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Introduction: Distinguishing benign from malignant biliary strictures remains challenging despite diagnostic advancements. Follicular cholangitis, a rare benign condition, presents with symptoms and imaging similar to malignancies like cholangiocarcinoma, often complicating diagnosis, particularly when tumor markers are elevated and imaging suggests metastasis. Case presentation: A 57-year-old woman with hypertension and diabetes was admitted with jaundice. Elevated bilirubin and liver enzymes alongside high carbohydrate antigen 19-9 (CA19-9) levels but normal carcinoembryonic antigen (CEA) were noted. Imaging showed thickening from the hilar duct to the proximal common bile duct, accompanied by suspected lymph node metastases. Comprehensive ERCP-guided biopsies found no malignancy. Surgical resection led to a diagnosis of follicular cholangitis. Conclusions: Follicular cholangitis' long-term prognosis is elusive due to its rarity, and preoperative diagnosis is challenging. Increased awareness may improve diagnostic and treatment approaches, as this case adds to the disease's understanding. [ABSTRACT FROM AUTHOR]

Follicular cholangitis (FC) is a rare non-neoplastic biliary tract disease first reported in 2003. A 74-year-old woman underwent extended left hepatectomy with a diagnosis of intrahepatic cholangiocarcinoma. Histopathological examination of the surgical specimen demonstrated no malignant findings, and lymphocytic infiltration with lymphoid follicles was observed within the bile duct wall. Along with immunohistochemical findings, the patient was diagnosed with FC. More than 3 years after surgery, the patient exhibited elevated hepatobiliary enzymes and total bilirubin. Endoscopic retrograde cholangiography revealed stricture and dilation from the extrahepatic bile duct to the right intrahepatic bile duct. Histopathological findings uncovered lymphocytic infiltration without malignant results. It was concluded that bile duct stricture due to FC had newly developed in her remnant liver. Subsequently, the patient developed hypoalbuminemia, and abdominal computed tomography revealed atrophy of the remnant liver and ascites accumulation. Esophagogastroduodenoscopy exposed the development of esophageal varices, which were not observed preoperatively. The patient was diagnosed with decompensated liver cirrhosis accompanied by portal hypertension. This case strongly suggests that long-term follow-up after surgery may be required for patients with FC for screening of potential new bile duct stricture and progression to liver cirrhosis due to cholestasis. [ABSTRACT FROM AUTHOR]

Introduction: Distinguishing benign from malignant biliary strictures remains challenging despite diagnostic advancements. Follicular cholangitis, a rare benign condition, presents with symptoms and imaging similar to malignancies like cholangiocarcinoma, often complicating diagnosis, particularly when tumor markers are elevated and imaging suggests metastasis. Case presentation: A 57-year-old woman with hypertension and diabetes was admitted with jaundice. Elevated bilirubin and liver enzymes alongside high carbohydrate antigen 19-9 (CA19-9) levels but normal carcinoembryonic antigen (CEA) were noted. Imaging showed thickening from the hilar duct to the proximal common bile duct, accompanied by suspected lymph node metastases. Comprehensive ERCP-guided biopsies found no malignancy. Surgical resection led to a diagnosis of follicular cholangitis. Conclusions: Follicular cholangitis’ long-term prognosis is elusive due to its rarity, and preoperative diagnosis is challenging. Increased awareness may improve diagnostic and treatment approaches, as this case adds to the disease’s understanding.

