title: Primary atypical teratoid/rhabdoid tumor of the spine in an adult patient.
abstract: BACKGROUND
  Atypical teratoid/rhabdoid tumor (AT/RT) is an aggressive neoplasm of the central nervous system that generally arises intracranially in patients under 2 years of age. Primary spinal AT/RT in an adult is rare.
  
  
  METHODS
  A 23-year-old female presented with left lower extremity sciatica attributed to a magnetic resonance imaging (MRI)-documented intradural mass between L2 and L4. The lesion was biopsied (was unresectable) and treated with high-dose chemotherapy (methotrexate, vincristine, cyclophosphamide, etoposide, and cisplatin) with autologous hematopoietic stem cells rescue, followed by 2 months of radiation therapy (36 Gy to craniospinal axis, 20 Gy to lumbar region) with concurrent temozolomide; the latter was discontinued after 3 weeks due to myelosuppression. Tumor relapsed 1 year later at C7-T1 level. She was started on oral metronomic therapy, and bevacizumab was added 2 months later. Three months later, a cervical MRI showed progression of the tumor, along with new lesions in the thoracic/lumbar spine plus intracranial punctate nodular tumors. Following resection of the C7/T1 lesion, she was started on palliative alisertib; a month later, a cranial computed tomography showed progression of her disease with hydrocephalus. Treatment was discontinued, and she expired 12 months after initial diagnosis.
  
  
  CONCLUSIONS
  Primary spinal AT/RT in the adult patient is rare. The pathology is associated with early recurrence and a poor prognosis. Although potential benefits of metronomic chemotherapy and alisertib have been reported, the patient in this study did not favorably respond to these modalities.
authors.affiliation: Department of Neurosurgery, Medical College of Wisconsin, Milwaukee, WI 53226, USA.
authors.forename: Luyuan
authors.initials: L
authors.lastname: Li
authors.affiliation: Department of Neurosurgery, Medical College of Wisconsin, Milwaukee, WI 53226, USA.
authors.forename: Mohit
authors.initials: M
authors.lastname: Patel
authors.affiliation: Department of Neurosurgery, Medical College of Wisconsin, Milwaukee, WI 53226, USA.
authors.forename: Ha Son
authors.initials: HS
authors.lastname: Nguyen
authors.affiliation: Department of Neurosurgery, Medical College of Wisconsin, Milwaukee, WI 53226, USA.
authors.forename: Ninh
authors.initials: N
authors.lastname: Doan
authors.affiliation: Department of Neurosurgery, Medical College of Wisconsin, Milwaukee, WI 53226, USA.
authors.forename: Abhishiek
authors.initials: A
authors.lastname: Sharma
authors.affiliation: Department of Neurosurgery, Medical College of Wisconsin, Milwaukee, WI 53226, USA.
authors.forename: Dennis
authors.initials: D
authors.lastname: Maiman
keywords: Adult spine tumor; alisertib; atypical teratoid/rhabdoid tumor; metronomic therapy; primary spine tumor