Patients suffering from different forms of acute hepatic porphyria present a high risk of primary liver cancer, specifically hepatocellular carcinoma and cholangiocarcinoma, determined by the activity of the disease even though an exact mechanism of carcinogenesis has not been recognized yet. Here, we present the clinical case of a 72-year-old woman who, approximately 29 years after the diagnosis of acute intermittent porphyria, presented with intrahepatic cholangiocarcinoma with a histological diagnosis of adenocarcinoma starting from the biliary-pancreatic ducts, which was diagnosed during the clinical and anatomopathological evaluation of a pathological fracture of the femur.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10178950PMC
http://dx.doi.org/10.3390/jcm12093091DOI Listing

Publication Analysis

Top Keywords

intrahepatic cholangiocarcinoma
8
acute intermittent
8
intermittent porphyria
8
cholangiocarcinoma acute
4
porphyria case
4
case report
4
report patients
4
patients suffering
4
suffering forms
4
forms acute
4

Similar Publications

Typically, patients with advanced cholangiocarcinoma have a poor prognosis because of the limited effective chemotherapy options available. Studies on genotype-directed therapies for cholangiocarcinoma are increasing. However, limited clinical data are currently available for evaluating the efficacy of molecular-targeted therapies.

View Article and Find Full Text PDF

Preventive interventions are expected to substantially improve the prognosis of patients with primary liver cancer, predominantly hepatocellular carcinoma (HCC) and cholangiocarcinoma. HCC prevention is challenging in the face of the evolving etiological landscape, particularly the sharp increase in obesity-associated metabolic disorders, including metabolic dysfunction-associated steatotic liver disease (MASLD). Next-generation anti-HCV and HBV drugs have substantially reduced, but not eliminated, the risk of HCC and have given way to new challenges in identifying at-risk patients.

View Article and Find Full Text PDF

High-intensity focused ultrasound thermal ablation (HIFU) is a novel non-invasive technique in the treatment of liver metastases (LIM) that allows focal destruction and is not affected by dose limits. This retrospective study aimed to explore the efficacy of HIFU in improving survival and the safety of the method in newly diagnosed patients with cancer with LIM who received first-line immune checkpoint inhibitor (ICI) therapy. Between January 2018 and December 2023, data from 438 newly diagnosed patients with cancer and LIM who were treated at Mianyang Central Hospital (Mianyang, China) were reviewed.

View Article and Find Full Text PDF
Article Synopsis
  • Clonorchis sinensis infection significantly worsens overall survival rates in patients with intrahepatic cholangiocarcinoma (ICC) compared to those without the infection.
  • Researchers used RNA sequencing and animal models to investigate how C. sinensis infection facilitates the progression of ICC.
  • The study found that C. sinensis infection leads to increased expression of fatty acid synthase (FASN), which promotes fatty acid synthesis and tumor growth, suggesting a potential new target for treatment in ICC patients infected with C. sinensis.
View Article and Find Full Text PDF

Common pancreatobiliary epithelial malignancies such as pancreatic ductal adenocarcinoma, cholangiocarcinoma and gallbladder carcinoma have poor prognosis. A small but significant portion of these malignancies arise from mass-forming grossly and radiologically visible premalignant epithelial neoplasms in the pancreatobiliary tree. Several lesions, including a few recently described entities, fall under this category and predominantly include papillary epithelial lesions with or without mucin production.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!