The authors report the case of a chordoma with malignant cytologic features, presenting as a mass lesion in the clival and infratemporal region at the level of the craniocervical junction in an 8-year-old female. Following gross resection of the mass, the patient subsequently developed distant subcutaneous and peritoneal metastases from the lesion. The rare histologic features, the surgical approach to the lesion, and the follow-up management of this unique case are discussed.
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