PMID- 24179658
OWN - NLM
STAT- PubMed-not-MEDLINE
DCOM- 20131101
LR  - 20211021
IS  - 2036-3605 (Print)
IS  - 2036-3613 (Electronic)
IS  - 2036-3605 (Linking)
VI  - 5
IP  - 3
DP  - 2013
TI  - Fatal malignant metastastic epithelioid angiomyolipoma presenting in a young 
      woman: case report and review of the literature.
PG  - e46
LID - 10.4081/rt.2013.e46 [doi]
LID - e46
AB  - Epithelioid angiomyolipomas (EAMLs) are rare mesenchymal tumors whose malignant 
      variant is extremely uncommon and highly aggressive. Treatment strategies include 
      chemo radiation, transcatheter arterial embolization and surgical resection, 
      which has remained the mainstay treatment. Targeted therapies including mammalian 
      target of rapamycin (mTOR) inhibitors such as Temsirolimus may offer some hope 
      for progressive malignant EAMLs that are not amenable to other treatment 
      modalities. We report a fatal case in a young female who presented with rapidly 
      progressive metastatic EAML that did not respond to mTOR therapy. The literature 
      has shown reduction in tumor burden with the use of mTOR inhibitors, but 
      unfortunately due to the rarity of malignant EAML, a meaningful approach to 
      treatment remains challenging.
FAU - Wyluda, Edward
AU  - Wyluda E
AD  - Department of Internal Medicine, Penn State Milton S. Hershey Medical Center , 
      Hershey, PA, USA.
FAU - Baquero, Giselle
AU  - Baquero G
FAU - Lamparella, Nicholas
AU  - Lamparella N
FAU - Abendroth, Catherine
AU  - Abendroth C
FAU - Drabick, Joseph
AU  - Drabick J
LA  - eng
PT  - Case Reports
DEP - 20130926
PL  - England
TA  - Rare Tumors
JT  - Rare tumors
JID - 101526926
PMC - PMC3804821
OTO - NOTNLM
OT  - malignant epithelioid angiomyolipomas
OT  - perivascular epithelioid cell tumors
OT  - targeted therapy
EDAT- 2013/11/02 06:00
MHDA- 2013/11/02 06:01
PMCR- 2013/09/26
CRDT- 2013/11/02 06:00
PHST- 2013/05/21 00:00 [received]
PHST- 2013/05/30 00:00 [accepted]
PHST- 2013/11/02 06:00 [entrez]
PHST- 2013/11/02 06:00 [pubmed]
PHST- 2013/11/02 06:01 [medline]
PHST- 2013/09/26 00:00 [pmc-release]
AID - 10.4081/rt.2013.e46 [doi]
PST - epublish
SO  - Rare Tumors. 2013 Sep 26;5(3):e46. doi: 10.4081/rt.2013.e46. eCollection 2013.