Abstract
Atypical teratoid/rhabdoid tumors (AT/RTs) are rare and aggressive tumors that most often occur in early childhood. These tumors usually are located in the cerebellum or cerebral hemispheres, but can occur in unusual locations. Prior to genetic analysis demonstrating a deletion of the tumor suppressor gene INI-1, these tumors were often misdiagnosed as primitive neuroectodermal tumors (PNETs). The patient presented here is a 7 year-old boy with an extradural clival-C2 AT/RT who was treated with a gross total resection (GTR) using an aggressive transoral resection. He was then stabilized with a halo and an instrumented fusion from occiput to C5, and received adjuvant therapy consisting of chemotherapy, radiation therapy, and a bone marrow transplant with a stem cell rescue. The patient survived an additional 42 months after surgery before dying of disseminated disease. Given the very small number of extradural spinal AT/RTs, the best treatment and prognosis is unknown. However, an aggressive approach of GTR followed by intensive chemotherapy and radiation therapy regimens appears to provide the best option at this point in time.
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Kramarz, M.J., Jackson, E.M., Resnick, A.C., Storm, P.B. (2012). Early Childhood Clival-C2 Atypical Teratoid/Rhabdoid Tumor: Gross Total Resection Followed by Aggressive Chemotherapy and Radiation. In: Hayat, M. (eds) Pediatric Cancer, Volume 3. Pediatric Cancer, vol 3. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-4528-5_7
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