Unique Presentation and Diagnostic Challenges in Metastatic Cerebral Angiosarcoma: Case Report

Abstract

Abstract BACKGROUND AND IMPORTANCE Angiosarcoma is a malignant endothelial tumor arising from vascular or lymphatic tissue. Metastatic cerebral angiosarcoma has been rarely reported and is associated with a poor prognosis. CLINICAL PRESENTATION We report a 22-yr-old man who presented with a complex partial seizure. Contrasted brain magnetic resonance imaging (MRI) revealed 3 enhancing masses of the left frontal-parietal junction, right occipital lobe, and right caudate nucleus. Computed tomography (CT) of the chest, abdomen, and pelvis and a cardiac MRI revealed no evidence of primary tumor. Immunohistochemistry following biopsy was positive for the ETS transcription factor ERG, CD31, FLI-1, and FosB, confirming angiosarcoma. CT/positron emission tomography (PET) scan showed skeletal metastases in the left iliac bone, sacrum, and T6 vertebral body. The patient underwent resection of the 2 cortical masses, stereotactic radiosurgery to the intracranial lesions as well as to his oligometastases, and was treated with 9 cycles of paclitaxel and bevacizumab. Brain MRI and CT/PET body imaging after cycle 9 revealed no tumor recurrence or progression. The progression-free and overall survival following the diagnosis of brain metastases was 27 mo. CONCLUSION Neurosurgeons should be aware of the unique presentation and diagnostic challenges in metastatic cerebral angiosarcoma, particularly in cases when the primary tumor site is unknown. Furthermore, combined surgery, ablative radiation, and targeted systemic and biological therapy can result in prolonged survival even in patients with metastases.