Background: Intracranial chondroid tumors are consist of less than 0.16% of all primary brain neoplasms. They are classified as mesenchymal, classic and myxoid according to their cytoarchitecture. They usually arise at the skull base from the cartilaginous synchondroses and the pluripotent mesenchymal cells of the overlying dura mater.Case: We present a 22-year old female patient, who had history of seizures for 10 years and recent increase in the frequency of the seizures. Cranial magnetic resonance imaging (MRI) demonstrated a well-demarcated, parasagittal left frontal mass, which compressed to the lateral ventricle. It was hypointense on T1-weighted and hyperintense on T2-weighted images and making an edema on the surrounding tissue. Gross total resection of the firm mass was performed with clear margins. The tumor was composed of lobules of mature-appearing hyaline cartilage. The tumor cellularity was increased, each lacuna contained one or two atypical chondrocytes which had two or more nuclei per cell. Neoplastic chondrocytes had plump and hyperchromatic nuclei. Mitotic rate was low and no atypical mitotic figures were noted. Neoplastic chondrocytes showed nuclear staining for S–100 protein, but not for pancytokeratin. Histopathologic examination revealed a classic low grade chondrosarcoma.Conclusion: Intracranial chondrosarcomas are rare malign tumors. We report a patient with a grade I intracranial chondrosarcoma of the classical subtype without any neurologic problem after complete surgical excision. She did not received any adjuvant therapy and 28 months follow-up showed no recurrence. So it seems that, especially in the low grade chondrosarcoma, radical neurosurgical resection is the first line of treatment, and if necessary adjuvant therapy can be planned.
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