Lines of Zahn in a Patient With Transjugular Intrahepatic Portosystemic Shunt Occlusion From a Tumor Thrombus: A Case Report

TL;DR

A patient with advanced liver cancer experienced TIPS occlusion due to a tumor thrombus, highlighting the need for close monitoring in similar cases.

Contribution

This is the first reported case of hepatocellular carcinoma causing TIPS occlusion with tumor thrombus and lines of Zahn.

Findings

Tumor thrombus occluded the TIPS, confirmed by imaging and microscopic analysis.

Lines of Zahn were observed, indicating acute thrombus formation.

TIPS revision improved flow and reduced portal venous pressures.

Abstract

Hepatocellular carcinoma (HCC) in patients with alcohol-related cirrhosis is commonly associated with complications such as ascites, variceal bleeding, hepatic encephalopathy, coagulopathy, and portal vein thrombosis (PVT). A significant number of patients with HCC require transjugular intrahepatic portosystemic shunt (TIPS) placement. To date, there have been no reported cases of HCC with tumor thrombus occluding a TIPS. We present the case of a 50-year-old man with alcohol-related cirrhosis, status post-TIPS placement, and unresectable stage IIIA/Barcelona Clinic Liver Cancer (BCLC) C HCC, who had been treated with brachytherapy. He presented with painful abdominal distension. Physical examination revealed large-volume ascites and caput medusae. Diagnostic paracentesis was performed, draining approximately 3 liters of sanguineous ascitic fluid. Laboratory analysis indicated non-neutrocytic, culture-negative transudative hemorrhagic ascites. A computed tomography (CT) scan of the abdomen and pelvis revealed interspersed areas of contrast-enhanced opacification within the TIPS, suggestive of partial TIPS thrombosis. Ultrasound of the liver with Doppler demonstrated slow TIPS flow with reversal of flow throughout the entire portal venous system, overall concerning for shunting of blood away from the TIPS and TIPS malfunction. Interventional radiology was consulted for TIPS revision and thrombectomy. The patient underwent aspiration thrombectomy, balloon venoplasty, and TIPS extension. Post-procedural TIPS venography demonstrated a significantly increased TIPS caliber, improved flow through the TIPS, and reduced portal venous pressures. Microscopic analysis of the evacuated thrombus revealed fragments of HCC with necrosis. Additionally, a rare pathology finding, lines of Zahn, was observed, indicating acute thrombus formation. Given the involvement of the portal vein, the patient was upstaged to American Joint Committee on Cancer (AJCC) stage IIIB/BCLC C. Oncology was consulted, with initial plans for first-line systemic therapy using tremelimumab and durvalumab contingent on clinical improvement and reduction in bilirubin levels below 3 mg/dL. However, given the patient's Child-Pugh Class C (score of 11), the potential benefit from systemic therapy was deemed limited. Outpatient follow-up and treatment reevaluation were planned. On hospital day 9, the patient was discharged in stable condition with oncology follow-up scheduled. This case highlights a rare but serious complication of TIPS placement in patients with advanced HCC, underscoring the importance of close monitoring for TIPS occlusion due to tumor thrombus, which can significantly affect treatment outcomes.

Figures