Mixed adenoneuroendocrine carcinoma of the extrahepatic bile duct: a case report

Background The concept of mixed adenoneuroendocrine carcinoma (MANEC) was introduced in the 2010 World Health Organization classification of digestive neuroendocrine neoplasms. Bile duct invasion by MANEC is exceptionally rare. We herein report a case of MANEC with invasion of multiple bile ducts. Case presentation: A 60-year-old man presented with a 7-day history of upper abdominal pain, and a mass in the cystic duct was suspected based on computed tomography findings. The patient underwent resection of the extrahepatic bile ducts with concomitant radical lymphadenectomy and Roux-en-Y cholangiojejunostomy. Large cell neuroendocrine carcinoma was detected in a component of the resected tumor. According to the pathological and immunohistochemical features of the tumor, the final histopathological diagnosis was a biliary MANEC, tumor stage T2N0M1 (Stage IIIC). The patient recovered uneventfully and was discharged from the hospital 10 days after surgery. Conclusions We have described a rare case of extrahepatic MANEC invading multiple bile ducts, with particular emphasis on the physician’s awareness of MANEC and its optimal treatment. MANEC arising from extrahepatic bile ducts is rare, and surgical resection is the most effective treatment method.

Download Full-text

Proximal Extrahepatic Bile Ducts: Comprehensive Review

Journal of oncology: diagnostic radiology and radiotherapy ◽

10.37174/2587-7593-2021-4-1-74-93 ◽

2021 ◽

Vol 4 (1) ◽

pp. 74-93

Author(s):

M. A. Shorikov ◽

O. N. Sergeeva ◽

M. G. Lapteva ◽

N. A. Peregudov ◽

B. I. Dolgushin

Keyword(s):

Bile Duct ◽

Bile Flow ◽

Bile Ducts ◽

Extrahepatic Bile Duct ◽

Hepatic Duct ◽

Biliary Tree ◽

Comprehensive Review ◽

Extrahepatic Bile Ducts ◽

And Function ◽

Variant Anatomy

Proximal extrahepatic bile ducts are the biliary tree segment within formal boundaries from cystic ductcommon hepatic duct junction to sectoral hepatic ducts. Despite being a focus of attention of diagnostic and interventional radiologists, endoscopists, hepatobiliary surgeons and transplantologists they weren’t comprehensively described in available papers. The majority of the authors regard bile duct confluence as a group of merging primitively arranged tubes providing bile flow. The information on the proximal extrahepatic bile duct embryonal development, variant anatomy, innervation, arterial, venous and lymphatic supply is too general and not detailed. The present review brought together and systemized exiting to the date data on anatomy and function of this biliary tract portion. Unique, different from the majority of hollow organs organization of the proximal extrahepatic bile duct adapts them to the flow of the bile, i.e. viscous aggressive due to pH about 8.0 and detergents fluid, under higher wall pressure than in other parts of biliary tree.

Download Full-text

Efemp1 modulates elastic fiber formation and mechanics of the extrahepatic bile duct

10.1101/2021.12.05.471313 ◽

2021 ◽

Author(s):

Jessica Llewellyn ◽

Emilia Roberts ◽

Chengyang Liu ◽

Ali Naji ◽

Richard K. Assoian ◽

...

Keyword(s):

