Angiosarcomas of the head and neck: a clinico-immunohistochemical study of 8 consecutive patients.

ABSTRACT

Angiosarcoma, also known as malignant hemangioendothelioma, is a rare and aggressive malignant vascular tumour arising from endothelial cells, which accounts for approximately 10% of soft tissue sarcomas in the head and neck. Between October 1996 and July 2008, 10 patients were diagnosed with angiosarcomas (AS) in the head and neck region, 8 of whom were included in this study (there were 7 high-grade and 1 low-grade lesions). AS were characteristically positive for vascular markers such as CD31, CD34, and factor VIII. CD31 was thought to be the most sensitive and specific marker for AS and was positive in 6 patients. Six patients were treated surgically with or without postoperative radiotherapy and/or chemotherapy. Two patients had large and extensive lesions that were considered to be inoperable, they were given palliative chemotherapy and/or radiotherapy. Of the 8 patients reviewed in this study, 5 died of local recurrence or distant metastasis with a survival time of 8-19 months, 2 patients are alive with disease and 1 patient is free of disease. The predeliction for local recurrence and distant metastasis and the high-grade characteristics of this tumour seemed to be correlated to the poor prognosis, although the small number of patients prevented statistical analysis.