The treatment landscape of advanced angiosarcoma in Asia—A multi‐national collaboration from the Asian Sarcoma Consortium

Abstract

Angiosarcoma (AS) is a rare disease with a dismal prognosis. The treatment landscape and prognostic factors for advanced AS, including locally advanced, unresectable, and metastatic disease remain elusive. The Asian Sarcoma Consortium is an international collaborative effort to understand the sarcoma treatment landscape in Asia. We undertook a retrospective chart review of AS patients seen in 8 sarcoma academic centers across Asia. Patients with complete clinical, treatment, and follow‐up data were enrolled. Overall, 276 advanced AS patients were included into this study; 84 (30%) of the patients had metachronous metastatic AS. The median age was 67 y; primary sites of AS was cutaneous in 55% and visceral in 45% of patients. In total, 143 (52%) patients received at least 1 line of systemic chemotherapy. The most common first‐line chemotherapy regimen used was paclitaxel (47.6%) followed by liposomal doxorubicin (19.6%). The median overall survival (OS) was 7.8 mo. Significant prognostic factors for OS included age > 65 (hazard ratio (HR) 1.54, P = .006), male gender (HR 1.39, P = .02), and a cutaneous primary AS site (HR 0.63, P = .004). The median progression‐free survival (PFS) for first‐line chemotherapy was 3.4 mo. PFS for single vs combination or paclitaxel vs liposomal doxorubicin chemotherapy regimens were comparable. This study provides an insight into the treatment patterns and prognostic factors of advanced AS patients in Asia. Prognosis of advanced AS remains poor. Data from this study serve as a benchmark for future clinical study design.

New nomograms to predict overall and cancer‐specific survival of angiosarcoma

“…And some retrospective studies have shown that adjuvant radiotherapy may improve the local control and survival of localized AS 4,29,30 . For patients who have unresectable or recurrent metastatic AS, the main treatment remains chemotherapy 31 . Thus, both radiotherapy and chemotherapy play essential roles in the treatment of AS and have important clinical significance.…”

“…In the current research, there were already some models of AS. However, these models usually focus on one specific organ 31,39,40 . Remarkably, our nomograms were constructed for AS that occurred throughout the body.…”

“… 4 , 29 , 30 For patients who have unresectable or recurrent metastatic AS, the main treatment remains chemotherapy. 31 Thus, both radiotherapy and chemotherapy play essential roles in the treatment of AS and have important clinical significance. In summary, considering the availability of clinical data, clinical evidence, the limitations of the SEER database, and statistical results in Tables 2 and 3 , we incorporated treatments including surgery, chemotherapy, and radiotherapy into the construction of the nomograms.…”

“…However, these models usually focus on one specific organ. 31 , 39 , 40 Remarkably, our nomograms were constructed for AS that occurred throughout the body. AS accounts for only 1%–2% of STS, and the number of samples is usually small.…”

Albumin-Bound Paclitaxel: Worthy of Further Study in Sarcomas

“…As the first taxanes, paclitaxel was proved to be ineffective in most soft tissue sarcomas, except angiosarcoma, in the 1990s ( 111 ). At present, paclitaxel is the first choice of chemotherapy for vasogenic malignancies ( 112 , 113 ). With the application of targeted drugs in the treatment of sarcoma, the combination of paclitaxel and targeted drugs also shows promising activity ( 114 ).…”