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Original Article
- Sarcoma
- Real-World Clinical Outcomes and Prognostic Factors for Patients with Advanced Angiosarcoma who Received Systemic Treatment
- Changhee Park, Miso Kim, Yoonjin Kwak, Kyung Chul Moon, Se Hyun Kim, Bhumsuk Keam, Yu Jung Kim, Tae Min Kim, Dong-Wan Kim
- Cancer Res Treat. 2021;53(4):1195-1203. Published online February 1, 2021
- DOI: https://doi.org/10.4143/crt.2020.1337
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Abstract
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Supplementary Material
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ePub - 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. -
Citations
Citations to this article as recorded by
- A systematic review and meta-analysis of pazopanib efficacy and adverse effects in sarcomas
Fernanda Picozzi, Alessandro Ottaiano, Antonella Lucia Marretta, Mariachiara Santorsola, Lucia Cannella, Antonio Pizzolorusso, Francesco Caraglia, Giuseppina Della Vittoria Scarpati, Alessandra Bracigliano, Ottavia Clemente, Ines Simeone, Michele Caraglia
Journal of Translational Medicine.2026;[Epub] CrossRef - Radiation-Induced Angiosarcoma Following Breast Surgery: A Case Report
Mutsumi Hayashi, Yukino Kobayashi, Ryoko Semba, Kanako Ogura, Fumi Murakami, Kiyomi Kimura
Surgical Case Reports.2026; 12(1): n/a. CrossRef - Combination of Immune Checkpoint Inhibitors and Tyrosine Kinase Inhibitors for the Treatment of Late Stage Diffuse Primary Hepatic Angiosarcoma: a Case Report and Literature Review
Ruolin Lyu, Hongyang Shu, Xiaofan Wu, Yiwen Liu, Ming Wang
SN Comprehensive Clinical Medicine.2026;[Epub] CrossRef - Angiosarcoma in a soft tissue sarcoma cohort: real-world patterns and outcomes
Kübra Canaslan, Özge Yetginoğlu, Hasan Oğuz Çetinayak, Emine Burçin Tuna, Tuğba Yavuzşen
Frontiers in Oncology.2026;[Epub] CrossRef - Punch Biopsy as a Diagnostic Keystone for Metastatic Cardiac Angiosarcoma Treated With Anthracycline-Based Chemotherapy: A Case Report
Aonghus Joyce, Gráinne Murphy, Cynthia C Heffron, David Aherne, Richard M Bambury
Cureus.2025;[Epub] CrossRef - Clinicopathological features, treatment outcomes, and prognostic factors of angiosarcoma: a 21-year experience at one center
Chenyan Fang, Xiaoting Zeng, Qing Ji, Tao Zhu, Meiyu Fang, Jun Cao
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The Journal of Dermatology.2025;[Epub] CrossRef - Primary Jejunal Angiosarcoma
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Haigan.2024; 64(7): 917. CrossRef - Hepatic Angiosarcoma with Peliosis Hepatis
Kensuke Kitsugi, Kazuhito Kawata, Moe Matsumoto, Masahiro Umemura, Tomohiko Hanaoka, Maho Yamashita, Shingo Takatori, Jun Ito, Kazuyoshi Ohta, Takeshi Chida, Hidenao Noritake, Takafumi Suda
Internal Medicine.2023; 62(8): 1157. CrossRef - Conversion surgery for recurrent hepatic angiosarcoma after systemic chemotherapy with paclitaxel
Yuta Ushida, Takafumi Sato, Tomotaka Kato, Yasuyuki Shigematsu, Hiromichi Ito, Takeshi Suzuki, Yosuke Inoue, Yoshihiro Ono, Atsushi Oba, Yu Takahashi
Clinical Journal of Gastroenterology.2022; 15(2): 427. CrossRef - Management of Cutaneous Angiosarcoma: an Update Review
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Current Treatment Options in Oncology.2022; 23(2): 137. CrossRef - Results of NC-6300 (Nanoparticle Epirubicin) in an Expansion Cohort of Patients with Angiosarcoma
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The Oncologist.2022; 27(10): 809. CrossRef
- A systematic review and meta-analysis of pazopanib efficacy and adverse effects in sarcomas
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Case Report
- A Case of Angiosarcoma of the Maxillary Sinus
- Young Chang Choi, Kun Ho So, Chun Dong Kim, Jeong Rye Kim, Bong Seog Kim
- J Korean Cancer Assoc. 1999;31(1):194-200.
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Abstract
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- Angiosarcomas are extremely rare in the nasal cavity and para-nasal sinus. Upto the present only nineteen cases have been reported in the literature. A 31-year-old male presented with right facial swelling and large oral cavity mass. He was treated with wide excision of the tumor by radical maxillectomy. The tumor was diagnosed as angiosarcoma by immunohistochemical staining with factor VIII-reactive antigen. No adjuvant radiotherapy after surgery was done. After 9 montbs from surgery, the tumor recurred in the primary sites of oral cavity and right maxillary sinus and metastasized to the both lungs. He was treated with palliative chemotherapy and showed a stable disease in follow-up magnetic resonance image of para-nasal sinus and simple chest X-ray after second cycle of chemotherapy. He is undergoing further chemotherapy without any problems. We report first in Korea a case of primary angiosarcoma of the right maxillary sinus in a 31-year-old male.
- 3,435 View
- 46 Download
Original Article
- A Case of Primary Splenic Angiosarcoma Associated with Kasabach-Merritt Syndrome
- Jin Seok Jeon, Gyu Taek Lee, Ki Ju Han, Jae Ho Byun, Seung Kyu Park, Jong Ho Won, Seung Ho Baick, Dae Sik Hong, Hee Sook Park, Hae Kyung Lee, So Young Jin
- J Korean Cancer Assoc. 1997;29(2):352-357.
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Abstract
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- PURPOSE
Primary malignant vascular neoplasms of the spleen are rare. It has been known that the prognosis was very poor and the splenectomy before rupture could increase survival. No effective chemotherapeutic protocol for angiosarcomas has yet to be established but patients with or without metastatic disease may be treated by chemotherapy. MATERIAL AND METHODS: We experienced a case of primary splenic angiosarcoma in a 42-year-old woman with multiple purpuric skin rashes associated with consumptive coagulopathy:the Kasabach-Merritt syndrome. The CT showed spleen is diffusely enlarged and inhomogenously enhanced with multiple metastasis in the liver. The splenectomy was done and angiosarcoma was diagnosed. We treated her with conventional combination chemotherapy and obtained partial response. For additional response, high-dose chemotherapy and stem cell rescue with autologous peripheral blood stem cell transplantation was done.
RESULT
Afer splenectomy, platelet count return to normal. The follow up abdominal CT scan after treatment showed complete disappeared multiple metastatic lesions in the both lobe of liver and the patient has continued to do well four months following discharge.
CONCLUSION
We herein report our experience of a splenic angiosarcoma whose multiple hepatic metastases responded well to the high-dose chemotherapy.
- 4,275 View
- 18 Download
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