摘要:Leiomyosarcomas are malignant mesenchymal tumours that derive from the smooth muscle lineage.They are studied and frequently treated as if they are the same as other soft tissue sarcomas. Recentdevelopments suggest that a different approach may be more appropriate. Their underlying geneticmechanisms remain unclear, and complex. Unbalanced karyotypic defects are the only shared featuresobserved across different leiomyosarcoma subtypes. Unlike other soft tissue sarcomas, leiomyosarcomasare particularly sensitive to the combination of gemcitabine and docetaxel. Furthermore, treatment withtrabectedin has shown a good efficacy in leiomyosarcomas, mainly in the form of chronic diseasestabilisation.