Durable complete response to chemotherapy in an infant with a clival chordoma.

Chordomas are rare bone tumors of notochord remnants that may occur anywhere within the axial skeleton. The standard of care is complete surgical removal. Proton beam irradiation is commonly used when the tumor is inaccessible or has recurred. Chemotherapy has been used in the treatment of patients at relapse but it has been generally proven ineffective. We report a 7-month-old infant with a clival chordoma who responded to combination chemotherapy consisting of cycles of vincristine/cyclophosphamide/doxorubicin alternating with etoposide/ifosfamide. She has been off chemotherapy for 2 years and is well at age 5.