• Ahmed Helal, Christopher S Graffeo, Avital Perry, John C Cheville, Michael J Link, and Bruce E Pollock.
• Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, USA. Electronic address: Helal.ahmed@Mayo.edu.
• World Neurosurg. 2020 Jul 1; 139: 12-19.

BackgroundEwing-like sarcoma with capicua transcriptional repressor (CIC) rearrangement is a unique class of undifferentiated round cell sarcomas characterized by CIC-double homeobox 4 gene fusion. Despite showing great histologic resemblance to Ewing sarcomas, they have proved to be a distinct pathological entity from the immunohistochemistry and genetic examinations and the response to treatment. We have presented a case of CIC-rearranged Ewing-like sarcoma with cerebral metastasis managed with operative resection and gamma knife radiosurgery.Case DescriptionA 56-year-old woman had initially presented with an ulcerating lesion of the right fifth toe. The histological and immunohistochemical analysis revealed features consistent with CIC-rearranged Ewing-like sarcoma, which was confirmed with genetic analysis. Despite aggressive local control and a multidrug chemotherapy regimen, the patient developed multifocal metastases involving the lungs, femur, and cerebrum. The cerebral lesions were managed with surgery and gamma knife radiosurgery, with mixed results.ConclusionCIC-rearranged Ewing-like sarcomas have recently been recognized as a distinct disease entity with a highly aggressive course. Treatment paradigms have yet to be defined to properly manage such an aggressive pathological process.Copyright © 2020 Elsevier Inc. All rights reserved.

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