Background: Intracranial extraskeletal mesenchymal chondrosarcoma is a rare, malignant variant of chondrosarcoma that is characterized by undifferentiated mesenchymal cells interspersed with pockets of mature hyaline cartilage.
Case description: In this study, we report a 23-year-old female patient who underwent multiple craniotomies for tumor resection, as well as adjuvant radiotherapy and chemotherapy. We review the literature for reported cases and discuss the histopathologic features, radiologic findings, therapeutic approaches, and outcomes associated with this rare tumor.
Conclusions: Intracranial extra-skeletal mesenchymal chondrosarcomas are very aggressive tumors, and their management should emphasize attempting gross total resection followed by adjuvant treatment modalities, including radiation therapy and/or chemotherapy.
Keywords: Gross total resection; Intracranial; Mesenchymal chondrosarcoma; Outcome; Radiation.
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