A rare but fatal outcome is hepatocellular carcinoma (HCC) progressing to the right atrial tumor thrombus and inferior vena cava

Abstract

Hepatocellular carcinoma (HCC) frequently presents with vascular invasion; however, extension of tumor thrombus into the right atrium via the inferior vena cava (IVC) is rare and associated with a poor prognosis. A 48-year-old man presented with progressive dyspnea on minimal exertion. Imaging studies, including transthoracic echocardiography and contrast-enhanced computed tomography, revealed a large hepatic mass with tumor thrombus extending through the hepatic veins and inferior vena cava (IVC) into the right atrium. Due to advanced liver disease and poor performance status, surgical and interventional therapies were not feasible. The patient received supportive treatment including anticoagulation with low molecular weight heparin. Despite treatment, his clinical condition rapidly deteriorated, leading to death shortly after diagnosis. This case highlights the importance of multimodal imaging in identifying intracardiac extension of Hepatocellular carcinoma (HCC) and underscores its grave prognosis.

Introduction

The most prevalent form of cancer of the liver, accounting for between 75% and 90% of all primary liver malignancies, is Hepatocellular carcinoma (HCC), which remains a global health concern [1]. Males are more likely than females to be affected by HCC, with a male-to-female ratio of 3:1. For HCC, the 5-year survival rate is about 18% [2,3]. Regardless of the cause, liver cirrhosis is one of the main risk factors for the development of HCC. Hepatitis B virus (HBV) was responsible for 56% of HCC cases, according to 1 study, while hepatitis C virus (HCV) was responsible for 20% [4]. Non-alcoholic fatty liver disease (NAFLD), obesity, the metabolic syndrome, diabetes mellitus, smoking, heavy consumption of alcohol, eating foods that contain aflatoxin B1 in the diet, and genetic conditions like alpha-1 antitrypsin deficiency and hereditary hemochromatosis are additional potential risk factors for HCC.

The lungs, abdominal lymph nodes, bone, and adrenal gland are the primary sites of HCC metastases. Even though HCC has a higher propensity for vascular invasion and the heart is located upstream from the liver through the inferior vena cava (IVC), intracardiac tumor thrombus metastasis is extremely rare [5]. The formation of bland thrombus is also common in patients with HCC, as HCC induces deviations in blood flow, impaired endothelial function, and a hypercoagulable condition [6].

In this case, we describe a patient who had a component of hepatic pathology and initially showed signs and symptoms of minor dyspnea on exertion. Ultrasound and CT scans showed an intensifying intrahepatic lesion with tumor thrombus extension into the IVC and right heart, along with significantly elevated alpha-fetoprotein (AFP) values. The unusual presentation of HCC is emphasized in this instance, as is the significance of transthoracic echocardiography and abdominal ultrasonography for HCC surveillance in those at risk to diagnose the illness early.

Case presentation

This 48-year-old man arrived at us as an outpatient with anorexia, dyspnea with minimal exertion for a week, and widespread body weakness for a month. A male with a Glasgow coma scale (GCS) score of 15/15 was found to be unwell. His blood pressure (BP) was 110/70, his respiratory rate was 18 breaths per minute, his oxygen saturation was 96% (room air), and his pulse rate was 86 beats per minute (regular). Additionally, he had mild bilateral pedal edema and a jugular venous pulse (JVP) up to 8 cm; however, no peripheral signs of chronic liver disease were found. The patient's systemic examination showed a slightly enlarged, non-tender abdomen with positive shifting dullness. He had no tricuspid systolic murmur and normal S1 and S2 heart sounds, as indicated by his cardiovascular assessment. On respiratory examination, the lower zones of the lungs revealed reduced breath sounds and bilateral dullness on percussion. Examination showed an elevated JVP, mild dyspnea, and minor bilateral pedal edema, which were mixed signs of right heart failure and liver failure.

A Doppler study determined that the mass lesion abutting the adjacent intrahepatic IVC was internally vascular, and an echogenic thrombus was seen at the confluence of the hepatic veins, likely resulting from tumor extension. The patient's abdominal ultrasound scan revealed an enlarged liver with a single lesion in the right lobe of the liver measuring about 125×80 mm, along with mild abdominal and pelvic ascites. Due to a massive tumor thrombus that extended to the right atrium and had an intracardiac lesion measuring roughly 7.2×7.2 mm, the IVC was dilated and obstruction (Fig. 1).

Fig. 1.

The ultrasound revealed a massive thrombus in IVC (left). Echocardiography showed thrombus in the right atrium (right).

An abdominal CT scan showed a 17.6 cm enlarged liver and a hypodense lesion with internal necrosis that occupied nearly the whole right lobe which mass was 54×60×54 mm, extending to the subcapsular region and surrounding neovascularization. As a result, the CT scan report proved the existence of HCC with intracardiac tumor thrombus extension and IVC (Fig. 2). He had abnormal liver function tests (LFT) with hepatitis B and extremely high alpha-fetoprotein (AFP) levels (>1000 IU/L) upon additional workup. Apart from somewhat low fibrinogen levels and elevated fibrinogen degradation products, the coagulation profile was otherwise normal, with an INR of 1.41 and a prothrombin time of 15 seconds (normal range 9.7-12.2) (Table 1).

