Capicua Transcriptional Repressor-Rearranged Undifferentiated Round Cell Sarcoma Metastatic to the Brain Treated with Surgery and Stereotactic Radiosurgery

Ahmed Helal, Christopher S. Graffeo, Avital Perry, John C. Cheville, Michael J. Link, Bruce E. Pollock

Research output: Contribution to journalArticle

Abstract

Background: Ewing-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 Description: A 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. Conclusion: CIC-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.

Original languageEnglish (US)
Pages (from-to)12-19
Number of pages8
JournalWorld neurosurgery
Volume139
DOIs
StatePublished - Jul 2020

Keywords

  • Brain metastasis
  • CIC rearrangement
  • Ewing-like sarcoma
  • Gamma knife
  • Radiosurgery

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

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