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Investigation of the location of atypical teratoid/rhabdoid tumor

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Abstract

Introduction

The location of a brain tumor is a fundamental characteristic, because various brain tumors develop in relatively specific locations. An atypical teratoid/rhabdoid tumor (AT/RT) is a highly age-specific tumor that occurs in infants and young children. However, AT/RTs develop in a variety of locations in the brain. This study aimed at uncovering the tumor location pattern of AT/RTs to enhance diagnoses.

Material and methods

Neuroimages from 27 patients with a pathologically proven AT/RT were reviewed, and the specific tumor locations were described and categorized. The association of imaging characteristics and tumor location was analyzed.

Results

The posterior fossa was the most frequent locations accounting for 19 patients (70 %), followed by the diencephalon (four patients; 15 %), cerebrum (three patients; 11 %), and midbrain (one patient; 4 %). In the posterior fossa, the superior medullary velum (SMV) and cerebellopontine angle (CPA) areas were the most common sites (eight patients each) and three patients had a tumor in the inferior medullary velum (IMV) region. AT/RTs in the SMV area had a significantly higher chance of no/minimal enhancement compared with tumors in other locations (P = 0.001) and a lower likelihood of leptomeningeal tumor seeding at presentation (P = 0.053).

Conclusion

The location spectrum of AT/RT follows a specific pattern, and some of the locations are linked with intriguing clinical characteristics. This information may not only help make correct preoperative diagnosis but also occasionally aid in postoperative pathological diagnosis.

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Acknowledgments

This study was supported by a grant from the National Research Foundation of Korea (No. 2014R1A1A1005765) to Phi JH.

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Authors and Affiliations

  1. Division of Pediatric Neurosurgery, Seoul National University Children’s Hospital, Seoul, Republic of Korea

    Yun-Sik Dho, Seung-Ki Kim, Kyu-Chang Wang, Ji Yeoun Lee & Ji Hoon Phi

  2. Department of Radiology, Seoul National University Children’s Hospital, Seoul, Republic of Korea

    Jung-Eun Cheon

  3. Department of Pathology, Seoul National University Children’s Hospital, Seoul, Republic of Korea

    Sung-Hye Park

  4. Division of Pediatric Neurosurgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, 110-744, Seoul, Republic of Korea

    Ji Hoon Phi

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  1. Yun-Sik Dho
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  2. Seung-Ki Kim
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  4. Sung-Hye Park
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  5. Kyu-Chang Wang
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  6. Ji Yeoun Lee
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Correspondence to Ji Hoon Phi.

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Dho, YS., Kim, SK., Cheon, JE. et al. Investigation of the location of atypical teratoid/rhabdoid tumor. Childs Nerv Syst 31, 1305–1311 (2015). https://doi.org/10.1007/s00381-015-2739-x

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  • DOI: https://doi.org/10.1007/s00381-015-2739-x

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