title: Myxoid liposarcoma and the mammalian target of rapamycin pathway.
abstract: OBJECTIVE
  Myxoid/round cell liposarcoma (MRCL) represents about 10% of all soft-tissue sarcomas. Therapeutic options for this subgroup of tumours are limited, essentially doxorubicin-based regimens and trabectedin. Recently, the mammalian target of rapamycin (mTOR) pathway has been identified as a therapeutic target in several sarcomas. MRCLs should be included among these, as various molecular aberrations of the mTOR pathway have been recently reported.
  
  
  RESULTS
  PI3KCA mutations were identified in 10-20% of MRCLs. Other molecular aberrations include loss of PTEN, Akt activation and overexpression of IGF1R. Recently, two minor responses to mTOR inhibitors were reported.
  
  
  CONCLUSIONS
  The relatively high frequency of mTOR signalling pathway alterations in MRCL provides a preclinical rationale for considering mTOR inhibition as a potential novel therapeutic strategy warranting further investigation.
authors.affiliation: Adult Mesenchymal Tumor Medical Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy. roberta.sanfilippo@istitutotumori.mi.it
authors.forename: Roberta
authors.initials: R
authors.lastname: Sanfilippo
authors.forename: Angelo P
authors.initials: AP
authors.lastname: Dei Tos
authors.forename: Paolo G
authors.initials: PG
authors.lastname: Casali
mesh_terms: D006801:Humans; D018208:Liposarcoma, Myxoid / Q000188:drug therapy* / Q000235:genetics / Q000378:metabolism*; D058990:Molecular Targeted Therapy; D015398:Signal Transduction / Q000187:drug effects; D058570:TOR Serine-Threonine Kinases / Q000378:metabolism*
chemical_list: C546842:MTOR protein, human; D058570:TOR Serine-Threonine Kinases