Extracellular Matrix ◽

Bile Duct ◽

Biliary Atresia ◽

Bile Ducts ◽

Matrix Protein ◽

Extrahepatic Bile Duct ◽

Fiber Formation ◽

Extracellular Matrix Protein ◽

Elastic Fibers ◽

Extrahepatic Bile Ducts

AbstractEGF-Containing Fibulin Extracellular Matrix Protein 1 (EFEMP1, also called fibulin 3) is an extracellular matrix protein linked in a genome-wide association study to biliary atresia, a fibro-inflammatory disease of the neonatal extrahepatic bile duct. EFEMP1 is expressed in most tissues and Efemp1 null mice have decreased elastic fibers in visceral fascia; however, in contrast to other short fibulins (fibulins 4 and 5), EFEMP1 does not have a role in the development of large elastic fibers, and its overall function remains unclear. We demonstrated that EFEMP1 is expressed in the submucosa of both neonatal and adult mouse and human extrahepatic bile ducts and that, in adult Efemp1+/- mice, elastin organization into fibers is decreased. We used pressure myography, a technique developed to study the mechanics of the vasculature, to show that Efemp1+/- extrahepatic bile ducts are more compliant to luminal pressure, leading to increased circumferential stretch. We conclude that EFEMP1 has an important role in the formation of elastic fibers and mechanical properties of the extrahepatic bile duct. These data suggest that altered expression of EFEMP1 in the extrahepatic bile duct leads to an abnormal response to mechanical stress such as obstruction, potentially explaining the role of EFEMP1 in biliary atresia.

Download Full-text

Cholangioscopy in diagnosis of diseases of extrahepatic bile ducts

Medical alphabet ◽

10.33667/2078-5631-2019-1-6(381)-30-36 ◽

2019 ◽

Vol 1 (6) ◽

pp. 30-36

Author(s):

A. G. Shuleshov ◽

N. V. Fomicheva ◽

D. N. Ulyanov ◽

A. S. Balalykin ◽

D. V. Danilov ◽

...

Keyword(s):

Differential Diagnosis ◽

Bile Duct ◽

Bile Ducts ◽

Extrahepatic Bile Duct ◽

Diagnostic Purpose ◽

Vascular Pattern ◽

Direct Visualization ◽

Bile Duct Diseases ◽

Extrahepatic Bile Ducts ◽

Diagnosis Of Diseases

An analysis of the diagnosis of extrahepatic bile duct diseases in 115 patients is presented. With the diagnostic purpose they performed ERCP, EPT, cholangioscopy. The method of direct visualization of the mucous membrane of the bile ducts allows you to identify endoscopic signs of strictures. Differential diagnosis of benign and malignant strictures of the bile ducts using cholangioscopy is difficult. Nevertheless, we were able to identify some typical signs for malignant strictures, including ulceration and mucosal infiltration, vascular pattern irregularity, stricture asymmetry.

Download Full-text

Coordinated development of the mouse extrahepatic bile duct: implications for neonatal susceptibility to biliary injury

10.1101/576256 ◽

2019 ◽

Cited By ~ 1

Author(s):

Gauri Khandekar ◽

Jessica Llewellyn ◽

Alyssa Kriegermeier ◽

Orith Waisbourd-Zinman ◽

Nicolette Johnson ◽

...

Keyword(s):

