Bulletin du Cancer
MENUTargeted therapy of sarcomas Volume 95, issue 10, octobre 2008
- Key words: sarcoma, gastrointestinal stroma tumors, GIST, chemotherapy, targeted therapy, angiogenesis, mTOR, KIT, PDGFR
- DOI : 10.1684/bdc.2008.0730
- Page(s) : 963-74
- Published in: 2008
Recent progress made in the field of sarcoma biology has shed new light on the pathophysiology of these numerous but rare diseases. Soft tissue sarcomas can be divided into 6 sub-types based on the underlying molecular biology of the disease : 1) translocation leading to fusion proteins involving transcription factors or growth factors (Ewing sarcoma, myxoid liposarcoma, dermatofibrosarcoma protuberans) ; 2) tyrosine kinase receptor mutations (gastrointestinal stromal tumors) ; 3) tumor-suppressor gene deletion (type 1 neurofibromatosis, rhabdoid tumors) ; 4) genetic alteration such as amplification of chromosomal regions (well differentiated/dedifferentiated liposarcoma) ; 5) sarcomas with more complex genetic alterations (leiomyosarcoma) and 6) abnormalities involving the cell-adhesion pathways (aggressive fibromatosis). Together with the current development of numerous targeted therapies, these recent progress are the basis of tomorrow’s personalised medicine for patients with soft tissue sarcoma.