NIRA-02 CENTRAL NERVOUS SYSTEM TROPISM IN CIC-REARRANGED SARCOMA: A RATIONALE FOR UPFRONT SCREENING
Aron Mebrahtu, Zach Creech, Will Jeck, Nicole Larrier, Juneko Olson, Julia Visgauss, Rory GoodwinAbstract
Background
CIC-rearranged sarcoma is a highly aggressive malignancy with a recognized propensity for central nervous system (CNS) metastasis. However, current guidelines do not recommend routine neuro-axis imaging, potentially resulting in inadequate surveillance and delayed diagnosis.
Methods
We retrospectively analyzed 20 patients with molecularly confirmed CIC-rearranged sarcoma treated at a single institution. The incidence, radiologic patterns, and clinical presentation of CNS metastases were evaluated to assess the efficacy of current symptom-driven imaging practices.
Results
Nine patients (45%) developed CNS metastases during their disease course. Since baseline and interval CNS imaging were not routinely performed, detection was almost exclusively driven by acute neurological symptoms. Consequently, intracranial lesions were frequently hemorrhagic, multifocal, and associated with severe morbidity at the time of discovery. Furthermore, while dedicated spinal imaging was largely absent, systemic imaging revealed direct paraspinal or neural foraminal tumor extension in 20% of the cohort, suggesting a broader neural affinity. Outcomes for patients with CNS involvement were universally poor (median survival post-CNS diagnosis < 3 months).
Conclusions
Symptom-driven imaging fails to identify intracranial disease before it becomes catastrophic. Routine baseline MRI of the brain and spine, followed by interval neuro-axis surveillance, should be recommended for all patients with CIC-rearranged sarcoma.