Roberta Sanfilippo1, Angelo P Dei Tos, Paolo G Casali. 1. Adult Mesenchymal Tumor Medical Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy. roberta.sanfilippo@istitutotumori.mi.it
Abstract
PURPOSE OF REVIEW: 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. RECENT FINDINGS: 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. SUMMARY: 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.
PURPOSE OF REVIEW: 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. RECENT FINDINGS: 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. SUMMARY: 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: Marieke A de Graaff; Jamie S E Yu; Hannah C Beird; Davis R Ingram; Theresa Nguyen; Jeffrey Juehui Liu; Svetlana Bolshakov; Károly Szuhai; Pierre Åman; Keila E Torres; Dina Lev; Torsten O Nielsen; Judith V M G Bovée; Alexander J Lazar; Neeta Somaiah Journal: Lab Invest Date: 2016-06-06 Impact factor: 5.662
Authors: Marieke A de Graaff; Shruti Malu; Irma Guardiola; Alwine B Kruisselbrink; Yvonne de Jong; Willem E Corver; H Gelderblom; Patrick Hwu; Torsten O Nielsen; Alexander J Lazar; Neeta Somaiah; Judith V M G Bovée Journal: Transl Oncol Date: 2017-06-24 Impact factor: 4.243