By: Ed Susman

HOLLYWOOD, FL – Although definite clinical trial data are lacking, doctors inserted into guidelines possible treatments for rare soft-tissue sarcomas at the 15th Annual National Comprehensive Cancer Network Conference on Clinical Practice Guidelines and Quality Cancer Care.

“This listing is based upon public literature and academic public presentations,” said George Demetri, MD, Center for Sarcoma and Bone Oncology at the Dana Farber Cancer Institute, Boston, Massachusetts. The listing includes all therapeutic agents approved for some indication by the US Food and Drug Administration. The list does not include any investigational agents, he said during his presentation at the meeting.

Some of the sarcomas — such as perivascular epithelioid cell tumour, for example, are so rare that only a handful of cases are reported each year. He said that treatment with sirolimus appears to have some activity in this disease.

He noted that most of the recommendations in the list involve low-level evidence of effectiveness — they do not meet the highest criteria of controlled clinical trials. In some cases, clinical trials have been performed that establish treatment preferences, such as the use of imatinib in treatment of gastrointestinal stromal tumours.

“The National Comprehensive Cancer Network believes that the best management of any cancer patient is in a clinical trial; participation in a clinical trial is especially encouraged,” the guidelines state.

Among the other disease states, and active agents used in those diseases are:

· Pigmented villonodular synovitis: Imatinib.
· Desmoid tumours: Sulindac, or other nonsteroidal anti-inflammatory drugs, including celecoxib; tamoxifen; toremifene; methotrexate and vinblastine; low-dose interferon; doxorubicin-based regimens; and imatinib.
· Angiosarcomas: Paclitaxel, docetaxel, vinorelbine, sorafenib, sunitinib, bevacizumab, and other systemic therapy options suggested for extremity sarcoma.
· Solitary fibrous tumours: Bevacizumab and temozolomide; sunitinib.
· Alveolar soft part sarcoma: Sunitinib.
· GIST: Imatinib, sunitinib, sorafenib, nilotinib, and dasatinib.

- MFP Wire Services
- 03-18-2010