Table 3.
Description of responses by histotypes and targeted therapy
| TT1 | n | % | CR | PR | RR (%) | TT2 | n | % | CR | PR | RR (%) | Other PRs (n) | ||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Total | 278* | 3 | 22 | 0 | 5 | 5 | ||||||||
| GIST | 39 | sorafenib | 31 | 79 | 0 | 3 | 10 | nilotinib | 3 | 8 | 0 | 0 | 0 | ─ |
| Leiomyosarcoma | 36 | sorafenib | 22 | 61 | 0 | 3 | 14 | sunitinib | 9 | 25 | 0 | 2 | 22 | ─ |
| Angiosarcoma | 18 | sorafenib | 14 | 78 | 0 | 3 | 21 | sunitinib | 2 | 11 | 0 | 1 | 50 | ─ |
| Unclassified sarcoma | 15 | sunitinib | 8 | 53 | 0 | 1 | 13 | sorafenib | 5 | 33 | 0 | 0 | 0 | ─ |
| Chordoma | 15 | imatinib | 8 | 53 | 0 | 0 | 0 | sunitinib | 3 | 20 | 0 | 0 | 0 | erlotinib (1) |
| Osteosarcoma | 15 | sirolimus | 10 | 67 | 0 | 0 | 0 | sorafenib | 3 | 20 | 0 | 0 | 0 | ─ |
| Synovialosarcoma | 15 | sunitinib | 8 | 53 | 0 | 4 | 50 | sorafenib | 3 | 20 | 0 | 0 | 0 | pazopanib (1) |
| Ewing / PNET | 14 | sunitinib | 8 | 57 | 1 | 0 | 13 | sorafenib | 2 | 14 | 0 | 0 | 0 | sirolimus + cyclop (1) |
| Chondrosarcoma | 12 | sirolimus | 5 | 42 | 0 | 0 | 0 | sorafenib | 3 | 25 | 0 | 0 | 0 | ─ |
| Uterine leiomyosarcoma | 12 | sorafenib | 6 | 50 | 0 | 1 | 17 | sunitinib | 4 | 33 | 0 | 0 | 0 | ─ |
| Liposarcoma | 12 | sorafenib | 7 | 58 | 0 | 0 | 0 | sunitinib | 3 | 25 | 0 | 0 | 0 | ─ |
| Solitary fibrous tumor | 10 | sorafenib | 3 | 30 | 0 | 0 | 0 | sunitinib | 2 | 20 | 0 | 0 | 0 | beva + TMZ (1) |
| Epithelioid sarcoma | 9 | sorafenib | 2 | 22 | 0 | 1 | 50 | sunitinib | 2 | 22 | 0 | 0 | 0 | beva + pacli (1) |
| MPNST | 8 | sorafenib | 4 | 50 | 0 | 0 | 0 | sunitinib | 2 | 25 | 0 | 1 | 50 | ─ |
| ASPS | 8 | sorafenib | 5 | 63 | 0 | 2 | 40 | sunitinib | 3 | 38 | 0 | 0 | 0 | ─ |
| DSRCT | 6 | sorafenib | 3 | 50 | 0 | 0 | 0 | sunitinib | 3 | 50 | 0 | 0 | 0 | ─ |
| AF | 6 | imatinib | 4 | 67 | 0 | 0 | 0 | sorafenib | 1 | 17 | 0 | 1 | 100 | ─ |
| DFSP | 5 | imatinib | 4 | 80 | 1 | 2 | 75 | sunitinib | 1 | 20 | 0 | 0 | 0 | ─ |
| PEComa | 4 | temsirolimus | 2 | 50 | 1 | 1 | 100 | sirolimus | 1 | 25 | 0 | 0 | 0 | ─ |
| Rhabdomyosarcoma | 3 | sunitinib | 2 | 67 | 0 | 0 | 0 | sorafenib | 1 | 33 | 0 | 0 | 0 | ─ |
| KS | 1 | everolimus | 1 | 100 | 0 | 1 | 100 | NA | ─ | ─ | ─ | ─ | ─ | ─ |
| Low grade ESS | 1 | sorafenib | 1 | 100 | 0 | 0 | 0 | NA | ─ | ─ | ─ | ─ | ─ | ─ |
| Phyllode tumor | 1 | sunitinib | 1 | 100 | 0 | 0 | 0 | NA | ─ | ─ | ─ | ─ | ─ | ─ |
| Other (including benign tumors) | 13 | ND | ─ | ─ | ─ | ─ | ND | ─ | ─ | ─ | ─ | ─ | ─ |
Complete and partial responses are indicated in bold.
*represents the total number of treatment lines (some patients had several lines of targeted therapy).
TT1 is the most frequent targeted therapy, TT2 is the second most frequent targeted therapy.
AF: aggressive fibromatosis.
ASPS: alveolar soft parts sarcoma.
beva: bevacizumab.
CR: complete response.
cyclop: cyclophosphamide.
DFSP: dermatofibrosarcoma protuberans.
DSRCT: desmoplastic small round cell tumor.
ESS: endometrial stromal sarcoma.
GIST: gastro-intestinal stromal tumor.
KS: kaposi sarcoma.
MPNST: malignant peripheral nerve sheath tumor.
NA: not applicable.
ND: not described.
pacli: paclitaxel.
PR: partial response.
RR: response rate.
TMZ: temozolomide.
TT: targeted therapy.