Pathologie der hepato-pankreato-biliären Tumoren

2021 ◽  
Vol 78 (10) ◽  
pp. 565-573
Author(s):  
Gieri Cathomas ◽  
Luigi M. Terracciano
Keyword(s):  
Small Duct ◽  
Fokale Noduläre Hyperplasie

Zusammenfassung. Die malignen Tumoren der hepato-pankreato-biliären Organe sind mehrheitlich typische Adenokarzinome mit Ausnahme der Leber, wo sie wegen ihrer charakteristischen Morphologie als hepatozelluläre Neoplasien bezeichnet werden. Neben diesen klassischen morphologischen Ansätzen haben in den letzten Jahren molekulare und vor allem morphomolekulare Untersuchungen viele neue Erkenntnisse ergeben, immer mit dem Ziel, den Patienten eine bestmögliche Therapie zukommen zu lassen. So wird heute zum Beispiel die fokale noduläre Hyperplasie (FNH) der Leber als reaktive Veränderung angesehen, welche keine weiteren Therapien braucht. Auf der einen Seite erlaubt die molekulare Stratifizierung der Leberzelladenome, Risikotumoren zu erkennen und entsprechend operativ zu behandeln. Als weiteres Beispiel seien Tumoren der extra- und intrahepatischen Gallengänge aufgeführt, welche heute in «Small-Duct-Type» und «Large-Duct-Type» unterschieden werden, welche unterschiedliche, therapeutisch relevante molekulare Profile aufweisen. Die vorliegende Arbeit gibt eine Übersicht über die wichtigsten und häufigsten Tumoren der hepato-pankreato-biliären Organe.

Small-duct sclerosing cholangitis associated with Schistosoma mansoni

Hepatology ◽  
2010 ◽  
Vol 53 (2) ◽  
pp. 712-713
Author(s):  
Catherine H. McCrann ◽  
James L. Boyer
Keyword(s):  
Schistosoma Mansoni ◽  
Sclerosing Cholangitis ◽  
Small Duct

Cytokeratin 7 and Copper Stains Facilitate Diagnosis of Small Duct Primary Sclerosing Cholangitis

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S125-S125
Author(s):  
A Wilhelm ◽  
H L Stevenson ◽  
K Kline
Keyword(s):  
Liver Biopsy ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Early Recognition ◽  
Clinical Suspicion ◽  
Cytokeratin 7 ◽  
Ductular Reaction ◽  
Elevated Alkaline Phosphatase ◽  
Pattern Of Injury ◽  
Small Duct

Abstract Introduction/Objective Classic primary sclerosing cholangitis (PSC) involves extrahepatic and/or intrahepatic biliary ducts with segmental biliary strictures and dilatations that often allow the diagnosis to be made via cholangiogram. Small duct PSC (sdPSC) is a rare subtype that presents similarly with a cholestatic pattern of injury, yet due to the small size of involved ducts, a cholangiogram is non-diagnostic and diagnosis is dependent on clinical suspicion and liver biopsy. The histopathological features of sdPSC are often subtle and may easily be overlooked. Diagnosis of this entity- though difficult- is important, as early recognition can facilitate the identification of associated disease processes and life-threatening complications. Methods/Case Report We encountered a 33-year-old female presenting with intermittent pruritis, episodes of jaundice, and persistently elevated alkaline phosphatase who was misdiagnosed with only fatty liver at an outside institution. Evaluation with MRCP showed no abnormalities within the biliary tract and a liver biopsy was performed to aid in the diagnosis. The H&E and trichrome findings of atrophic bile ducts and some peribiliary sclerosis were extremely subtle and may have been overlooked without clinical suspicion. Cytokeratin 7 (CK7) highlighted cholangiolar metaplasia in hepatocytes and the bile ductular reaction that occurs in cholestatic disease states. A Rhodamine copper stain showed periportal deposition suggestive of chronic biliary obstruction. Use of CK7 and copper stains supported the presence of chronic biliary injury and suboptimal bile flow, confirming the diagnosis of sdPSC. Results (if a Case Study enter NA) NA Conclusion Diagnosis of sdPSC has historically relied on H&E and trichrome stains. In this case, the findings on H&E and trichrome stains were non-diagnostic, while the use of CK7 and copper stains confirmed the diagnosis of sdPSC. We recommend using CK7 and copper stains to evaluate for sdPSC.

