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Peer-reviewedCC BY 4.0Frontiers in Oncology

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

  • Arissa Khan1
  • Maryam Khan2
  • Abigail Bowser3
  • Sahara Cathcart4
  • Nicole Shonka5
  • Sean Kelly6
  • Daniel Surdell7
  • Breann Bowar3
  • Danielle Cunningham8
  • Qateeb Khan3
8 affiliations
  1. 1Case Western Reserve University, Cleveland, OH, United States
  2. 2Medical College of Wisconsin, Milwaukee, WI, United States
  3. 3Department of Radiation Oncology, University of Nebraska, Omaha, NE, United States
  4. 4Department of Pathology, University of Nebraska, Omaha, NE, United States
  5. 5Department of Medical Oncology, University of Nebraska, Omaha, NE, United States
  6. 6Department of Radiology, University of Nebraska, Omaha, NE, United States
  7. 7Department of Neurosurgery, University of Nebraska, Omaha, NE, United States
  8. 8Department of Radiation Oncology, University of Kansas, Kansas City, KS, United States
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Abstract

Background: 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.

Case presentation: 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.

Conclusion: 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.

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Cite

BibTeXRIS
BibTeX
@article{khan2026natural,
  title = {{Natural history and literature review of primary intracranial CIC-rearranged sarcomas: case report}},
  author = {Arissa Khan and Maryam Khan and Abigail Bowser and Sahara Cathcart and Nicole Shonka and Sean Kelly and Daniel Surdell and Breann Bowar and Danielle Cunningham and Qateeb Khan},
  journal = {Frontiers in Oncology},
  year = {2026},
  month = oct,
  doi = {10.3389/fonc.2026.1958689},
  url = {https://doi.org/10.3389/fonc.2026.1958689},
  note = {CC BY 4.0},
}
RIS
TY  - JOUR
TI  - Natural history and literature review of primary intracranial CIC-rearranged sarcomas: case report
AU  - Arissa Khan
AU  - Maryam Khan
AU  - Abigail Bowser
AU  - Sahara Cathcart
AU  - Nicole Shonka
AU  - Sean Kelly
AU  - Daniel Surdell
AU  - Breann Bowar
AU  - Danielle Cunningham
AU  - Qateeb Khan
PY  - 2026
DA  - 2026/10/12
JO  - Frontiers in Oncology
DO  - 10.3389/fonc.2026.1958689
UR  - https://doi.org/10.3389/fonc.2026.1958689
AB  - Background: 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.  Case presentation: 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.  Conclusion: 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.
SN  - 2234-943X
ER  -