Introduction:: Atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system is a highly aggressive neoplasm which prevalently affects children and is characterized by a severe prognosis. Case presentation:: The authors describe an extremely rare case of a primary spinal AT/RT that occurred in a young girl. The patient underwent a wide surgical resection of a lumbar mass, followed by aggressive chemotherapy, myeloablative treatment, and local radiotherapy. After 7 months from the end of the treatment, the patient experienced local recurrence and was treated with surgery and second-line chemotherapy with antiangiogenic purposes, consisting of oral vinorelbine, cyclophosphamide, and celecoxib. Treatment was well tolerated, and patient was still alive 36 months after diagnosis. Conclusion:: The peculiarity of this case report is the clinical-radiological response to a metronomic therapy in a case of early-relapsing spinal AT/RT despite previous maximal surgery, chemotherapy, and radiotherapy.
- Atypical teratoid/rhabdoid tumor
- Metronomic chemotherapy
- Spinal tumor
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Clinical Neurology