PMID- 21139932
OWN - NLM
STAT- PubMed-not-MEDLINE
DCOM- 20110714
LR  - 20211020
IS  - 2036-3613 (Electronic)
IS  - 2036-3605 (Print)
IS  - 2036-3605 (Linking)
VI  - 1
IP  - 2
DP  - 2009 Dec 28
TI  - Temsirolimus in the treatment of renal cell carcinoma associated with Xp11.2 
      translocation/TFE gene fusion proteins: a case report and review of literature.
PG  - e53
LID - 10.4081/rt.2009.e53 [doi]
LID - e53
AB  - Xp11.2 translocation renal cell carcinomas (TRCCs) are a rare family of tumors 
      newly recognized by the World Health Organization (WHO) in 2004. These tumors 
      result in the fusion of partner genes to the TFE3 gene located on Xp11.2. They 
      are most common in the pediatric population, but have been recently implicated in 
      adult renal cell carcinoma (RCC) presenting at an early age. TFE3-mediated direct 
      transcriptional upregulation of the Met tyrosine kinase receptor triggers 
      dramatic activation of downstream signaling pathways including the protein kinase 
      B (Akt)/phosphatidylinositol-3 kinase (PI3K) and mammalian target of rapamycin 
      (mTOR) pathways. Temsirolimus is an inhibitor of mammalian target of rapamycin 
      (mTOR) kinase, a component of intracellular signaling pathways involved in the 
      growth and proliferation of malignant cells. Here we present a case of a 22-year 
      old female who has been treated with temsirolimus for her Xp11.2/TFE3 gene fusion 
      RCC.
FAU - Parikh, Jigarkumar
AU  - Parikh J
AD  - Department of Medicine;
FAU - Coleman, Teresa
AU  - Coleman T
FAU - Messias, Nidia
AU  - Messias N
FAU - Brown, James
AU  - Brown J
LA  - eng
PT  - Case Reports
DEP - 20091228
PL  - England
TA  - Rare Tumors
JT  - Rare tumors
JID - 101526926
PMC - PMC2994474
OTO - NOTNLM
OT  - Xp11.2 translocation
OT  - renal cell carcinoma
OT  - temsirolimus.
EDAT- 2009/01/01 00:00
MHDA- 2009/01/01 00:01
PMCR- 2009/12/28
CRDT- 2010/12/09 06:00
PHST- 2009/10/12 00:00 [received]
PHST- 2009/10/16 00:00 [accepted]
PHST- 2010/12/09 06:00 [entrez]
PHST- 2009/01/01 00:00 [pubmed]
PHST- 2009/01/01 00:01 [medline]
PHST- 2009/12/28 00:00 [pmc-release]
AID - rt.2009.e53 [pii]
AID - 10.4081/rt.2009.e53 [doi]
PST - epublish
SO  - Rare Tumors. 2009 Dec 28;1(2):e53. doi: 10.4081/rt.2009.e53.