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How specific is the MRI appearance of supratentorial atypical teratoid rhabdoid tumors?

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Abstract

Background

Supratentorial atypical teratoid rhabdoid tumor (ATRT) in many cases has a distinctive appearance on post-gadolinium MRI.

Objective

We sought to determine whether this is a unique appearance allowing ATRT to be distinguished accurately from other types of pediatric supratentorial tumors.

Materials and methods

Retrospective review of all available preoperative MRI of pediatric supratentorial tumors at two tertiary children’s hospitals, and systematic literature review of case series and reports describing the MRI imaging appearances of supratentorial ATRT.

Results

We had 61 supratentorial tumors, including 32 gliomas, 6 ATRT, 8 ependymomas, 6 gangliogliomas, 2 pilomyxoid astrocytomas, 3 primitive neuro-ectodermal tumors, 2 choroid plexus papillomas, and 2 meningiomas. ATRT presented in significantly younger patients than astrocytomas (mean age 2.6 years vs. 9.9 years, P < 0.05). The visual pattern of a thick, wavy (irregular) heterogeneously enhancing wall around a cystic center was seen in 5/6 (83%) ATRTs and only 3/55 (5.4%) other tumors (P < 0.0001), for specificity of 95%, sensitivity of 83%, positive predictive value of 63% and a negative predictive value of 95%.

Conclusion

A supratentorial tumor with a thick, wavy (irregular) heterogeneously enhancing wall surrounding a central cystic region is suggestive of ATRT in the appropriate clinical setting, especially in a child of preschool age.

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Authors and Affiliations

  1. Department of Radiology and Diagnostic Imaging, University of Alberta Hospital, 8440-112 Street NW, Edmonton, Canada, T6G 2B7

    Kong Jung Au Yong, Jacob L. Jaremko & Ravi Bhargava

  2. Department of Radiology and Medical Imaging, Ghent University Hospital, Gent, Belgium

    Lennart Jans

  3. Department of Radiology and Pediatrics, University of Melbourne and Murdoch Children’s Research Institute, Melbourne, Australia

    Lee T. Coleman

  4. Medical Imaging, Royal Children’s Hospital, Parkville, Australia

    Lee T. Coleman

  5. Department of Neurosurgery, University of Alberta Hospital, Edmonton, Canada

    Vivek Mehta

  6. Diagnostic Imaging, Monash Children’s and Monash University, Monash Medical Centre, Clayton, Australia

    Michael R. Ditchfield

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  1. Kong Jung Au Yong
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Correspondence to Kong Jung Au Yong.

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Au Yong, K.J., Jaremko, J.L., Jans, L. et al. How specific is the MRI appearance of supratentorial atypical teratoid rhabdoid tumors?. Pediatr Radiol 43, 347–354 (2013). https://doi.org/10.1007/s00247-012-2530-z

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  • DOI: https://doi.org/10.1007/s00247-012-2530-z

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