Chordoma presenting as a posterior mediastinal mass in a pediatric patient.

Chordomas are slow-growing malignant tumors that arise from fetal notochord cells. They are commonly found at the cranial or caudal ends of the spinal cord, but can be seen infrequently in other areas of the cord. Thoracic chordomas can present as either a posterior mediastinal mass or with cord compression symptoms. The tumors present most frequently in the 4th to 5th decade of life. It is extremely rare to see thoracic chordoma in a child. The treatment of choice for chordomas is surgery. The tumors should be totally excised when possible. If the tumor is large or invasive, debulking is indicated. Adjuvant therapy is controversial. Chordomas are very resistant to radiotherapy but there have been several reports of increased survival with high-dose radiotherapy (7,500 rad). Chemotherapy is unproven in the treatment of chordomas. Chordomas tend to recur locally quite frequently and occasionally with distant metastases. Survival ranges from 10% to 30% at 5 years.