Case Report |
Corresponding author: Nikola Mumdzhiev ( niko_mu2002@yahoo.com ) © 2023 Nikola Mumdzhiev, Borislav Borisov, Rumen Tenev, Mariana Radicheva.
This is an open access article distributed under the terms of the Creative Commons Attribution License (CC BY 4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Citation:
Mumdzhiev N, Borisov B, Tenev R, Radicheva M (2023) Treating unresectable intrahepatic cholangiocarcinoma with transarterial chemoembolization and an unusual progression with cardiac involvement. Folia Medica 65(2): 326-330. https://doi.org/10.3897/folmed.65.e76329
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Intrahepatic cholangiocarcinoma is a rare type of cancer that is usually discovered at an advanced stage where surgical treatment is not an option. When compared to standard systemic therapy, transarterial chemoembolization (TACE) can provide a survival benefit to unresectable patients. Extrahepatic tumor spread is not rare, but cardiac involvement is an unusual complication.
We present the case of a 56-year-old man with histologically proven intrahepatic cholangiocarcinoma. Oncologic risk factors include hepatitis B and liver cirrhosis. Being in an unresectable stage of the disease, three TACE procedures were performed. Partial response was achieved (according to RECIST) leading to a 16-month survival. However, disease progression was present, with unusual heart metastases.
TACE can bring a survival benefit to unresectable cholangiocarcinoma patients. Defining the best disease stages in which TACE can be implemented and introducing it as part of standard treatment guidelines still presents a challenge.
cardiac metastases, intrahepatic cholangiocarcinoma, transarterial chemoembolization
Cholangiocarcinomas (CCCs) are a rare type of malignancies with an incidence rate of 0.3-6 per 100,000 people that may originate from the biliary tree.[
When discovered early, surgery is the treatment of choice. Most cases of iCCA (around 60%–88%) are often diagnosed at an advanced stage when surgery is not feasible. For these patients, systemic therapy is the standard practice.[
Oncologic progression is often defined by the appearance of distant metastases. Overall, cardiac involvement (when sought) is not that rare, accounting for 9% of all cancers.[
We present the case of a 56-year-old man who was admitted to the Gastroenterology Department complaining of jaundice and increasing waist circumference. The concomitant high blood pressure was treated adequately. The patient was neither smoker nor used excessive amounts of alcohol. Ultrasound examination of the abdomen revealed changes characteristic for hepatic cirrhosis (portal hypertension) - hepatosplenomegaly, ascites - and a hypoechoic nodule 5.7 cm in diameter in the right liver lobe (segments VII-VIII). The etiology of the disease was found to be hepatitis B – the patient was HBsAg (+) positive, anti-HBcore total (+) positive, anti-HCV(−) negative, anti-HDV (−) negative with a high HBV (hepatitis B virus) DNA load - 3 133 525 IU/ml. Significant portal hypertension was confirmed. An upper GI endoscopy showed esophageal varices grade II-III in the OMED classification. A CT scan of the abdomen with contrast characterized the liver nodule as a primary liver tumor, with normal Alpha-fetoprotein level and elevated level of the CA19-9 marker (166 IU/ml, normal ranges 0-39 IU/ml). At this point, the patient was classified as a newly-found cirrhotic. In Child score, he received 8 points (max. 15) - class B, while in MELD score he received 11 points. He started therapy with diuretics due to ascites and treatment of hepatitis B with nucleotide analogue (tenofovir, TDF).
Ascites was completely eliminated a month later, making a percutaneous liver biopsy feasible. The results of liver biopsy demonstrated an intrahepatic cholangiocarcinoma (immunohistochemical staining was positive for CK7 and CK 20). The disease stage was T1bN0M0 according to the 8th edition of the American Joint Cancer Committee (AJCC). [
With the outbreak of the COVID-19 pandemic, the patient ceased to attend the follow-ups for eight months. He presented to the Department with signs of ascites and shortness of breath. The ultrasound of the abdomen revealed multiple enlarged abdominal lymph nodes in addition to the ascites. Pericardial effusion was visible on ultrasound in subxiphoid view with a large mass protruding into the right ventricle. Contrast CT of the chest and abdomen verified multiple pulmonary metastases, severely enlarged (metastatic) lymph nodes in the mediastinum and abdominally (Fig.
A. Pre-TACE CT image of the CCC; B. CT image – a post-TACE shrinkage in tumor size, white arrows mark the liver tumor; C. angiographic image of the involved hepatic vessels pre-TACE; D. Post-TACE occlusion of the involved vessel.
The risk factors for intrahepatic cholangiocarcinoma seem to differ between perihilar and distal cholangiocarcinoma. Chronic ductal inflammation from flukes, primary sclerosing cholangitis (PSC), biliary cysts, or hepatolithiasis is not a crucial risk factor for iCCA. As acknowledged lately (and as in the presented case), the risk factors of iCCA seem to be the same as the ones for HCC, that is, hepatitis B or C, cirrhosis, alcohol consumption and obesity.[
Transarterial therapies aim to deliver a high load of chemotherapeutic agents in the tumor while sparing other organs; they are often combined with embolization of the tumor-feeding arteries. Several modalities exist – hepatic artery infusion (HAI), conventional TACE (cTACE), TACE with eluting drug beats (deb-TACE), selective internal radiation therapy (SIRT), and transarterial radioembolization (TARE). Each has provided survival advantages, but TACE seems to be the most practiced.[
Tumor metastasis to the heart is an uncommon finding. However, when searched for systemically in autopsies, they can be found in up to 9% of all autopsies for malignancy.[4] Often, as in our case, they are associated with widespread metastatic disease.[
TACE with biodegradable starch microspheres can be used as an alternative to standard chemotherapy in unresectable intrahepatic cholangiocarcinoma. Progression of the disease (CCC) can include atypical metastases such as myocardial involvement.