Intracranial extraskeletal mesenchymal chondrosarcoma: Case report and review of the literature

Intracranial extraskeletal mesenchymal chondrosarcoma is a malignant tumor reported in fewer than 40 cases in the published literature. This case report describes a 60-year-old black man who developed blindness as a presenting symptom of an intracranial tumor. Computed tomography (CT) and magnetic resonance imaging (MRI) studies revealed the presence of a large bifrontal mass with calcification and edema that appeared to arise from the anterior aspect of the falx. Following craniotomy and gross total resection, the tumor was diagnosed by histologic characteristics as a mesenchymal chondrosarcoma. The tumor demonstrated the two distinguishing mesenchymal chondrosarcoma components: poorly differentiated spindle cells with hyperchromatic nuclei (in some areas with an appearance suggestive of hemangiopericytoma) and well differentiated cartilage containing areas of calcification and bone formation. The patient was treated with adjuvant radiation therapy. Nevertheless, the tumor recurred, and 27 months after craniotomy the patient succumbed to the disease. Pertinent literature is reviewed concerning the diagnosis, treatment, and prognosis of this tumor. Although 4 decades have passed since this tumor was first identified as a distinctive entity, gross total resection remains the mainstay of treatment. Adjuvant therapies of radiation and chemotherapy show promise, but prognosis is one of recurrence, sometimes metastasis, and ultimately death.