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| http://dx.doi.org/10.1016/j.sopen.2024.05.017 | DOI Listing |
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Updates in Biliary Atresia: Aetiology, Diagnosis and Surgery.
Children (Basel)
January 2025
Department of Paediatric Surgery, Kings College Hospital, Denmark Hill, London SE5 9RS, UK.
Biliary atresia (BA) is an obliterative disease of the bile ducts affecting between 1 in 10,000-20,000 infants with a predominance in Asian countries. It is clinically heterogeneous with a number of distinct variants (e.g.
View Article and Find Full Text PDFPrimary Cholangiocarcinoma of the Liver Presenting as a Complicated Hepatic Cyst: A Diagnostic Challenge.
Korean J Gastroenterol
January 2025
Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Primary cholangiocarcinoma is a rare bile duct epithelial neoplasm that can present with atypical clinical manifestations, complicating its diagnosis. A 62-year-old male showed symptoms suggestive of a complicated hepatic cyst that was later identified as intrahepatic cholangiocarcinoma. The patient presented with abdominal discomfort without fever.
View Article and Find Full Text PDFCase report: Cancer-free survival after chemotherapy, targeted immunotherapy combination with proton therapy following space making technique in a patient with cholangiocarcinoma after choledochal cyst resection.
Front Immunol
January 2025
Department of Hepatobiliary-Pancreatic-Splenic Surgery, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China.
Choledochal cysts (CCs) are rare cystic dilations of the intrahepatic and/or extrahepatic bile ducts. Malignancies arising during follow-up after excision of CCs have been reported in both children and adults, with no typical time frame for malignancy development. We present a case of a patient diagnosed with CCs 36 years ago, who underwent resection and subsequently developed cancer.
View Article and Find Full Text PDFCholangioblastic Cholangiocarcinoma (NIPBL::NACC1 cholangiocarcinoma): Expanded Morphologic Spectrum and Further Genetic Characterization.
Am J Surg Pathol
January 2025
Bioinformatics Core Facility, Lyda Hill Department of Bioinformatics, Department of Pathology University of Texas Southwestern Medical Center, Dallas, TX.
The cholangioblastic variant of intrahepatic cholangiocarcinoma is a distinctive neoplasm that typically affects young women without underlying liver disease. Morphologically, it demonstrates solid, trabecular, and tubulocystic architecture, biphasic small cell-large cell cytology, and immunoreactivity for inhibin, neuroendocrine markers, and biliary but not hepatocellular markers. In 2021, our group identified a characteristic NIPBL::NACC1 gene fusion in cholangioblastic cholangiocarcinoma, and since then ~20 genetically confirmed cases have been reported in the literature.
View Article and Find Full Text PDFCystic lesions and their mimics involving the intrahepatic bile ducts and peribiliary space: diagnosis, complications, and management.
Abdom Radiol (NY)
December 2024
Mallinckrodt Institute of Radiology, Washington University School of Medicine, Saint Louis, MO, USA.
Biliary and peribiliary cystic lesions represent a diverse group of abnormalities, often discovered incidentally during imaging for unrelated conditions. These lesions, typically asymptomatic, necessitate precise imaging modalities to characterize their nature and determine subsequent clinical actions, such as follow-up imaging, biopsy, or surgical referral. The anatomic location of these cystic lesions, whether biliary or peribiliary, influences both diagnostic and prognostic outcomes.
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