Abstract
Angiosarcoma is a rare, highly aggressive tumor. It is a malignant vascular tumor that compromises only approximately 1% of all skin malignancies in Japan. The overall prognosis is poor, with reported 5-year survival rates of approximately 10%. Given the rarity of the tumor, relatively little is known concerning its features, natural history, or optimal treatment. Although surgical resection remains the cornerstone of therapy, because of the pattern of diffuse, clinically undetectable spread, the disease is sometimes difficult to resect completely. Adjuvant radiation therapy has resulted in a significant benefit in local control. Effective systemic treatment options are limited for patients with angiosarcoma. Two taxanes (paclitaxel and docetaxel) demonstrated good efficacy in patients with advanced angiosarcoma. We also reviewed the efficacy of MAID (Mesna/Ifosfamide/Doxorubicin/Dacarbazine) combination chemotherapy in them. Future clinical research should be explored by therapeutic centers for establishing a treatment policy for this rare tumor with a very poor prognosis.[Skin Cancer (Japan) 2009 ; 24 : 385-391]
