A Congenital Giant Hepatic Hemangioendothelioma Treated with Interferon-$\alpha$ and Complete Tumor Resection

인터페론 투여 후 완전 절제를 시행한 거대 선천성 간내 혈관내피종

  • Cho, Min-A (Department of Pediatrics, Ajou University School of Medicine) ;
  • Yu, Jae-Eun (Department of Pediatrics, Ajou University School of Medicine) ;
  • Park, Moon-Sung (Department of Pediatrics, Ajou University School of Medicine) ;
  • Park, Jun-Eun (Department of Pediatrics, Ajou University School of Medicine) ;
  • Hong, Jeong (Department of Surgery, Ajou University School of Medicine) ;
  • Kim, Young-Bae (Department of Surgery, Ajou University School of Medicine)
  • 조민아 (아주대학교 의과대학 소아과학교실) ;
  • 유재은 (아주대학교 의과대학 소아과학교실) ;
  • 박문성 (아주대학교 의과대학 소아과학교실) ;
  • 박준은 (아주대학교 의과대학 소아과학교실) ;
  • 홍정 (아주대학교 의과대학 외과학교실) ;
  • 김영배 (아주대학교 의과대학 외과학교실)
  • Published : 2008.11.30

Abstract

Infantile hepatic hemangioendotheliomas (IHHEs) are benign vascular tumors, but can be associated with the life-threatening complications, such as congestive heart failure, disseminated intravascular coagulation, and massive bleeding. Various therapeutic options have been developed and the treatment response depends on the patient's clinical status and the nature of the lesion. In the case of a symptomatic IHHE, a non-invasive and precise diagnosis should be performed promptly before the therapeutic method is chosen. Additionally, it should be kept in mind that the residual lesions have malignant potential. We report a case of a congenital giant IHHE that was successfully reduced in size by interferon-$\alpha$ and completely removed by surgical tumor resection with a hepatic lobectomy.

신생아의 선천성 거대 간내 혈관내피종을 복부 단층 촬영과 $^{99m}Tc$-RBC 간스캔을 통해 진단하고, 대증요법과 함께 IFN$\alpha$를 11개월간 단독으로 피하 투여하여 크기 감소를 유도한 후 남은 병변의 완전한 수술적 제거와 조직학적 확진을 시행하였음을 보고하는 바이다.

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