Abstract Background Follicular cholangitis (FC) is a benign bile duct disease that was first reported 2003. Pathologically, it is characterized by lymphoplasmacytic infiltration with multiple lymphoid follicle formations under the mucosal layer of the biliary tract. However, as this disease is extremely rare, little is known about its etiology and pathogenesis. Case presentation A 77-year-old woman was diagnosed with middle bile duct stenosis and potential increases in alkaline phosphatase (ALP) and γ-glutamyl transpeptidase levels (γ-GTP). Carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9) and IgG4 levels were all within the normal limits. Contrast-enhanced computed tomography (CE-CT) and magnetic resonance imaging (MRI) revealed bile duct dilation from intrahepatic to upper common bile duct and an irregular mass lesion in distal bile duct. Additionally, multiple overlapping leaf-like folds were detected. 18F-fluorodeoxyglucose positron emission tomography–computed tomography (18F-FDG-PET/CT) did not demonstrate fluorodeoxyglucose uptake. Subtotal stomach-preserving pancreaticoduodenectomy with regional lymph node dissection was performed because common bile duct cancer could not be ruled out. The resected specimen showed diffuse homogeneous middle bile duct wall thickening. Microscopically, the lesion exhibited thick fibrosis with several invaded lymphoplasmacytic cells, and lymphoid follicle formations were detected under the mucosal layer. Immunohistochemical staining (IHC) revealed positive for CD3, CD4, CD20 and CD79a, and these findings led to a final diagnosis of FC. The patient has not experienced recurrence to date (42 months postoperatively). Conclusions Currently, accurate preoperative diagnosis of FC is difficult. More cases must be accumulated to generate additional knowledge on its precise diagnosis and proper treatment.

Introduction: The differential diagnosis of focal biliary strictures comprises both malignant and benign conditions. We report a rare case of follicular cholangitis presenting with segmental stricture of the left hepatic duct. Case description: An asymptomatic 59-year-old male with no past medical history presented with dilation of the left intrahepatic bile ducts revealed as an incidental finding on an abdominal ultrasound. Blood examinations showed only a slightly elevated gamma- glutamyl transferase (gammaGT) value, while carbohydrate antigen 19-9 (Ca 19-9) and serum immunoglobulin G4 (IgG4) were within normal range. Abdominal computed tomography and magnetic resonance imaging/magnetic resonance cholangiopancreatography (MRI/MRCP) scans revealed a high grade focal intrahepatic stricture of the left hepatic duct (FIHS type III) with proximal dilatation. Given that a diagnosis of cholangiocarcinoma could not be ruled out, the patient was referred for a left hepatectomy with regional lymph node dissection. Histological analysis showed a lymphoplasmacytic infiltration of the left hepatic duct with fibrosis and follicle formations in the submucosa, findings consistent with follicular cholangitis. The postoperative course was uneventful and there is no evidence of recurrence 8 months after the surgery. Discussion: The clinical and imaging presentation of follicular cholangitis is very similar to cholangiocarcinoma, rendering it a challenging diagnosis preoperatively. Conclusion: The approach to these cases should be primarily surgical. Even though it is very rare -- our report is the 13th case reported worldwide -- follicular cholangitis should be included in the differential diagnosis of focal biliary strictures.

Background: Follicular cholangitis (FC) is a benign bile duct disease that was first reported 2003. Pathologically, it is characterized by lymphoplasmacytic infiltration with multiple lymphoid follicle formations under the mucosal layer of the biliary tract. However, as this disease is extremely rare, little is known about its etiology and pathogenesis. Case presentation: A 77-year-old woman was diagnosed with middle bile duct stenosis and potential increases in alkaline phosphatase (ALP) and γ-glutamyl transpeptidase levels (γ-GTP). Carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9) and IgG4 levels were all within the normal limits. Contrast-enhanced computed tomography (CE-CT) and magnetic resonance imaging (MRI) revealed bile duct dilation from intrahepatic to upper common bile duct and an irregular mass lesion in distal bile duct. Additionally, multiple overlapping leaf-like folds were detected. 18F-fluorodeoxyglucose positron emission tomography–computed tomography (18F-FDG-PET/CT) did not demonstrate fluorodeoxyglucose uptake. Subtotal stomach-preserving pancreaticoduodenectomy with regional lymph node dissection was performed because common bile duct cancer could not be ruled out. The resected specimen showed diffuse homogeneous middle bile duct wall thickening. Microscopically, the lesion exhibited thick fibrosis with several invaded lymphoplasmacytic cells, and lymphoid follicle formations were detected under the mucosal layer. Immunohistochemical staining (IHC) revealed positive for CD3, CD4, CD20 and CD79a, and these findings led to a final diagnosis of FC. The patient has not experienced recurrence to date (42 months postoperatively). Conclusions: Currently, accurate preoperative diagnosis of FC is difficult. More cases must be accumulated to generate additional knowledge on its precise diagnosis and proper treatment. [ABSTRACT FROM AUTHOR]

