Giant intracranial mesenchymal chondrosarcoma with uncal herniation

Abstract

Summary Mesenchymal chondrosarcomas are very rare central nervous system (CNS) tumors consisting of undifferentiated mesenchymal cells plus islets of cartilage. We report a case of giant intracranial mesenchymal chondrosarcomas presenting with acute neurologic deterioration. A 22-year-old woman presented with right facial and hand numbness and ocular torsion for about 2 weeks. Magnetic resonance imaging (MRI) disclosed a mass in the right middle cranial fossa with a mass effect. Two days before scheduled surgery, the patient suddenly lost consciousness and was found to have uncal herniation. At emergency surgery, the tumor was totally excised. Pathologically, there was a dimorphic pattern of undifferentiated cells and islets of cartilage, consistent with mesenchymal chondrosarcoma. Although the patient survived surgery, she remained in a vegetative state. The tumor recurred 3 years after surgery. Intracranial mesenchymal chondrosarcoma is rare and should be considered when evaluating a tumor with dural involvement, particularly in younger adults. Differential diagnoses include intracranial meningioma and schwannoma. An aggressive surgical resection is highly recommended because of the high risk of recurrence.