CS-14 A CASE OF CIC-REARRANGED INTRACRANIAL SARCOMA

INTRODUCTION: Intracranial sarcoma is extremely rare among primary brain tumors and often misdiagnosed. Its standard treatment is yet to be established, and treatment options are discussed on a case-by-case basis. Here we report our recent case of intracranial sarcoma review the relevant literature. CASE ILLUSTRATION: A 57-year-old right-handed man presented with headache and was found to have a 5cm mass in the right frontal lobe. Gross total resection was achieved without complications. Given the local pathological diagnosis being glioblastoma, adjuvant radiotherapy with concurrent temozolomide was administered. Further pathological examination revealed Capicua (CIC) rearrangement on FISH, which lead to the diagnosis of sarcoma. No further treatment was pursued at that time. However, he noticed rapid decline in the right visual acuity 7 months from the initial diagnosis. MRI demonstrated a rapidly-growing mass in the right optic nerve sized 1.5cm, which was depicted as a high uptake area on FDG-PET, suggestive of recurrence. Two cycles of chemotherapy with vincristine, ifosfamide, doxorubicin, and etoposide as well as GammaKnife stereotactic radiosurgery were performed with partial response. Sustained myelosuppression and debilitating constitutional symptoms precluded additional chemotherapy. No further recurrence was noted 1 year after diagnosis. CONCLUSION: We have recently experienced a case of CIC-rearranged intracranial sarcoma. FISH was useful in detecting CIC rearrangement and reaching the correct pathological diagnosis. Rapid recurrence of the tumor was noted, but well controlled with radiochemotherapy.