| ALT: adipocytic, sclerosing, and inflammatory |
40–45 |
FISH analysis of MDM2/CDK4
|
Locally aggressive; recurrence is likely to occur if excision is not complete; progression from ALT to DDLPS is reported in 25–40% of patients |
MDM2 amplification; CDK4 amplification; YEATS4 amplification; TKIs |
MDM2 inhibitors: cis-imidazoline analogs (Nutlin-3)/spirooxindoles/AT-219/Ascenta; E2F1 activator: Nutlin-3a; pazopanib/sorafenib |
Strong binding to MDM2 and irreversibly disrupts the MDM2/p53 protein complex; apoptosis Induction in Null-p53 cancer cells; negative P53 regulation; target RAF, VEGFR1-3, PDGFRB, c-kit and flt-3 |
| DDLPS |
Strong propensity for distant lung metastases (10–15%) and recurrence |
| MLPS |
30 |
FISH analysis of aberrant fusion Gene FUS–CHOP/DDIT3 |
Frequent recurrence; 10–20% of patients develop distant metastases |
T(12;16) or t(12;22): FUS-DDIT3/EWSR1-DDIT3/TLS-CHOP fusion; PTEN mutations (loss of function mutations); PI3K mutations (p110 alpha mutations) |
PTEN/AKT inhibitors: nelfinavir |
Localize to the nucleus and the cytoplasm, bind RN, and are also involved in nucleo-cytoplasmic shuttling involved in the induction of transcription; PI3k/AKT pathway dysregulation |
| PLS |
5 |
Histologic analysis |
The rarest and most aggressive LPS subtype; highly resistant to all current treatment options with a very poor clinical outcome; local recurrence in 30–35% of patients; lung is a frequent site of relapse; cutaneous and subcutaneous PLS exhibit a better outcome than that of deep soft tissue, with 17% incidence of local recurrence and rare cases of distant metastases |
Complex karyotype with chromosome arrangements including gains and losses |
p14ARF methylation |
p14ARF methylation in the origin and growth of PLS |
