Treatment for primary spinal atypical teratoid/rhabdoid tumor

Excerpt

Atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system (CNS) is an uncommon condition that accounts for only 1.3% of primary CNS tumors. It is a highly aggressive neoplasm that commonly occurs in infants and children younger than 3 years of age [1‐5]. AT/RT of the CNS is often located in the posterior fossa [6, 7]; primary spinal AT/RT is extremely rare [8‐11]. Medulloblastoma (MB) and a prototype primitive neuroectodermal tumor (PNET) are the most common malignant CNS tumors in children [2, 5], and it can be challenging to differentiate spinal AT/RT from spinal PNET [12]. However, differentiation is necessary because AT/RT has a poor prognosis compared with that of PNET, with median survival for AT/RT of approximately 6 months [13‐15]. Thus, AT/RTs may be among the most malignant CNS tumors. Several reports of AT/RT in the CNS are available, but only a few cases of primary spinal AT/RT have been described and there is no standard treatment for spinal AT/RT. Here, we report a surgical case with adjuvant therapy for primary spinal AT/RT in a child, and we review the literature on the topic. Consent for publication was obtained from the parents of the patient. …