Primary cerebral angiosarcoma: a case report

Abstract

Introduction and importance: Central nervous system sarcomas are rare tumors of mesenchymal origin. Angiosarcomas are an even rarer subtype with poor prognosis and no consensus regarding therapeutic approach. Case presentation: This article presents the case of a 50-year-old Palestinian female patient with a history of treated breast cancer who presented to the emergency room with a tonic-clonic seizure. Brain computed tomography showed a cerebral space-occupying lesion managed with craniotomy and gross tumor resection. Histopathology revealed an epithelioid cerebral angiosarcoma. The patient was treated with concurrent chemoradiotherapy with temozolomide resulting in the resolution of the tumor as well as symptoms and complaints. Clinical discussion: Primary cerebral epithelioid angiosarcoma is a highly malignant tumor of an unknown etiology. The patient history of breast created an additional challenge, as recurrence with metastasis had to be excluded. Signs of blood product degradation are an important radiological feature. According to existing literature, gross tumor resection followed by concurrent chemoradiotherapy offers the best approach and outcome. Conclusion: Cerebral angiosarcoma is a rare disease with a challenging therapeutic approach due to the scarcity of available literature. It should be included in the differential diagnosis of space-occupying lesions, especially in those with a history of exposure to radiotherapy. Immunohistochemistry is key for diagnosis. Surgical resection followed by concurrent chemoradiotherapy is associated with a longer disease-free survival when compared to either option alone.