shen, Qin
AU  - Shen Q
AD  - Department of Pathology, Jinling Hospital, Southern Medical University, 305 East 
      Zhongshan Road, Nanjing, 210002, China.
FAU - Rao, Qiu
AU  - Rao Q
FAU - Xia, Qiu-Yuan
AU  - Xia QY
FAU - Yu, Bo
AU  - Yu B
FAU - Shi, Qun-Li
AU  - Shi QL
FAU - Zhang, Ru-Song
AU  - Zhang RS
FAU - Zhou, Xiao-Jun
AU  - Zhou XJ
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20140920
PL  - Germany
TA  - Virchows Arch
JT  - Virchows Archiv : an international journal of pathology
JID - 9423843
RN  - 0 (Basic Helix-Loop-Helix Leucine Zipper Transcription Factors)
RN  - 0 (Biomarkers, Tumor)
RN  - 0 (TFE3 protein, human)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Basic Helix-Loop-Helix Leucine Zipper Transcription Factors/*genetics
MH  - Biomarkers, Tumor/metabolism
MH  - Fatal Outcome
MH  - Female
MH  - *Gene Rearrangement
MH  - Humans
MH  - Immunohistochemistry
MH  - In Situ Hybridization, Fluorescence
MH  - Kidney Neoplasms/genetics/pathology
MH  - Middle Aged
MH  - Perivascular Epithelioid Cell Neoplasms/genetics/*pathology
EDAT- 2014/09/23 06:00
MHDA- 2014/12/23 06:00
CRDT- 2014/09/21 06:00
PHST- 2014/07/05 00:00 [received]
PHST- 2014/09/11 00:00 [accepted]
PHST- 2014/08/15 00:00 [revised]
PHST- 2014/09/21 06:00 [entrez]
PHST- 2014/09/23 06:00 [pubmed]
PHST- 2014/12/23 06:00 [medline]
AID - 10.1007/s00428-014-1655-x [doi]
PST - ppublish
SO  - Virchows Arch. 2014 Nov;465(5):607-13. doi: 10.1007/s00428-014-1655-x. Epub 2014 
      Sep 20.