Metastatic saccrococcygeal chordoma

• Sacrococcygeal chordomas are locally invasive notochord-related sarcomas.
• Tumors are considered radio-resistant and gross total resection remains the gold standard.
• Systemic therapeutic choices such as imatinib have shown some promise.
• Recent studies have discovered aberrant cellular pathways which may be targeted in the future.

We report a 46-year-old man who presented with a 2 week history of worsening headaches and acute onset left sided hemiplegia. He had undergone a surgical resection of a sacral chordoma 13 years prior, followed by adjuvant radiotherapy and chemotherapy. MRI revealed multiple enhancing lesions in the brain, and the two largest were resected. The histopathology was consistent with chordoma. Sacrococcygeal chordomas are locally invasive notochord-related sarcomas. They rarely metastasize to the brain, and only eight patients have been reported. While currently available adjuvant radiotherapy and systemic chemotherapeutic regimens can be implemented in the management of these rare patients, they have shown limited success. The newer strategies that are reported here have also been disappointing.