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학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2011.1
- 수록면
- 313 - 318 (6page)
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This is a case report of a 68-year-old man with hepatocellular carcinoma (HCC) accompanied by hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu disease, and hepatic vascular malformation. HHT is an autosomal dominant disorder of the fibrovascular tissue that is characterized by recurrent epistaxis, mucocutaneous telangiectasias, and visceral arteriovenous malformations. HHT is caused by mutation of the genes involved in the signaling pathway of transforming growth factor-β, which plays an important role in the formation of vascular endothelia1.
Hepatic involvement has been reported as occurring in 30-73% of patients with HHT. However, symptomatic liver involvement is quite rare, and the representative clinical presentations of HHT in hepatic involvement are high-output heart failure, portal hypertension, nodular regenerative hyperplasia, and symptoms of biliary ischemia. Some cases of HCC in association with HHT have been reported, but are very rare. We present herein the characteristic radiologic and genetic findings of HHT that was diagnosed during the evaluation and treatment of HCC.
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참고문헌 (18)
참고문헌 신청
Begbie ME / 2003 / Hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome): a view from the 21st century / Postgrad Med J 79 : 18 ~ 24
Blewitt RW / 2003 / The pathology of acute hepatic disintegration in hereditary haemorrhagic telangiectasia / Histopathology 42 : 265 ~ 269
Bossler AD / 2006 / Novel mutations in ENG and ACVRL1 identified in a series of 200 individuals undergoing clinical genetic testing for hereditary hemorrhagic telangiectasia
Buscarini E / 1994 / Hepatic vascular malformations in hereditary hemorrhagic telangiectasia: imaging findings / Am J Roentgenol 163 : 1105 ~ 1110
Buscarini E / 2005 / Liver involvement in hereditary haemorrhagic telangiectasia or Rendu-Osler-Weber disease / Dig Liver Dis 37 : 635 ~ 645
Blewitt RW / 2003 / The pathology of acute hepatic disintegration in hereditary haemorrhagic telangiectasia / Histopathology 42 : 265 ~ 269
Bossler AD / 2006 / Novel mutations in ENG and ACVRL1 identified in a series of 200 individuals undergoing clinical genetic testing for hereditary hemorrhagic telangiectasia
Buscarini E / 1994 / Hepatic vascular malformations in hereditary hemorrhagic telangiectasia: imaging findings / Am J Roentgenol 163 : 1105 ~ 1110
Buscarini E / 2005 / Liver involvement in hereditary haemorrhagic telangiectasia or Rendu-Osler-Weber disease / Dig Liver Dis 37 : 635 ~ 645
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