Journal of the Korean Society of Radiology (대한영상의학회지)

The Korean Society of Radiology (대한영상의학회)
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Sporadic Intradural Extramedullary Hemangioblastoma Not Associated with von Hippel-Lindau Syndrome: A Case Report and Literature Review

폰 히펠린다우 증후군과 관련 없는 특발성 경막 내 수외 혈관모세포종: 증례 보고와 문헌 고찰

  • Mi-Kyung Um (Department of Radiology, Seoul National University Bundang Hospital) ;
  • Eugene Lee (Department of Radiology, Seoul National University Bundang Hospital) ;
  • Joon Woo Lee (Department of Radiology, Seoul National University Bundang Hospital) ;
  • Kyu Sang Lee (Department of Pathology, Seoul National University Bundang Hospital) ;
  • Yusuhn Kang (Department of Radiology, Seoul National University Bundang Hospital) ;
  • Joong Mo Ahn (Department of Radiology, Seoul National University Bundang Hospital) ;
  • Heung Sik Kang (Department of Radiology, Seoul National University Bundang Hospital)
  • 엄미경 (분당서울대학교병원 영상의학과) ;
  • 이영준 (분당서울대학교병원 영상의학과) ;
  • 이준우 (분당서울대학교병원 영상의학과) ;
  • 이규상 (분당서울대학교병원 병리과) ;
  • 강유선 (분당서울대학교병원 영상의학과) ;
  • 안중모 (분당서울대학교병원 영상의학과) ;
  • 강흥식 (분당서울대학교병원 영상의학과)
  • Received : 2020.03.11
  • Accepted : 2020.08.17
  • Published : 2021.05.01
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Abstract

Hemangioblastomas are low-grade, highly vascular tumors that are usually associated with von Hippel-Lindau syndrome. Hemangioblastomas most commonly occur in the cerebellum, and intradural extramedullary hemangioblastoma of the cauda equina is very rare, especially in patients without von Hippel-Lindau syndrome. Herein, we report a case of intradural extramedullary hemangioblastoma of the cauda equina that was not associated with von Hippel-Lindau syndrome, with a focus on its imaging characteristics and differential diagnoses. We compared the clinical presentation and imaging features of our case with those of previously reported cases in the review of the literature.

혈관모세포종은 저등급, 고혈관성 종양으로 주로 소뇌에 발생한다. 드물게 척추에 발생하는 경우 대부분 수내에 발생하며 본히펠린다우 증후군(von Hippel-Lindau syndrome)과 연관이 있는 경우가 많다. 하지만 경막 내 수외 종양으로 척수 말총에 본히펠린다우 증후군이 없이 발생하는 혈관모세포종은 극히 드물어 현재까지 24예만이 보고되어 있다. 우리는 본히펠린다우 증후군이 아닌 환자에서 척수 말총에 생긴 경막 내 수외 혈관모세포종의 특징적인 영상 소견과 그 감별진단에 초점을 맞춰 보고하고자 한다. 또한 문헌 고찰을 통해 이전에 보고되었던 증례들과 임상 양상 및 영상 소견을 비교하여 분석하였다.

Keywords

References

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