Prevalence of Gastroparesis in Patients with Small Duct Chronic Pancreatitis

Pancreas ◽  
2003 ◽  
Vol 26 (3) ◽  
pp. 235-238 ◽  
Author(s):  
Riaz S. Chowdhury ◽  
Chris E. Forsmark ◽  
Richard H. Davis ◽  
Phillip P. Toskes ◽  
G. Nicholas Verne
Keyword(s):  
Chronic Pancreatitis ◽  
Small Duct

Small-duct primary sclerosing cholangitis: Prevalence and natural history

2000 ◽  
Vol 118 (4) ◽  
pp. A902 ◽  
Author(s):  
Paul Angulo ◽  
Yaacov Maor-Kendler ◽  
Jessica J. Donlinger ◽  
Keith D. Lindor
Keyword(s):  
Natural History ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Small Duct

Papilloma, Large Duct and Small Duct

2016 ◽  
pp. 146-155
Author(s):  
David G. Hicks ◽  
Susan C. Lester
Keyword(s):  
Small Duct

scholarly journals Different iron-handling in inflamed small and large cholangiocytes and in small and large-duct type intrahepatic cholangiocarcinoma

2020 ◽  
Vol 64 (4) ◽  
Author(s):  
Romina Mancinelli ◽  
Antimo Cutone ◽  
Luigi Rosa ◽  
Maria Stefania Lepanto ◽  
Paolo Onori ◽  
...  
Keyword(s):  
Iron Homeostasis ◽  
Tumor Development ◽  
In Vitro Models ◽  
Molecular Forms ◽  
Cellular Processes ◽  
Small Duct ◽  
Transferrin Receptor 1 ◽  
The Difference ◽  
Tumor Management

Cholangiocarcinoma (CCA) represents the second most common primary hepatic malignancy and originates from the neoplastic transformation of the biliary cells. The intrahepatic subtype includes two morpho-molecular forms: large-duct type intrahepatic CCA (iCCA) and small-duct type iCCA. Iron is fundamental for the cellular processes, contributing in tumor development and progression. The aim of this study was to evaluate iron uptake, storage, and efflux proteins in both lipopolysaccharide-inflamed small and large cholangiocytes as well as in different iCCA subtypes. Our results show that, despite an increase in interleukin-6 production by both small and large cholangiocytes, ferroportin (Fpn) was decreased only in small cholangiocytes, whereas transferrin receptor-1 (TfR1) and ferritin (Ftn) did not show any change. Differently from in vitro models, Fpn expression was increased in malignant cholangiocytes of small-duct type iCCA in comparison to large-duct type iCCA and peritumoral tissues. TfR1, Ftn and hepcidin were enhanced, even if at different extent, in both malignant cholangiocytes in comparison to the surrounding samples. Lactoferrin was higher in large-duct type iCCA in respect to small-duct type iCCA and peritumoral tissues. These findings show a different iron handling by inflamed small and large cholangiocytes, and small and large-duct type iCCA. The difference in iron homeostasis by the iCCA subtypes may have implications for the tumor management.

scholarly journals Intrahepatic cholangiocarcinomas with IDH1/2 mutation-associated hypermethylation at selective genes and their clinicopathological features

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Kyoungbun Lee ◽  
Young Seok Song ◽  
Yoonju Shin ◽  
Xianyu Wen ◽  
Younghoon Kim ◽  
...  
Keyword(s):  
Dna Methylation ◽  
Cpg Island ◽  
Methylation Status ◽  
Intraepithelial Neoplasia ◽  
Clinicopathological Features ◽  
Isocitrate Dehydrogenase 1 ◽  
Cancer Specific Survival ◽  
Mutation Status ◽  
Mucin Production ◽  
Small Duct

Abstract Intrahepatic cholangiocarcinoma (ICC) is a rare but fatal tumor. The isocitrate dehydrogenase 1 and 2 (IDH1/2) genes are known to be mutated in ICC. IDH1/2 mutations tend to be accompanied by enhanced hypermethylation at a subset of genomic loci. We sought to clarify the clinicopathological features, including prognostic value, of ICCs with IDH1/2 mutation-associated hypermethylation at a subset of genes. The mutation status of IDH1/2 and methylation status of 30 gene CpG island loci were analyzed in 172 cases of ICC using pyrosequencing and the MethyLight assay, respectively. The mutation status of IDH1/2 was correlated with clinicopathological features and the DNA methylation status at 30 gene loci. Then, the clinicopathological characteristics were analyzed regarding three-tiered methylation statuses in genes showing IDH1/2 mutation-associated methylation. IDH1/2 mutations were found in 9.3% of ICCs, and IDH1/2-mutated tumors were associated with the histological subtype, including the bile ductular type and small duct type, and poor differentiation. Eight DNA methylation markers showed associations with IDH1/2 mutations, and ICCs with > 5/8 methylated markers were associated with the bile ductular type or small duct type, absence of mucin production, absence of biliary intraepithelial neoplasia, and presence of chronic liver disease. > 5/8 methylated markers were an independent prognostic marker associated with better survival in both cancer-specific survival and recurrence-free survival. In summary, by analyzing the association between IDH1/2 mutations and DNA methylation in individual genes, we developed a panel of DNA methylation markers that were significantly associated with IDH1/2 mutations and were able to identify a subset of ICC with better clinical outcomes.

Magnetic resonance imaging features of small-duct primary sclerosing cholangitis

2020 ◽  
Vol 45 (8) ◽  
pp. 2388-2399
Author(s):  
Kazuto Kozaka ◽  
Shannon P. Sheedy ◽  
John E. Eaton ◽  
Sudhakar K. Venkatesh ◽  
Jay P. Heiken
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Imaging Features ◽  
Resonance Imaging ◽  
Small Duct
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