A case of relapsing spinal atypical teratoid/rhabdoid tumor (AT/RT) responding to vinorelbine, cyclophosphamide, and celecoxib.

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.