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Front. Oncol. | doi: 10.3389/fonc.2018.00046

Cutaneous angiosarcoma: the possibility of new treatment options especially for patients with large primary tumor.

 Yasuhiro Fujisawa1*, Koji Yoshino2,  Taku Fujimura3,  Yoshiyuki Nakamura1, Naoko Okiyama1, Yosuke Ishitsuka1,  Rei Watanabe1 and Manabu Fujimoto1
  • 1Dermatology, University of Tsukuba, Japan
  • 2Dermatology, Tokyo Metropolitan Komagome Hospital, Japan
  • 3Dermatology, Tohoku University, Japan

The most widely accepted treatment for cutaneous angiosarcoma (CAS) is wide local excision and postoperative radiation to decrease the risk of recurrence. Positive surgical margins and large tumors (T2, >5cm) are known to be associated with poor prognosis. Moreover, T2 tumors are known to be associated with positive surgical margins. According to previous reports, the majority of CAS patients in Japan had T2 tumors, whereas less than half of the patients in the studies from western countries did so. Consequently, the reported 5-year overall survival of Japanese CAS patients without distant metastasis was only 9%, lower than that for stage-IV melanoma.
For patients with T2 tumors, management of subclinical metastasis should be considered when planning the initial treatment. Several attempts to control subclinical metastasis have been reported, such as using adjuvant/neoadjuvant chemotherapy in addition to conventional surgery plus radiation. Unfortunately, those attempts did not show any clinical benefit. Besides surgery, new chemotherapeutic approaches for advanced CAS have been introduced in the past couple of decades, such as paclitaxel and docetaxel. We proposed the use of chemoradiotherapy (CRT) using taxanes instead of surgery plus radiation for patients with T2 tumors without distant metastasis, and showed a high response ratio with prolonged survival. However, this prolonged survival was seen only in patients who received maintenance chemotherapy after CRT, indicating that continuous chemotherapy is mandatory to control subclinical residual tumors.
With the recent development of targeted drugs for cancer, many potential drugs for CAS are now available. Given that CAS expresses a high level of vascular endothelial growth factor (VEGF) receptor, drugs that target VEGF signaling pathways such as anti-VEGF monoclonal antibody and tyrosine kinase inhibitors are also promising, and several successful treatments have been reported. Besides targeted drugs, several new cytotoxic anticancer drugs such as eribulin or trabectedin have also been shown to be effective for advanced sarcoma. However, most of the clinical trials did not include a sufficient number of CAS patients. Therefore, clinical trials focusing only on CAS should be performed to evaluate the effectiveness of these new drugs.

Keywords: Cutaneous angiosarcoma, concurrent chemoradiotherapy, Maintenance Chemotherapy, Adjuvant chemotherapy, taxanes, eribulin, Pazopanib, surgery followed by radiation, angiosarcoma of the scalp

Received: 23 Dec 2017; Accepted: 14 Feb 2018.

Edited by:

Atsushi Otsuka, Kyoto University, Japan

Reviewed by:

Toshihiko Hoashi, Nippon Medical School, Japan
Takuya Miyagi, University of the Ryukyus, Japan
Shigeto Matsushita, Kagoshima Medical Center (NHO), Japan  

Copyright: © 2018 Fujisawa, Yoshino, Fujimura, Nakamura, Okiyama, Ishitsuka, Watanabe and Fujimoto. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: MD, PhD. Yasuhiro Fujisawa, University of Tsukuba, Dermatology, 1-1-1 Tennodai, Tsukuba, 3058577, Ibaraki, Japan,