Congenital absence of the portal vein complicated by hepatocellular carcinoma in the liver of an adult woman: review of imaging, literature and management

Abstract

We present a case of absence of the portal vein and Laennec’s cirrhosis in a 51-year-old female who was diagnosed with hepatocellular carcinoma (HCC). Only 101 cases of this malformation of the splanchnic vasculature have been reported of which 4 were reported to have HCC. Patient had disease progression while waiting for a liver transplant. Patient was treated with 3 separate conventional transarterial chemoembolization procedures at an outside hospital. At our institution, radioembolization of the right hepatic lobe was performed. She succumbed to liver insufficiency 8 years after being diagnosed with HCC. The features of this patient’s clinical course are reviewed.

Keywords

Hepatocellular cancer / radioembolization / abernathy malformation

References

[1] Mistinova J,Varga I.Congenital absence of the portal vein - case report and a review of literature..Clin Anat2010;23:750-8

[2] Marks C.Developmental basis of the portal venous system..Am J Surg1969;117:671-81

[3] Abernethy J.Account of two instances of uncommon formation in the viscera of the human body..Phil Trans R Soc Lond B Biol Sci1793;83:295-9

[4] Hao Y,Zhao X.Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease: a case report and literature review..Oncol Lett2015;9:695-700 PMCID:PMC4301562

[5] Morgan G.Congenital absence of the portal vein: two cases and a proposed classification system for portosystemic vascular anomalies..J Pediatr Surg1994;29:1239-41

[6] Cholongitas E,Vangeli M,Patch D.Systematic review: the model for end-stage liver disease--should it replace Child-Pugh's classification for assessing prognosis in cirrhosis?.Aliment Pharmacol Ther2005;22:1079-89

[7] Lencioni R.Chemoembolization for hepatocellular carcinoma..Semin Oncol2012;39:503-9

[8] Starzl TE,Halgrimson CG,Porter KA,Putnam CW.The origin, hormonal nature, and action of hepatotrophic substances in portal venous blood..Surg Gynecol Obstet1973;137:179-99 PMCID:PMC2747591

[9] Kamiya S,Yamamoto T,Kai M,Tsuda M,Nakasaki H.Analysis of intestinal flora of a patient with congenital absence of the portal vein..FEMS Immunol Med Microbiol1993;7:73-80

[10] Lundstedt C,Tranberg KG.Congenital absence of the intrahepatic portion of the portal vein in an adult male resected for hepatocellular carcinoma..Eur Radiol2001;11:2228-31

[11] Morotti RA,Shneider BL,Emre S.Hepatocellular carcinoma and congenital absence of the portal vein in a child receiving growth hormone therapy for turner syndrome..Semin Liver Dis2007;27:427-31

[12] Pichon N,Pichon-Lefievre F,Pillegand B.Hepatocarcinoma with congenital agenesis of the portal vein..Jpn J Clin Oncol2003;33:314-6

[13] Hu GH,Yang J,Zhu YF.Insight into congenital absence of the portal vein: is it rare?.World J Gastroenterol2008;14:5969-79 PMCID:PMC2760189

[14] Reynolds AR,Fetzer DT,Borhani AA,Tublin ME.Infiltrative hepatocellular carcinoma: what radiologists need to know..Radiographics2015;35:371-86

[15] Thian YL,Chow PK,Chung AY,Xie W.Atypical enhancement pattern of hepatocellular carcinoma with portal vein thrombosis on multiphasic CT..Ann Acad Med Singapore2011;40:454-9

[16] Lam A,Sirlin CC,Goodwin SC,Lall C.Value of the portal venous phase in evaluation of treated hepatocellular carcinoma following transcatheter arterial chemoembolisation..Clin Radiol2017;72:994.e9-16

[17] Furlan A,Vanzulli A,Ronzoni A,Bazzocchi M,Brancatelli G.Hepatocellular carcinoma in cirrhotic patients at multidetector CT: hepatic venous phase versus delayed phase for the detection of tumour washout..Br J Radiol2011;84:403-12 PMCID:PMC3473662

[18] Monzawa S,Nakajima H,Omata K.Dynamic CT for detecting small hepatocellular carcinoma: usefulness of delayed phase imaging..AJR Am J Roentgenol2007;188:147-53

[19] López-Larramona G,González-Castillo S.Hepatic osteodystrophy: an important matter for consideration in chronic liver disease..World J Hepatol2011;3:300-7 PMCID:PMC3246548

[20] Lange PA.The hepatopulmonary syndrome: effect of liver transplantation..Clin Chest Med1996;17:115-23

[21] Lau WY,Chen PJ,Chow P,Leung T,Poon RT.Treatment for hepatocellular carcinoma with portal vein tumor thrombosis: the emerging role for radioembolization using yttrium-90..Oncology2013;84:311-8

[22] Joskin J,Auperin A,Guiu B,Boige V,Leboulleux S,Baudin E.Predisposing factors of liver necrosis after transcatheter arterial chemoembolization in liver metastases from neuroendocrine tumor..Cardiovasc Intervent Radiol2015;38:372-80

[23] Gorodetski B,Schernthaner R,Lin M,Lenis D,Nonyane BA,Tamrazi A,Schlachter T,Geschwind JF.Advanced-stage hepatocellular carcinoma with portal vein thrombosis: conventional versus drug-eluting beads transcatheter arterial chemoembolization..Eur Radiol2017;27:526-35 PMCID:PMC5470590

[24] Zhao Y,Chapiro J,Sahu S,Smolka S,Schernthaner R,Lee H,Lin M.Transarterial chemoembolization for the treatment of advanced-stage hepatocellular carcinoma..J Gastrointest Surg2016;20:2002-9 PMCID:PMC5106296

[25] Riaz A,Kulik LM,Lewandowski RJ,Ibrahim SM,Baker T,Newman S,Abecassis M,Salem R.Alpha-fetoprotein response after locoregional therapy for hepatocellular carcinoma: oncologic marker of radiologic response, progression, and surivival..J Clin Oncol2009;27:5734-42

[26] Giannini EG,Farinati F,Pecorelli A,Di Marco M,Zoli M,Cabibbo G,Gasbarrini A,Foschi FG,Morisco F,Virdone R.Determinants of alpha-fetoprotein levels in patients with hepatocelular carcinoma: implications for its clinical use..Cancer2014;120:2150-7

[27] Salem R,Sangro B.Yttrium 90 radioembolization for the treatment of hepatocellular carcinoma: biological lessons, current challenges and clinical perspectives..Hepatology2013;58:2188-97 PMCID:PMC3732811

[28] Riaz A,Kulik LM,Sato KT,Omary RA.Complications following radioembolization with yttrium-90 microspheres: a comprehensive literature review..J Vasc Interv Radiol2009;20:1121-30

[29] Verslype C,Rougier P.Hepatocellular carcinoma: ESMO-ESDO Clinical Practice Guidelines for diagnosis, treatment and follow-up..Ann Oncol2012;23:vii41-8