Secondary angiosarcoma following irradiation--case report and review of the literature

Angiosarcoma is a rare malignant tumor that accounts for 1% to 2% of all soft tissue sarcomas. This paper reports a case of radiation-associated angiosarcoma with a literature review of 66 cases. Twenty-nine years after transabdominal hysterectomy and adjuvant radiation therapy for uterine cervix cancer, cutaneous angiosarcoma developed in the abdominal wall of a 63-year-old woman. She underwent wide excision of the abdominal wall mass, radiation therapy, and chemotherapy, and has been alive and well without any evidence of metastasis for 23 months since the initial diagnosis of angiosarcoma. After an intensive literature review, we found 66 cases of radiation-associated angiosarcoma. The most common primary disease was breast cancer (44%), with gynecologic cancer (21%) next. Eighty-five percent of radiation-associated angiosarcomas developed in the cutaneous area. The median age at diagnosis was 65 years, and the median latency period from irradiation to diagnosis was 96 months. The median survival period of 48 patients whose survival lengths could be reviewed was 12 months.