Cancer Res Treat.  2021 Oct;53(4):1195-1203. 10.4143/crt.2020.1337.

Real-World Clinical Outcomes and Prognostic Factors for Patients with Advanced Angiosarcoma who Received Systemic Treatment

• Park C
• Kim M
• Kwak Y
• Moon KC
• Kim SH
• Keam B
• Kim YJ
• Kim TM
• Kim DW

• KMID: 2521594
• DOI: http://doi.org/10.4143/crt.2020.1337

Abstract

Purpose
Angiosarcoma is a highly aggressive mesenchymal tumor. Although systemic chemotherapy is often considered for the inoperable or metastatic angiosarcoma, the outcome of such treatment is unsatisfactory and poorly delineated.
Materials and Methods
We reviewed electronic medical records of 75 patients with angiosarcoma who were treated with systemic chemotherapy for inoperable or metastatic disease. Patients were classified as having liver involvement if they had either primary or metastatic hepatic lesions.
Results
Among the patients evaluated, 51 patients were male (68%) and 24 patients (32%) had primary cutaneous angiosarcoma. Liver involvement was present in 28 patients (37.3%). A total of 59 patients received first-line weekly paclitaxel (wPac) and showed an objective response rate (ORR) of 23.7% (n=14), a median progression free survival (mPFS) of 4.0 months (95% confidence interval [CI] 3.0–6.1), and a median overall survival (mOS) of 10.2 months (95% CI 7.0–14.6). Among patients without liver involvement, patients receiving wPac (n=35) had significantly prolonged mPFS (5.8 vs. 3.2 months, respectively, p=0.014) with a tendency for prolonged mOS (13.8 vs. 11.6 months, respectively, p=0.13) than those receiving other regimens (n=12). A total of 24 patients received second- or later-line pazopanib monotherapy and showed an ORR of 16.7% (n=4), a mPFS of 2.4 months (95% CI 1.8–4.3) and a mOS of 5.4 months (95% CI 3.5–NA).
Conclusion
Treatment with first-line wPac and later-line pazopanib seems to provide survival benefit, especially for patients with advanced angiosarcoma without liver involvement.

Keyword

Angiosarcoma; Paclitaxel; Pazopanib; Prognosis

Figure

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  Fig. 1 Overall survival of advanced angiosarcoma patients treated with systemic chemotherapy and the associated prognostic factors. Kaplan-Meier survival curves represent overall survival of advanced angiosarcoma patients. (A) Overall survival of patients included in the study. (B) Blue line represents patients without any liver involvement; red line represents patients with primary hepatic angiosarcoma; green line represents patients with liver metastasis. (C) Blue line represents patients without any liver involvement; red line represents patients with liver involvement. Censored data are marked with vertical segments and numbers at risk are demonstrated on the table at the bottom of each plot.

• 

  Fig. 2 Progression-free survival and overall survival of advanced angiosarcoma patients treated with first-line weekly paclitaxel (wT) or other regimens. Kaplan-Meier survival curves represent progression-free survival and overall survival of advanced angiosarcoma patients. (A, B) Survival curves show progression-free survival and overall survival of patients according to response to first-line wT. Blue line represents the progression-free survival of all patients who received first-line wT. Red dashed line represents non-responder and green dashed line represents responder. (C, D) Survival curves show progression-free survival and overall survival of patients without liver involvements according to the chemotherapy regimens. Blue line represents patients who received first-line wT and red line represents patients who received other regimens. Censored data are marked with cross segments and numbers at risk are demonstrated on the table at the bottom of each plot.

• 

  Fig. 3 Progression-free survival and overall survival of advanced angiosarcoma patients treated with second- or later-line pazopanib. Kaplan-Meier survival curves represent progression-free survival and overall survival of advanced angiosarcoma patients. (A, B) Survival curves show progression-free survival and overall survival of patients according to response to second- or later-line pazopanib. Blue lines represent the progression-free survival and overall survival of all patients who received first-line weekly paclitaxel. Red lines represent non-responder and green lines represent responder. Censored data are marked with cross segments and numbers at risk are demonstrated on the table at the bottom of each plot.

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