Atypical Teratoid/Rhabdoid Tumor of the Conus Medullaris

Pediatr Neurosurg. 2020;55(4):215-221. doi: 10.1159/000509165. Epub 2020 Sep 3.


Introduction: Atypical teratoid/rhabdoid tumor (AT/RT) is a rare tumor of the central nervous system, especially when involving the spinal column or spinal cord.

Case presentation: We present a case of a 5-year-old girl with progressive bilateral lower extremity pain found to have a discrete nodular lesion of the conus with mild heterogeneous enhancement. Surgical decompression and resection demonstrated a pathologic tumor consistent with AT/RT with loss of INI1 protein on immunohistochemistry.

Discussion and conclusion: AT/RT lesions of the conus medullaris are exceedingly rare and associated with extensive disease. We report a rare case of AT/RT with selective involvement of the conus medullaris, as well as describe the surgical, radiographic, and pathologic findings of this tumor.

Keywords: Atypical teratoid/rhabdoid tumor; Conus medullaris; Pediatric spine; Spinal tumor.

Publication types

  • Case Reports

MeSH terms

  • Central Nervous System Neoplasms*
  • Child, Preschool
  • Decompression, Surgical
  • Female
  • Humans
  • Rhabdoid Tumor* / diagnostic imaging
  • Rhabdoid Tumor* / surgery
  • Spinal Cord Neoplasms* / diagnostic imaging
  • Spinal Cord Neoplasms* / surgery