Table 3. Models of sarcomas generated upon the expression of specific fusion proteins in MSCs or mesenchymal lineages.
| Sarcoma type1 |
Oncogenic events2 |
Cell/tissue of origin |
Reference |
|
|---|---|---|---|---|
| Fusion gene | 2nd hit | |||
| Mouse MSCs | ||||
| Ewing's sarcoma | EWS-FLI-1 | p53 loss | BM-mMSCs | 54 |
| Ewing's sarcoma | EWS-FLI-1 | - | BM-mMSCs | 55 |
| Myxoid liposarcoma | FUS-CHOP | - | BM-mMSCs | 65 |
| Liposarcoma | FUS-CHOP | p53−/− | mASCs | 40 |
| Alveolar rhabdomyosarcoma | PAX3/7-FKHR | p53dn (±H-RAS) or SV40-LT (±H-RAS) | BM-mMSCs | 74 |
| Mouse models3 | ||||
| Ewing's sarcoma | EWS-FLI-1 | p53−/− | Mesenchymal cells of limb buds | 58 |
| Liposarcoma | FUS-CHOP | - | Ubiquitous | 66 |
| Liposarcoma | CHOP-FUS | - | Ubiquitous | 67 |
| Liposarcoma | FUS + CHOP | - | Ubiquitous | 68 |
| Alveolar rhabdomyosarcoma | PAX3-FKHR | p53−/− or INK4A/ARF−/− | Differentiated muscle cells (MYF6-expressing cells) | 76 |
| Synovial sarcoma | SYT-SSX2 | - | Immature myoblasts (MYF5-expressing cells) | 78 |
| Human MSCs | ||||
| NT | EWS-FLI-1 | - | BM-hMSCs | 59 |
| NT | FUS-CHOP | p53 depletion | hASCs | 40 |
| NT | SYT-SSX1 | - | BM-hMSCs | 80 |
1Type of tumors observed upon inoculation in immunodeficient mice. NT: no tumors formed upon inoculation in immunodeficient mice.
2Transforming hits used to achieve tumoral transformation.
3In mouse models where distinct types of tumors were reported only the most represented one is denoted.