Pediatric Atypical Teratoid Rhabdoid Tumor

  • Etiology: Rhabdoid cells are hallmark but only comprise fraction of tumor
  • Imaging:
    — Heterogeneous (cysts, hemorrhage, calcification)
    — Off midline in 50%
    — Cerebellar (50%) more common than supratentorial
  • DDX: Medulloblastoma look alike in infants less than 2 years old (typical medulloblastoma age is 5 years old)
  • Complications: 20% with cerebrospinal fluid metastases at presentation
  • Treatment:
  • Clinical: Consider ATRT when large brain tumor found in child less than 3 years old

Radiology Cases of Atypical Teratoid Rhabdoid Tumor

Radiology Cases of Supratentorial Atypical Teratoid Rhabdoid Tumor

CT of atypical teratoid rhabdoid tumor
Axial (above), coronal (below left) and sagittal (below right) CT without contrast of the brain show an oval high density solid mass centered in the region of the pineal gland.
MRI of atypical teratoid rhabdoid tumor
Sagittal T1 MRI without contrast of the brain (above left) shows a large, isointense, heterogenous but primarily solid mass arising in the region of the pineal gland. On axial T2 MRI without contrast (above right) the mass is also isointense but has some cysts within it. Axial DWI MRI (below left) shows diffusion restriction in the mass while axial T1 MRI with contrast (below right) shows heterogenous enhancement of the mass.

Radiology Cases of Spinal Atypical Teratoid Rhabdoid Tumor

MRI of atypical teratoid rhabdoid tumor (ATRT) of the spine
Sagittal T1 without contrast (left), sagittal T2 (middle) and sagittal T1 with contrast (right) MRI of the lumbar spine shows an extramedullary intradural lesion that is inferior to the conus medullaris and fills the spinal canal from the level of the L1-L3 vertebral bodies. The lesion has heterogenous signal intensity on T2 and heterogenous enhancement with contrast.