Sorafenib for Patients with Advanced Angiosarcoma: A Phase II Trial from the French Sarcoma Group (GSF/GETO)

Abstract

Background. Angiosarcomas account for <2% of all soft tissue sarcomas. This subtype is one of the most aggressive forms of soft tissue sarcoma. The prognosis for angiosarcoma patients in the advanced phase remains poor with current cytotoxic agents (progression-free survival [PFS] time of ϳ4 months and overall survival [OS] time of ϳ8 months). We investigated the antitumor activity of sorafenib in patients with metastatic or advanced angiosarcomas in a phase II trial.

Spontaneous regression of chemotherapy-resistant metastases from a scalp angiosarcoma

“…For metastatic angiosarcomas, chemotherapy is the primary choice of treatment 9) . Agents reported to be effective against angiosarcoma are PTX 10,11) , DOC 12,13) , gemcitabine 14) , bevacizumab 15) , and sorafenib 16) . However, it is difficult to prevent the disease from progressing to metastatic angiosarcoma.…”

Vascular-targeted agents for the treatment of angiosarcoma

“…In vitro these mutations induced VEGFR2 autophosphorylation sensitive to inhibition with sorafenib and sunitinib [72]. However, a recently published phase II study found no activating mutations of VEGFR2 in 41 patients with advanced angiosarcoma, and no evidence for efficacy of single agent sorafenib in these patients, suggesting angiosarcomas harbouring VEGFR2 mutations are a rare sub-group [107].…”

“…In contrast, only one partial response was seen in the other sarcoma patients. Another phase II study of sorafenib in 41 patients with angiosarcoma reported responses in 4 (10%) [107]. Final results from a phase II study of sunitinib in 48 patients with metastatic soft tissue sarcomas included 2 patients with angiosarcoma; one partial response and 10 stable disease were reported, but none in angiosarcoma [108].…”

Fine-needle aspiration accuracy in the diagnosis of primary epithelioid angiosarcoma of the adrenal gland: a case report and review of the literature

“…Epithelioid angiosarcoma (EA) of the adrenal gland has poor prognosis: 5-year overall survival ranges between 25% and 45% in primary tumors, while is shorter than 12 months in metastatic patients [3]. To our knowledge, just 37 Fine-needle aspiration accuracy in the diagnosis of primary epithelioid angiosarcoma of the adrenal gland been reported in literature. Nevertheless, among them, only 29 are available for full review in English, according to PubMed systemic research through MeSH terms (Table I) .…”