![]() ![]() Furthermore, because of this rarity, no randomized phase-3 study has been conducted, especially for angiosarcoma, and consequently, no clinical trial-proven standardized treatment has thus far been established. Owing to this rarity, most previous publications have been case reports or small case series, making it difficult to interpret the results because of the selection bias and small number of patients included in those studies. Although the incidence of angiosarcoma has increased in the past couple of decades, it is around 0.5 per 1,000,000 persons, or fewer than 200 new patients, per year in the United States ( 3). Moreover, angiosarcoma accounts for only 1% of all sarcomas, so patients with angiosarcoma constitute only 1 in 10,000 of all patients with malignant neoplasms ( 1– 3). Therefore, clinical trials focusing only on CAS should be performed to evaluate the effectiveness of these new drugs.Īccording to the Surveillance, Epidemiology, and End Results Program database, the number of patients with sarcoma recorded between 20 was only 1/100 of the number of patients with carcinoma in the same period. However, most of the clinical trials did not include a sufficient number of CAS patients. Besides targeted drugs, several new cytotoxic anticancer drugs such as eribulin or trabectedin have also been shown to be effective for advanced sarcoma. 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. With the recent development of targeted drugs for cancer, many potential drugs for CAS are now available. 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. 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. Besides surgery, new chemotherapeutic approaches for advanced CAS have been introduced in the past couple of decades, such as paclitaxel and docetaxel. Unfortunately, those attempts did not show any clinical benefit. Several attempts to control subclinical metastasis have been reported, such as using adjuvant/neoadjuvant chemotherapy in addition to conventional surgery plus radiation. For patients with T2 tumors, management of subclinical metastasis should be considered when planning the initial treatment. Consequently, the reported 5-year overall survival of Japanese CAS patients without distant metastasis was only 9%, lower than that for stage-IV melanoma. 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. ![]() Moreover, T2 tumors are known to be associated with positive surgical margins. Positive surgical margins and large tumors (T2, >5 cm) are known to be associated with poor prognosis. The most widely accepted treatment for cutaneous angiosarcoma (CAS) is wide local excision and postoperative radiation to decrease the risk of recurrence. 3Dermatology, Tohoku University, Sendai, Japan.2Dermatology, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan.1Dermatology, University of Tsukuba, Tsukuba, Ibaraki, Japan.Koji Yoshino 2 Taku Fujimura 3 Yoshiyuki Nakamura 1 Naoko Okiyama 1 Yosuke Ishitsuka 1 Rei Watanabe 1 Manabu Fujimoto 1 ![]()
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