Primary angiosarcoma of the cranial vault: a case report and review of the literature

Angiosarcoma is a rare neoplasm. It is most likely to affect the scalp in elderly people and involvement of the cranium is uncommon. We report a case of primary malignant angiosarcoma of the cranial vault. An 82-year-old man presented with right hemiparesis, deformity of the cranium and mildly swollen scalp. Further studies disclosed bilateral parietal lesions, with destruction of the dura matter and infiltration of the brain parenchyma. Thrombocytopenia was found. Faint enhancement of the lesions was seen on magnetic resonance imaging (MRI) after IV administration of contrast medium with gadolinium. The tumor was excised including the affected portion of the skull and dura matter. Histologic examination revealed typical pictures of angiosarcoma with immunohistochemical evidence of factor VIII-related antigen. Postoperative radiotherapy was employed. The patient remained well at 10 months postoperative follow up. Primary angiosarcoma of the cranium is sometimes difficult to differentiate from trauma on clinical examination. We review the literature pertaining to the pathogenesis, clinical course and treatment of this condition. Determination of platelet count may be helpful in monitoring the progression of the disease. The tumor may show faint enhancement on MRI.