Natural history and literature review of primary intracranial CIC-rearranged sarcomas: case report

Abstract

Intracranial sarcomas are particularly rare primary central nervous system malignancies. Here we report the complete oncologic course of a patient diagnosed with a primary intracranial CIC-rearranged sarcoma with subsequent intracranial spread. A 36-year-old female patient with a prior history of melanoma presented to the emergency department with sudden-onset headaches and paresthesia. After workup demonstrated an intracranial lesion, she underwent gross total resection, with pathology showing primary intracranial sarcoma. She completed adjuvant radiation with an excellent local response. Unfortunately, she developed distant intracranial parenchymal lesions, managed by further resection, radiotherapy, and systemic therapy. One year after initial diagnosis, she presented with profoundly altered mental status with both local and distant intracranial failure, and she passed away a few days later. CIC-rearranged sarcomas are very rare malignancies and should be managed by a multidisciplinary team. While aggressive local failures are reported in these malignancies, here we report an anomalous pattern of rapid distant intracranial spread.