CIC-rearranged undifferentiated round cell sarcoma metastatic to the brain treated with surgery and stereotactic radiosurgery: Case report.

CIC-rearranged undifferentiated round cell sarcoma metastatic to the brain treated with surgery and stereotactic radiosurgery: Case report.:

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CIC-rearranged undifferentiated round cell sarcoma metastatic to the brain treated with surgery and stereotactic radiosurgery: Case report.

World Neurosurg. 2020 Apr 03;:

Authors: Helal A, Graffeo CS, Perry A, Cheville JC, Link MJ, Pollock BE

Abstract

BACKGROUND: Ewing-like sarcoma with CIC-rearrangement are a unique class of undifferentiated round cell sarcomas characterized by the CIC-DUX4 gene fusion. Despite showing great histologic resemblance to Ewing sarcomas, they have proven to be a distinct pathological entity on the basis of immunohistochemistry, genetics, and response to treatment. We present a case of CIC-rearranged Ewing-like sarcoma with cerebral metastasis managed with operative resection and gamma knife radiosurgery.

METHODS: Case Report RESULTS: A 56 year old woman initially presented with an ulcerating lesion of the right fifth toe. Histological and immunohistochemical analysis revealed features consistent with CIC-rearrangement Ewing-like sarcoma, which was confirmed with genetic analysis. Despite aggressive local control and multi-drug chemotherapy regimen, patient developed multi-focal metastases involving the lungs, femur, and cerebrum. Cerebral lesions were managed with surgery and gamma knife radiosurgery with mixed results.

CONCLUSION: CIC-rearrangement 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 aggressive pathological process.

PMID: 32251827 [PubMed - as supplied by publisher]