Bile Acid ◽

Bile Duct ◽

Biliary Atresia ◽

Bile Ducts ◽

Extrahepatic Bile Duct ◽

Collagen I ◽

Cell Junctions ◽

Reporter Mouse ◽

Extrahepatic Bile Ducts

AbstractBackground & AimsThe extrahepatic bile duct is the primary tissue initially affected by the cholangiopathy biliary atresia. Biliary atresia affects neonates exclusively and current animal models suggest that the developing bile duct is uniquely susceptible to damage. In this study, we aimed to define the anatomical and functional differences between the neonatal and adult mouse extrahepatic bile ducts.MethodsWe studied mouse passaged cholangiocytes, mouse BALB/c neonatal and adult primary cholangiocytes and isolated extrahepatic bile ducts, and a collagen reporter mouse. Methods included transmission electron microscopy, lectin staining, immunostaining, rhodamine uptake assays, bile acid toxicity assays, and in vitro modeling of the matrix.ResultsThe cholangiocyte monolayer of the neonatal extrahepatic bile duct was immature, lacking the uniform apical glycocalyx and mature cell-cell junctions typical of adult cholangiocytes. Functional studies showed that the glycocalyx protected against bile acid injury and that neonatal cholangiocyte monolayers were more permeable than adult monolayers. In adult ducts, the submucosal space was filled with collagen I, elastin, hyaluronic acid, and proteoglycans. In contrast, the neonatal submucosa had little collagen I and elastin, although both increased rapidly after birth. In vitro modeling suggested that the composition of the neonatal submucosa relative to the adult submucosa led to increased diffusion of bile. A Col-GFP reporter mouse showed that cells in the neonatal but not adult submucosa were actively producing collagen.ConclusionWe identified four key differences between the neonatal and adult extrahepatic bile duct. We showed that these features may have functional implications, suggesting the neonatal extrahepatic bile ducts are particularly susceptible to injury and fibrosis.Lay SummaryBiliary atresia is a disease that affects newborns and is characterized by extrahepatic bile duct injury and obstruction with resulting liver injury. We identify four key differences between the epithelial and submucosal layers of the neonatal and adult extrahepatic bile duct and show that these may render the neonatal duct particularly susceptible to injury.HighlightsThe apical glycocalyx is thin and patchy in neonatal compared to adult cholangiocytesNeonatal cholangiocytes have immature cell-cell junctions and increased permeabilityThe neonatal submucosal space has minimal collagen I or elastinThe neonatal submucosal space contains many actively collagen-secreting cellsGraphical abstract

Download Full-text

Adenocarcinoma with pyloric gland phenotype of the extrahepatic bile ducts: a previously unrecognized and distinctive morphologic variant of extrahepatic bile duct carcinoma

Human Pathology ◽

10.1016/j.humpath.2012.04.003 ◽

2012 ◽

Vol 43 (12) ◽

pp. 2292-2298 ◽

Cited By ~ 7

Author(s):

Jorge Albores-Saavedra ◽

Fredy Chablé-Montero ◽

Nahum Méndez-Sánchez ◽

Miguel Ángel Mercado ◽

Mario Vilatoba-Chapa ◽

...

Keyword(s):

Bile Duct ◽

Bile Ducts ◽

Extrahepatic Bile Duct ◽

Bile Duct Carcinoma ◽

Pyloric Gland ◽

Duct Carcinoma ◽

Extrahepatic Bile Duct Carcinoma ◽

Extrahepatic Bile Ducts

Download Full-text

Mixed adenoneuroendocrine carcinoma of the hepatic bile duct: a case report and review of the literature

BMC Gastroenterology ◽

10.1186/s12876-020-01550-2 ◽

2020 ◽

Vol 20 (1) ◽

Author(s):

Sulai Liu ◽

Zhendong Zhong ◽

Meng Xiao ◽

Yinghui Song ◽

Youye Zhu ◽

...

Keyword(s):

Bile Duct ◽

Neuroendocrine Tumors ◽

Radical Surgery ◽

Malignant Tumors ◽

World Health ◽

Ki 67 ◽

Mixed Adenoneuroendocrine Carcinoma ◽

Hilar Bile Duct ◽

Hilar Tumor

Abstract Background The World Health Organization's updated classification of digestive system neuroendocrine tumors in 2010 first proposed the classification of mixed adenoneuroendocrine carcinoma (MANEC). The incidence of biliary malignant tumors with neuroendocrine tumors accounts for less than 1% of all neuroendocrine tumors. Moreover, the incidence of hilar bile duct with MANEC is very rare. Case presentation A 65-year-old female patient came to our hospital for repeated abdominal pain for more than 4 months and skin sclera yellow staining for 1 week. Contrast-enhanced computed tomography imaging and magnetic resonance results suggested a hilar tumor for Bismuth-Corlette Type II. The patient underwent radical surgery for hilar cholangiocarcinoma. Finally, the patient was diagnosed with hilar bile duct MANEC, staged 1 (pT1N0M0) based on the eighth edition of the AJCC. Histopathology showed that the tumor was a biliary tumor with both adenocarcinoma and neuroendocrine carcinoma. No evidence of recurrence and metastasis after 20 months of follow-up. Conclusions We first reported a MANEC that originated in the hilar bile duct. As far as we known, there were few reports of biliary MANEC, and the overall prognosis was poor. We also found that the higher the Ki-67 index, the worse the prognosis of this type of patient. Radical surgery is the most effective treatment.