Fig. 2.

The CTscan revealed the IVC was dilated, which resulted from a massive thrombus, and showed a tumor thrombus extending to the right atrium with about a 7.2×7.2 mm intracardiac lesion.

Table 1.

Laboratory investigations.

Tumor maker	Alpha-fetoprotein	> 1000IU/L
Liver function tests	Serum billirubin	30.4 (<24)
	AST	180 U/L (<50U/L)
	ALT	74.7 U/L (<50 U/L)
	GGT	206 U/L (< 60U/L)
Virology	HBV	Posive
	HCV	Negative
	HIV	Negative
Coagulation profile	PT (INR)	15 seconds (9.7-12.2)
	Platelets	166.000 G/L
	Fibrinogen	1.6 g/dl (2-4g/dl)

AST, aspartate transaminase, ALT, alanine transaminase, HBV, hepatitis B virus, HCV, hepatitis C virus, HIV, human immunodeficiency virus, PT, prothrombin time, INR, international normalized ratio.

His echocardiography indicated an intracardiac thrombus extending from the IVC, his chest X-ray showed no pleural effusion and a normal cardiothoracic ratio, and his electrocardiogram (ECG) showed normal sinus rhythm. Medical treatment was chosen over surgical or radiological intervention because of the patient's performance status, advanced stage of liver disease, liver function, and chronic liver disease.

Discussion

Primary liver cancer is the fourth leading cause of cancer death globally and the sixth most prevalent type of cancer to be diagnosed. Among liver cancers, HCC is the most prevalent type. Aflatoxin, steatosis liver disease linked to metabolic dysfunction, alcohol-associated liver disease, HBV infection, and hepatitis C virus (HCV) infection are important risk factors for HCC [7].

It is rare for HCC to emerge as intracardiac metastases of the tumor thrombus. Both a direct spread of HCC from different surrounding structures into the heart and a continuous hematogenous spread of the tumor thrombus via the IVC into the heart are possible [8]. In Japan, the incidence of HCC linked to a tumor thrombus that extends through the IVC up to the right atrium is rare, occurring in 2.9% by imaging methods, 0.7% after surgery, and 18.2% during autopsy [9]. If untreated or not diagnosed in early stages, the average survival time for thrombus formation and its expansion to the right heart caused by HCC is 3 days to 2 months. On the other hand, 1 study reported that surgery may improve 1-year survival to 40%, emphasizing the importance of early intervention [10].

However, early echocardiography for screening for cardiac involvement in patients with a high tumor burden of HCC may prevent this diagnostic delay. A biopsy sample and cardiac catheterization can be implemented to definitively diagnose the cardiac mass as metastatic HCC. Hypo fractionated radiation therapy and surgery with embolectomy for HCC related to tumor thrombus formation are 2 potentially effective procedures that may eventually be used to improve the overall condition and enhance patients' progression-free survival [10].

Depending on the patient's underlying comorbidities, hepatic functional reserve, and the extent of tumor spread, various therapy approaches may be implemented to treat intracardiac tumor thrombus extension. According to research by Pesi et al., [11] they include radiofrequency ablation, chemotherapy, trans-arterial chemoembolization (TACE), and surgical resection with embolectomy, which overall had a 7%-18% one-year improvement in survival.

Regarding survival benefit, surgery has been shown to help prevent or treat acute life-threatening complications like pulmonary embolism, valvular (tricuspid) compromise, and right ventricular outflow obstruction. It is also used palliatively to lessen the severity of heart failure symptoms. Although the best surgical technique is not standardized, there are a few commonly utilized techniques that have shown promising outcomes [11].

In our instance, the features of the Echo and CT scans suggested tumor thrombus in the IVC, and RA is a risk factor for pulmonary embolism as well. Low molecular weight heparin (LMWH) might be started as a preoperative therapeutic anticoagulant in situations with tumor thrombus that are totally or almost obstructing the IVC, embolic events, or bland thrombus in addition to tumor thrombus. With a goal INR of 2-3, warfarin may be initiated in individuals who are contraindicated for long-term LMWH or who cannot access long-term LMWH [12].

In conclusion

Intracardiac extension of HCC tumor thrombus should be considered in patients with HCC presenting with symptoms of right-sided heart failure. Early recognition through multimodal imaging is essential for diagnosis and therapeutic planning. Despite available treatment options, prognosis remains poor in patients with advanced disease and limited hepatic reserve.

Ethical approval

The Ethics Committee of Hung Vuong Gia Lai Hospital.

Patient consent

Written informed consent was obtained from the patient to publish this report by the journal's patient consent policy.