Primary cerebellar angiosarcoma: a rare case report and literature review

Angiosarcoma is a highly aggressive malignant mesenchymal tumor, accounting for less than 1% of all soft tissue sarcomas. While most cases arise in the skin, soft tissues, liver, or heart, primary intracranial angiosarcoma (PIA) is an exceptionally rare and distinct clinicopathological entity. Due to nonspecific clinical presentations and imaging features, PIA poses significant diagnostic and therapeutic challenges. It is characterized by aggressive behavior, with high rates of recurrence and metastasis, resulting in poor overall prognosis. Here, we report a rare case of primary cerebellar angiosarcoma and review its epidemiology, pathogenesis, diagnostic considerations, and treatment strategies. A 53-year-old woman presented with progressive dizziness, occipital headache, nausea, and vomiting for five days. MRI revealed a hemorrhagic, highly vascular right cerebellar mass initially suspected to be a hemangioblastoma. Prior to surgery, her condition deteriorated, and emergency CT showed acute tumor hemorrhage, necessitating urgent resection. Intraoperatively, the tumor was highly vascular and poorly circumscribed, suggestive of a high-grade sarcoma, later confirmed as angiosarcoma. Despite gross total resection, the patient remained comatose due to brainstem compression. Follow-up CT on day 20 revealed rapid tumor recurrence. Given the poor prognosis, the family opted for palliative care, and the patient subsequently passed away. PIA is a rare and aggressive tumor with a poor prognosis, characterized by rapid progression and recurrence. Early detection and surgical resection are essential, but effective adjuvant therapies remain limited. A multidisciplinary approach is crucial to improve outcomes, and further research is needed to refine therapeutic strategies and enhance survival.