Fibrolamellar hepatocellular carcinoma presenting with budd-chiari syndrome, right atrial thrombus, and pulmonary emboli

Summary Background There have been encouraging reports on transjugular intrahepatic portosystemic stent‐shunt (TIPSS) for Budd–Chiari syndrome (BCS). Long‐term data are lacking. Aim To assess long‐term outcomes and validate prognostic scores following TIPSS for BCS. Methods A single centre retrospective study. Patients underwent TIPSS using bare or polytertrafluoroethane (PTFE)‐covered stents. Results Sixty‐seven patients received successful TIPSS between 1996 and 2012 using covered (n = 40) or bare (n = 27) stents. Patients included had a Male: Female ratio of 21:46, and were characterised (mean ± s.d.) by age 39.9 ± 14.3 years, Model of end stage liver disease (MELD) 16.1 ± 7.0 and Child's score 8.8 ± 2.0. Seventy‐eight percent had haematological risk factors. Presenting symptoms were ascites (n = 61) and variceal bleeding (n = 6). Nine patients underwent hepatic vein dilatation or stenting prior to TIPSS. Mean follow‐up was 82 months (range 0.5–184 months). Fifteen percent had post‐TIPSS encephalopathy. Two have been transplanted. Primary patency rates (76% vs. 27%, P < 0.001) and shunt re‐interventions (22% vs. 100%, P < 0.001) significantly favoured covered stents. Secondary patency was 99%. Six‐, 12‐, 24‐, 60‐ and 120‐month survival was 97%, 92%, 87%, 80% and 72% respectively. Six patients had liver related deaths. Two patients developed hepatocellular carcinoma. The BCS TIPS PI independently predicted mortality in the whole cohort, but no prognostic score was a significant predictor of mortality after subgroup validation. Conclusions Long‐term outcomes following TIPSS for Budd–Chiari syndrome are very good. PTFE‐covered stents have significantly better primary patency. The value of prognostic scores is controversial. TIPSS should be considered as first line therapy in symptomatic patients in whom hepatic vein patency cannot be restored.

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“…There are multiple case reports of hepatocellular carcinoma associated with BCS . The absolute risk of HCC development is unknown.…”

Section: Discussionmentioning

confidence: 99%

“…There are multiple case reports of hepatocellular carcinoma associated with BCS. [25][26][27][28] The absolute risk of HCC development is unknown. One study showed HCC developed in 11 of 97 patients followed up for a mean of 5 years; the cumulative incidence of HCC during follow-up was 4%.…”

Section: Discussionmentioning

confidence: 99%

Good clinical outcomes following transjugular intrahepatic portosystemic stent‐shunts in Budd–Chiari syndrome

Tripathi

MacNicholas

Kothari

et al. 2014

Aliment Pharmacol Ther

103

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“…Also, it has been described in the literature the stepby-step occlusion of one vein followed by two or more veins, with development of venous collaterals, without significant symptoms [3]. Inferior vena cava and right atrium thrombosis, with secondary Budd-Chiari syndrome, has been rarely reported as the presenting feature of HCC [6,7]. Patients with Budd-Chiari syndrome secondary to hepatocellular carcinoma have a poor prognosis [5].…”

Section: Discussionmentioning

confidence: 99%

Budd-Chiari syndrome as an initial presentation of hepatocellular carcinoma: a case report

2014

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We report the case of a 84-year-old admitted with symptoms of congestive heart failure. Ultrasonography revealed a hyperechoic nodule in the left lobe of the liver, with a peripheral hypoechoic rim, multiple irregular hypoechoic nodules in both hepatic lobes, portal vein, inferior vena cava, and right atrium thrombosis. On ultrasonographic and alpha-fetoprotein criteria the case was interpreted as hepatocellular carcinoma with Budd-Chiari syndrome. The particularity of the case is the initial presentation of the hepatocellular carcinoma as Budd-Chiari syndrome. The inferior vena cava and right atrium thrombosis, as a cause of secondary Budd-Chiari syndrome in a patient with hepatocellular carcinoma, has been rarely reported.

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“…Jaundice may be seen in up to 40% of cases (Liu et al, 2009). Patients may also present with various rare symptoms or signs, such as gynecomastia in men or children (McCloskey et al, 1988;Muramori et al, 2011), metastatic lesions in other organs such as the bone (Kutluk et al, 2001), lung (Mroz et al, 2010), pancreas (Thirabanjasak et al, 2009) and ovary (Benito et al, 2012), hyperammonemic encephalopathy (Sethi et al, 2009), cold agglutinin disease (Al-Matham et al, 2011), shoulder pain (Moghadam et al, 2008), severe inferior vena cava obstruction caused by cardiac spread (Knudson et al, 2012), recurrent deep vein thrombosis (Marrannes et al, 2005), paraneoplastic hyperthyroidism (Carri et al, 1989), Budd-Chiari syndrome with right atrial thrombus, and pulmonary emboli (Asrani and LaRusso, 2012), nonbacterial thrombotic endocarditis (Vaideeswar et al, 1993), and hypoglycemia (Tangkijvanich et al, 2000). The serum levels of aspartate aminotransferase, alanine aminotransferase, and α-fetoprotein (AFP) are usually normal but may be mildly elevated in a minority of cases.…”

Section: Clinicsmentioning

confidence: 99%

Liver: Fibrolamellar carcinoma

Zhang¹,

Ward²

2012

Atlas of Genetics and Cytogenetics in Oncology and Haematology

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Fibrolamellar hepatocellular carcinoma presenting with budd-chiari syndrome, right atrial thrombus, and pulmonary emboli

Cited by 19 publications

References 6 publications

Good clinical outcomes following transjugular intrahepatic portosystemic stent‐shunts in Budd–Chiari syndrome

Good clinical outcomes following transjugular intrahepatic portosystemic stent‐shunts in Budd–Chiari syndrome

Budd-Chiari syndrome as an initial presentation of hepatocellular carcinoma: a case report

Liver: Fibrolamellar carcinoma

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