The adipogenic origin-derived liposarcoma (LPS) family is the most common soft tissue sarcoma histological subtype. This group is composed of three categories as per the 2002 WHO guidelines: (1) well-differentiated and dedifferentiated liposarcoma (WDLPS/DDLPS); (2) myxoid and round cell liposarcoma (MLS and RCL); and (3) pleomorphic liposarcoma (PLS). While clustered together, these histological subtypes are widely diverse in their clinical, pathological, and molecular characteristics. In general, surgery still remains the mainstay of LPS therapy and the only approach offering the potential of cure. Effective therapeutic strategies for locally advanced and metastatic disease are currently lacking and are crucially needed. With the current gradually increasing knowledge of LPS genetic- and epigenetic-associated deregulations, the ultimate goal is to develop drugs that can specifically eliminate LPS cells while sparing normal tissues. This tumor-tailored target-orientated approach will hopefully result in a significant improvement in the outcome of patients suffering from these poor prognosis malignancies.
- Targeted therapy