Download Full-text

Magnetic Resonance Imaging in Primary Sclerosing Cholangitis

Wiedza Medyczna ◽

10.36553/wm.92 ◽

2021 ◽

Vol 3 (2) ◽

pp. 20-24

Author(s):

Aldona Wybraniec-Zaręba ◽

Julia Tuchalska-Czuroń ◽

Gabriela Półtorak-Szymczak ◽

Mariusz Furmanek ◽

Jerzy Walecki ◽

...

Keyword(s):

Bile Duct ◽

Magnetic Resonance ◽

Primary Sclerosing Cholangitis ◽

Sclerosing Cholangitis ◽

Bile Ducts ◽

Extrahepatic Bile Duct ◽

Magnetic Resonance Cholangiopancreatography ◽

Left Liver Lobe ◽

Bile Duct Stenosis ◽

Mri Features

Primary sclerosing cholangitis (PSC) is a chronic liver disease in which there are inflammation and scarring of the bile ducts leading to fibrosis, destruction and narrowing of the bile ducts, resulting in cholestasis. In the long run, PSC can cause liver cirrhosis and failure. In clinical practice, the diagnosis of PSC is generally based on blood tests and imaging studies (currently preferably magnetic resonance cholangiopancreatography). To make a diagnosis of PSC it is necessary to exclude secondary causes of sclerosing cholangitis. The most common MRI features of PSC concerning bile ducts are: bile duct dilatation, beading, extrahepatic bile duct stenosis, wall enhancement and thickening. The most common MRI features of PSC concerning hepatic parenchyma are: rounded shape of the liver caused by hypertrophy of caudate lobe and left liver lobe, atrophy of the right lobe, enlargement of portal and/or portacaval lymph nodes, peripheral parenchymal inflammation, wedge-shaped confluent fibrosis, heterogeneity of the liver parenchyma, periportal oedema, cirrhosis with indirect signs of portal hypertension such as splenomegaly, ascites and collateral vasculature.

Download Full-text

Giant Primary Neuroendocrine Neoplasms of the Liver: Report of 2 Cases With Molecular Characterization

International Journal of Surgical Pathology ◽

10.1177/1066896919855764 ◽

2019 ◽

Vol 27 (8) ◽

pp. 893-899

Author(s):

Laura G. Pastrián ◽

Ignacio Ruz-Caracuel ◽

Raul S. Gonzalez

Keyword(s):

World Health Organization ◽

Neuroendocrine Tumor ◽

Large Cell ◽

The Other ◽

World Health ◽

Neuroendocrine Neoplasms ◽

Molecular Testing ◽

Well Differentiated ◽

Grade 3 ◽

Health Organization

Primary neuroendocrine neoplasms of the liver have occasionally been reported in the liver, though many reports do not convincingly exclude metastases. In this article, we report 2 “giant” hepatic neuroendocrine lesions without evidence of a primary elsewhere after clinical workup. One occurred in a 21-year-old male; the lesion was a large cell neuroendocrine carcinoma measuring 24 cm. The patient died of disease in 10 months. The other occurred in a 25-year-old patient, was 18 cm wide, and was diagnosed as a well-differentiated neuroendocrine tumor, World Health Organization grade 3. The patient died of disease after 30 months. Molecular testing demonstrated only the presence of TP53 mutations in common. These cases expand our knowledge of seemingly primary neuroendocrine neoplasms of the liver, in particular, giant cases measuring more than 8 cm. Guidelines for clinical workup and therapy for these lesions remain unclear, but future thorough workup of such cases is necessary for specific characterization.

Download Full-text