The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
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A Case Report of Recurrent Malignant Paraganglioma with Lung and Spine Metastasis that Occurred Sixteen Years after Primary Tumor Excision

초기 절제술 16년 후 다발성 원격 전이된 악성 부신경절종 1예

  • Moon, Eul Sun (Department of Internal Medicine, Korea University College of Medicine) ;
  • Min, Hye Sook (Department of Internal Medicine, Korea University College of Medicine) ;
  • Jung, Chang Ho (Department of Internal Medicine, Korea University College of Medicine) ;
  • Choi, Jong Hyun (Department of Internal Medicine, Korea University College of Medicine) ;
  • Kim, Jun Suk (Department of Internal Medicine, Korea University College of Medicine) ;
  • Yoo, Hye Jin (Department of Internal Medicine, Korea University College of Medicine) ;
  • Choi, Sang Yong (Department of Surgery, Korea University College of Medicine)
  • 문을선 (고려대학교 의과대학 내과학교실) ;
  • 민혜숙 (고려대학교 의과대학 내과학교실) ;
  • 정창호 (고려대학교 의과대학 내과학교실) ;
  • 최종현 (고려대학교 의과대학 내과학교실) ;
  • 김준석 (고려대학교 의과대학 내과학교실) ;
  • 류혜진 (고려대학교 의과대학 내과학교실) ;
  • 최상룡 (고려대학교 의과대학 외과학교실)
  • Received : 2013.07.24
  • Accepted : 2013.09.12
  • Published : 2014.10.01
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Abstract

Paragangliomas are rare tumors that arise from the extra-adrenal chromaffin cells, and malignancy is defined by the presence of metastases to sites that normally lack chromaffin tissue. The main therapeutic targets are tumor reduction and control of excessive catecholamine secretion. Currently, the adjuvant therapy to surgery is radiotherapy treatment using high-dose MIBG; chemotherapy is currently evolving. We report herein a case of malignant paraganglioma with lung and spine metastasis that occurred 16 years after primary tumor excision and was treated with high dose MIBG radiotherapy and chemotherapy.

부신경절종은 부신 이외의 크롬친화성 세포에서 기원하는 드문 종양으로 원격전이가 있는 경우 악성으로 진단할 수 있다. 치료의 주요 목적은 종양의 감축과 카테콜아민 과다분비로 인한 증상감소에 있으며, 최근에는 수술외 보조요법으로 고용량 MIBG 치료와 함께 항암치료가 제시되고 있다. 국내에서는 간전이 되었으나 수술적 치료는 하지 못하고 보존적 치료하였던 1예, 후종격동 부신경절종으로 수술 치료하였던 1예, 그 외 마비성 장폐쇄를 동반한 변비와 고혈당 악화로 발현된 부신경절종 1예 등이 보고된 바 있으나 수술후 항암, 방사성 동위원소 치료까지 하였던 보고는 없었다. 본 증례에서는 부신경절종 절제술을 받은 지 16년 뒤 폐와 척추 전이로 재발된 악성 부신경절종 환자에게 고용량 MIBG 및 전신 항암 치료를 시행하면서 치료 반응을 추적관찰하였던 증례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

Keywords

References

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