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Imaging findings in primary intracranial atypical teratoid/rhabdoid tumors

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Abstract

Background: Intracranial atypical teratoid/rhabdoid tumors (AT/RT) are rare and extremely aggressive neoplasms seen primarily in childhood. Imaging features are often considered non-specific. However, correct diagnosis of AT/RT is important because these tumors have a markedly different clinical prognosis and require more aggressive therapy.Objective: To determine the imaging features of AT/RT.Materials and methods: We retrospectively analyzed imaging findings in 11 patients with primary intracranial AT/RT presenting over a period of 5 years. CT (n=11), MR (n=7), clinical (n=11) and pathological (n=11) features were evaluated. FISH analysis showing monosomy of chromosome 22 (absence of bcr 22q11 locus) was available for three patients. Immunohistochemical staining for INI-1 (BAF47) was performed on all tumors. Results: There were 11 patients, 6 boys and 5 girls. The age of presentation varied from 1 month to 15 years (average age 3 years 8 months). Six tumors were located in the posterior fossa and five in the supratentorial compartment. The tumors showed a hyperdense solid component (64%) that showed moderate to marked enhancement with contrast medium. On MR imaging, the predominant signal pattern was isointensity on T1-weighted images (57%) and T2 shortening with heterogeneity on T2-weighted images (86%). All tumors were large in size (average 4.2×3.7 cm), and there was a tendency for calcification (36%), hemorrhage (46%), necrosis (46%) and perifocal edema (100%). There was also a high tendency for subarachnoid dissemination, with five patients (46%) demonstrating brain and/or spinal metastasis. At follow-up (n=7), six patients showed local recurrence. At the time of recurrence, all these patients showed extensive leptomeningeal spread of the disease in both intracranial and intraspinal compartments. Conclusion: There are no specific imaging features for intracranial AT/RT. But a high tendency toward large size, a hyperdense solid component on CT scan with calcification, hemorrhage, necrosis and subarachnoid spread suggest that this tumor should be considered in the differential diagnosis of large pediatric intracranial tumors.

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Authors and Affiliations

  1. Department of Diagnostic Imaging, The Hospital for Sick Children, 555 University Ave., Toronto, ON, M5G 1X8, Canada

    Hemant Parmar & Manohar Shroff

  2. Department of Neuropathology, The Hospital for Sick Children, Toronto, ON, Canada

    Cynthia Hawkins

  3. Department of Neuro-Oncology, The Hospital for Sick Children, Toronto, ON, Canada

    Eric Bouffet

  4. Department of Neurosurgery, The Hospital for Sick Children, Toronto, ON, Canada

    James Rutka

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  1. Hemant Parmar
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  2. Cynthia Hawkins
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  5. Manohar Shroff
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Correspondence to Manohar Shroff.

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Parmar, H., Hawkins, C., Bouffet, E. et al. Imaging findings in primary intracranial atypical teratoid/rhabdoid tumors. Pediatr Radiol 36, 126–132 (2006). https://doi.org/10.1007/s00247-005-0037-6

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  • DOI: https://doi.org/10.1007/s00247-005-0037-6

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