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Extrarenal rhabdoid tumor mimicking a sacral peripheral nerve sheath tumor

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Abstract

Extrarenal rhabdoid tumor is a rare, highly aggressive tumor of childhood with a poor prognosis. It represents <1% of pediatric soft tissue malignancies, typically involving infants . Frequently involved extrarenal sites include deep locations of the neck, abdomen, and paraspinal regions. The presence of “rhabdoid” cells is the characteristic histologic feature. Recent discovery of a specific genetic mutation enables a more accurate diagnosis. We present a case in an adolescent of extrarenal rhabdoid tumor arising within the sacral canal. This appears to be the first reported case of an extrarenal rhabdoid tumor arising within the sacral canal and mimicking a peripheral nerve sheath tumor. While rare, this tumor can be included in the radiologic differential diagnosis of peripheral nerve sheath tumors in children.

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Authors and Affiliations

  1. Department of Radiology and Radiological Sciences, Vanderbilt University Medical Center, 1161 Medical Center Dr, MCN CCC-1106, Nashville, TN, 37232–2675, USA

    Matthew D. Dobbs & J. Herman Kan

  2. Department of Pathology, Vanderbilt Children’s Hospital, 1161 21st Ave South CC3322 MCN, Nashville, TN, 37232–2561, USA

    Hernan Correa

  3. Department of Orthopedic Oncology, Vanderbilt University Medical Center, Suite 4200, South Tower, MCE 1215 21st Ave South, Nashville, TN, 37232, USA

    Herbert S. Schwartz

Authors
  1. Matthew D. Dobbs
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  2. Hernan Correa
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  3. Herbert S. Schwartz
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  4. J. Herman Kan
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Correspondence to Matthew D. Dobbs.

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Dobbs, M.D., Correa, H., Schwartz, H.S. et al. Extrarenal rhabdoid tumor mimicking a sacral peripheral nerve sheath tumor. Skeletal Radiol 40, 1363–1368 (2011). https://doi.org/10.1007/s00256-011-1160-0

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  • DOI: https://doi.org/10.1007/s00256-011-1160-0

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