Atypical Presentation of Atypical Teratoid Rhabdoid Tumor in a Child

Atypical Teratoid Rhabdoid Tumor (ATRT) is a rare malignant intracranial neoplasm more commonly diagnosed in young children. The authors report the case of an 11-year-old boy with a long standing history of slowly progressive weight loss, fatigue, and weakness over 1.5 years whose magnetic resonance...

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Main Authors: Y. T. Udaka, K. Shayan, N. A. Chuang, J. R. Crawford
Format: Article
Language:English
Published: Wiley 2013-01-01
Series:Case Reports in Oncological Medicine
Online Access:http://dx.doi.org/10.1155/2013/815923
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author Y. T. Udaka
K. Shayan
N. A. Chuang
J. R. Crawford
author_facet Y. T. Udaka
K. Shayan
N. A. Chuang
J. R. Crawford
author_sort Y. T. Udaka
collection DOAJ
description Atypical Teratoid Rhabdoid Tumor (ATRT) is a rare malignant intracranial neoplasm more commonly diagnosed in young children. The authors report the case of an 11-year-old boy with a long standing history of slowly progressive weight loss, fatigue, and weakness over 1.5 years whose magnetic resonance imaging revealed a large heterogeneous enhancing dorsally exophytic lower brainstem mass. Examination revealed extreme cachexia, gaze-evoked nystagmus, dysphagia, dysarthria, bilateral dysmetria, and global weakness without ambulation. The protracted history and neuroimaging features were most suggestive of a low grade glioma. However, pathology revealed a hypercellular tumor with large hyperchromatic nucleoli and loss of INI-1 staining on immunohistochemistry consistent with a diagnosis of an ATRT. The child died shortly after surgery due to complications from his brainstem infiltrative disease. This case illustrates the diverse presentation of ATRT in childhood that can clinically and radiographically mimic that of low grade glioma.
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spelling doaj-art-a673aabfe2c6479bb722555fb6a70cb22025-02-03T01:03:31ZengWileyCase Reports in Oncological Medicine2090-67062090-67142013-01-01201310.1155/2013/815923815923Atypical Presentation of Atypical Teratoid Rhabdoid Tumor in a ChildY. T. Udaka0K. Shayan1N. A. Chuang2J. R. Crawford3The Department of Pediatrics, University of California, San Diego and Rady Children’s Hospital, 3020 Children’s Way, San Diego, CA 92123, USAThe Department of Pediatrics, University of California, San Diego and Rady Children’s Hospital, 3020 Children’s Way, San Diego, CA 92123, USAThe Department of Radiology, University of California, San Diego and Rady Children’s Hospital, 3020 Children’s Way, San Diego, CA 92123, USAThe Department of Pediatrics, University of California, San Diego and Rady Children’s Hospital, 3020 Children’s Way, San Diego, CA 92123, USAAtypical Teratoid Rhabdoid Tumor (ATRT) is a rare malignant intracranial neoplasm more commonly diagnosed in young children. The authors report the case of an 11-year-old boy with a long standing history of slowly progressive weight loss, fatigue, and weakness over 1.5 years whose magnetic resonance imaging revealed a large heterogeneous enhancing dorsally exophytic lower brainstem mass. Examination revealed extreme cachexia, gaze-evoked nystagmus, dysphagia, dysarthria, bilateral dysmetria, and global weakness without ambulation. The protracted history and neuroimaging features were most suggestive of a low grade glioma. However, pathology revealed a hypercellular tumor with large hyperchromatic nucleoli and loss of INI-1 staining on immunohistochemistry consistent with a diagnosis of an ATRT. The child died shortly after surgery due to complications from his brainstem infiltrative disease. This case illustrates the diverse presentation of ATRT in childhood that can clinically and radiographically mimic that of low grade glioma.http://dx.doi.org/10.1155/2013/815923
spellingShingle Y. T. Udaka
K. Shayan
N. A. Chuang
J. R. Crawford
Atypical Presentation of Atypical Teratoid Rhabdoid Tumor in a Child
Case Reports in Oncological Medicine
title Atypical Presentation of Atypical Teratoid Rhabdoid Tumor in a Child
title_full Atypical Presentation of Atypical Teratoid Rhabdoid Tumor in a Child
title_fullStr Atypical Presentation of Atypical Teratoid Rhabdoid Tumor in a Child
title_full_unstemmed Atypical Presentation of Atypical Teratoid Rhabdoid Tumor in a Child
title_short Atypical Presentation of Atypical Teratoid Rhabdoid Tumor in a Child
title_sort atypical presentation of atypical teratoid rhabdoid tumor in a child
url http://dx.doi.org/10.1155/2013/815923
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AT jrcrawford atypicalpresentationofatypicalteratoidrhabdoidtumorinachild