Successful multimodal therapies for a primary atypical teratoid/rhabdoid tumor in the cervical spine.

Atypical teratoid/rhabdoid tumor (AT/RT) occurring in the central nervous system is a high-grade malignant tumor, and its prognosis is poor for patients younger than 3 years of age. In this article, we present a case of infant AT/RT in the cervical spine and its successful treatment by intensive chemotherapy. The patient, a 1.75-year-old girl, developed an acute, progressive tetraparesis. MRI revealed a large, intradural mass in the cervical spine.Total surgical resection was performed, and the specimen was diagnosed as AT/RT. Continuously, she received intensive chemotherapy using thiotepa with autologous bone marrow transplantation. At the age of nearly 3 years, she received radiation therapy to the local tumor bed and craniospinal axis. She is now 4 years old and has been maintained in complete remission with a good clinical course and no neurological deficit. The success of this treatment for the patient was that we could prevent tumor recurrence until she was able to receive radiotherapy. (c) 2008 S. Karger AG, Basel.