Journal of the Korean Society of Radiology (대한영상의학회지)

The Korean Society of Radiology (대한영상의학회)
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A Rare Case of Cardiac Neurofibroma in a Patient with Neurofibromatosis Type 1: Radiologic Findings

신경섬유종증 1형 환자에서 드물게 발생하는 심장 신경섬유종: 영상의학적 소견

  • Sanghyun Seo (Department of Radiology, Wonkwang University Hospital, Wonkwang University School of Medicine) ;
  • Ji Young Rho (Department of Radiology, Wonkwang University Hospital, Wonkwang University School of Medicine)
  • 서상현 (원광대학교 의과대학 원광대학교병원 영상의학과) ;
  • 노지영 (원광대학교 의과대학 원광대학교병원 영상의학과)
  • Received : 2020.09.24
  • Accepted : 2021.01.06
  • Published : 2021.09.01
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Abstract

Neurofibromatosis type 1 (NF1) is a relatively common inherited disorder characterized by the formation of neurofibromas, pigmentary abnormalities of the skin, Lisch nodules of the iris, and skeletal abnormalities. Multiple cutaneous neurofibromas are benign nerve sheath tumors and the main manifestation of NF1. Cardiac neurofibroma associated with NF1 is very rare, and few cases have been reported in the literature. Herein, we present the CT and MRI findings of a surgically confirmed left ventricular neurofibroma in a 32-year-old female with NF1.

신경섬유종증 1형은 비교적 흔한 유전 질환이며 신경섬유종 형성, 피부의 색소 이상, 홍채의 리쉬결절 및 골격 이상들을 특징으로 한다. 다발성 피부 신경섬유종은 양성 신경초 종양이며 신경섬유종증 1형의 특징적인 병변이다. 신경섬유종증 1형과 관련된 심장 신경섬유종은 매우 드물며 문헌에 몇 가지 사례가 보고되었다. 이에 저자들은 신경섬유종증 1형을 가진 32세 여성에서 수술로 확진된 좌심실 신경섬유종의 컴퓨터단층촬영 및 자기공명영상 소견을 보고한다.

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References

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