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CardioVascular and Interventional Radiology

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01-08-2018 | Case Report

Radioembolization for Hepatocellular Carcinoma Arising in the Setting of a Congenital Extrahepatic Portosystemic Shunt (Abernethy Malformation)

Authors: Thomas Kroencke, Marcus Murnauer, Frank Alexander Jordan, Vera Blodow, Hannes Ruhnke, Tina Schaller, Bruno Märkl

Published in: CardioVascular and Interventional Radiology | Issue 8/2018

Abstract

In a 50-year-old male with an incidentally discovered hepatocellular carcinoma, a congenital extrahepatic portosystemic shunt with the absence of the portal vein (Abernethy syndrome) was diagnosed by imaging. This study aims to discuss the variant anatomy relevant to this splanchnic vascular malformation, review its association with the development of liver neoplasms, and report the safety and efficacy of TARE followed by resection for a HCC arising in this rare condition.

Abernethy J, Banks J. Account of two instances of uncommon formation, in the viscera of the human body. Philos Trans R Soc Lond. 1793;83:59–66.CrossRef

Murray CP, Yoo S-J, Babyn PS. Congenital extrahepatic portosystemic shunts. Pediatr Radiol. 2003;33:614–20.CrossRefPubMed

De Gaetano AM, Gui B, Macis G, et al. Congenital absence of the portal vein associated with focal nodular hyperplasia in the liver in an adult woman: imaging and review of the literature. Abdom Imaging. 2004;29:455–9.CrossRefPubMed

Kobayashi N, Niwa T, Kirikoshi H, et al. Clinical classification of congenital extrahepatic portosystemic shunts. Hepatol Res. 2010;40:585–93.CrossRefPubMed

Benedict M, Rodriguez-Davalos M, Emre S, et al. Congenital extrahepatic portosystemic shunt (Abernethy malformation type Ib) with associated hepatocellular carcinoma: case report and literature review. Pediatr Dev Pathol. 2017;20:354–62.CrossRefPubMed

Happaerts S, Foucault A, Billiard JS, et al. Combined hepatocellular-cholangiocarcinoma in a patient with Abernethy malformation and tetralogy of Fallot: a case report. Hepatology. 2016;64(5):1800–2.CrossRefPubMed

Tacyildiz N, Ozdemir EG, Gordu Z, et al. Hepatocellular carcinoma with congenital absence of the portal vein in a child. J Clin Case Rep. 2015;504:2.

Sharma R, Suddle A, Quaglia A, et al. Congenital extrahepatic portosystemic shunt complicated by the development of hepatocellular carcinoma. Hepatobiliary Pancreat Dis Int. 2015;14:552–7.CrossRefPubMed

Gordon-Burroughs S, Balogh J, Weiner MA, et al. Liver transplantation in an adult with adenomatosis and congenital absence of the portal vein: a case report. Transpl Proc. 2014;46:2418–21.CrossRef

10.

Scheuermann U, Foltys D, Otto G. Focal nodular hyperplasia proceeds hepatocellular carcinoma in an adult with congenital absence of the portal vein. Transpl Int. 2012;25:e67–8.CrossRefPubMed

11.

Lisovsky M, Konstas AA, Misdraji J. Congenital extrahepatic portosystemic shunts (Abernethy malformation): a histopathologic evaluation. Am J Surg Pathol. 2011;35:1381–90.CrossRefPubMed

12.

Morotti RA, Killackey M, Shneider BL, et al. Hepatocellular carcinoma and congenital absence of the portal vein in a child receiving growth hormone therapy for turner syndrome. Semin Liver Dis. 2007;27:427–31.CrossRefPubMed

13.

Pichon N, Maisonnette F, Pichon-Lefièvre F, et al. Hepatocarcinoma with congenital agenesis of the portal vein. Jpn J Clin Oncol. 2003;33:314–6.CrossRefPubMed

14.

Lundstedt C, Lindell G, Tranberg KG, et al. Congenital absence of the intrahepatic portion of the portal vein in an adult male resected for hepatocellular carcinoma. Eur Radiol. 2001;11:2228–31.CrossRefPubMed

15.

Taïeb J, Castera L, Boige V, et al. Hepatocellular carcinoma complicating agenesis of the portal vein. Gastroentérol Clin Biol. 1998;22:246.PubMed

16.

Joyce AD, Howard ER. Rare congenital anomaly of the portal vein. Br J Surg. 1988;75:1038–9.CrossRefPubMed

17.

Hu GH, Shen LG, Yang J, et al. Insight into congenital absence of the portal vein: is it rare? World J Gastroenterol. 2008;14(39):5969–79.CrossRefPubMed

18.

Iñarrairaegui M, Pardo F, Bilbao JI, et al. Response to radioembolization with yttrium-90 resin microspheres may allow surgical treatment with curative intent and prolonged survival in previously unresectable hepatocellular carcinoma. Eur J Surg Oncol. 2012;38:594–601.CrossRefPubMed

Title: Radioembolization for Hepatocellular Carcinoma Arising in the Setting of a Congenital Extrahepatic Portosystemic Shunt (Abernethy Malformation)
Authors: Thomas Kroencke
Marcus Murnauer
Frank Alexander Jordan
Vera Blodow
Hannes Ruhnke
Tina Schaller
Bruno Märkl
Publication date: 01-08-2018
Publisher: Springer US
Published in: CardioVascular and Interventional Radiology / Issue 8/2018
Print ISSN: 0174-1551
Electronic ISSN: 1432-086X
DOI: https://doi.org/10.1007/s00270-018-1965-5

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Radioembolization for Hepatocellular Carcinoma Arising in the Setting of a Congenital Extrahepatic Portosystemic Shunt (Abernethy Malformation)

Abstract

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Abstract

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