Follicular cholangitis is a new, rare disease that causes severe biliary stricture. We herein describe the findings from a resected case of follicular cholangitis, suggesting a distinct disease entity that causes benign biliary stricture. A 60‐year‐old man who was referred to our hospital due to elevated γ‐glutamyl transpeptidase levels and dilatation of the B8 bile duct. Although bile juice cytology and bile duct brushing cytology showed no malignancy, the dilatation was progressive. Therefore, right hepatectomy combined with caudate lobectomy was carried out on suspicion of intrahepatic cholangiocarcinoma. The wall of the resected bile duct was markedly thickened due to severe fibrosis under the mucosal layer. Histology of the mucosal epithelium indicated no malignancy. Infiltration of plasma cells characterized by remarkable formation of lymphoid follicles with germinal centers was observed around the bile ducts. The patient was diagnosed with follicular cholangitis based on histological findings. We thus observed a rare case of follicular cholangitis. This case and review of published reports suggest that, despite its rarity, follicular cholangitis should be considered at the differential diagnosis of biliary stricture. This case report could contribute to a better understanding of how to address this disease. [ABSTRACT FROM AUTHOR]

Biliary epithelial cells preferentially respond to various insults under chronic pathological conditions leading to reactively atypical changes, hyperplasia, or the development of biliary neoplasms (such as biliary intraepithelial neoplasia, intraductal papillary neoplasm of the bile duct, and cholangiocarcinoma). Moreover, benign biliary strictures can be caused by a variety of disorders (such as IgG4-related sclerosing cholangitis, eosinophilic cholangitis, and follicular cholangitis) and often mimic malignancies, despite their benign nature. In addition, primary sclerosing cholangitis is a well-characterized precursor lesion of cholangiocarcinoma and many other chronic inflammatory disorders increase the risk of malignancies. Because of these factors and the changes in biliary epithelial cells, biliary strictures frequently pose a diagnostic challenge. Although the ability to differentiate neoplastic from non-neoplastic biliary strictures has markedly progressed with the advance in radiological modalities, brush cytology and bile duct biopsy examination remains effective. However, no single modality is adequate to diagnose benign biliary strictures because of the low sensitivity. Therefore, understanding the underlying causes by compiling the entire clinical, laboratory, and imaging data; considering the under-recognized causes; and collaborating between experts in various fields including cytopathologists with multiple approaches is necessary to achieve an accurate diagnosis. [ABSTRACT FROM AUTHOR]

Follicular cholangitis may lead to severe focal biliary stricture, which is difficult to distinguish from cholangiocarcinoma. This is the report of a rare case of follicular cholangitis associated with an intrahepatic focal biliary stricture in a 69-year-old woman. The patient visited our hospital for upper abdominal pain in 2006. Computed tomography revealed common bile duct stones and focal stricture of the left intrahepatic duct. Endoscopic retrograde cholangiopancreatography revealed a severe B3 stricture and dilation of the peripheral biliary tract. Repeated cytology revealed no malignancy. The patient was followed up for 8 years and repeated attacks of cholangitis severely compromised her quality of life. A left hepatectomy was performed, and the pathological findings were consistent with follicular cholangitis. It is generally difficult to make a definitive diagnosis after identifying a biliary stricture, and the stricture is often treated with surgical resection. The clinical presentation and radiological findings of follicular cholangitis are similar to those of cholangiocarcinoma; therefore, if a conservative approach is selected, frequent follow-up is recommended. The present case suggests that follicular cholangitis should be considered in the differential diagnosis of biliary stricture. [ABSTRACT FROM AUTHOR]

Follicular cholangitis is a sclerosing cholangitis with hilar biliary stricture that must be differentiated from both immunoglobulin G4-related sclerosing cholangitis and primary sclerosing cholangitis. This disorder is extremely rare and difficult to distinguish from hilar biliary cholangiocarcinoma. We report here a case of a Japanese female patient in her 60s with this disease. The patient visited a family doctor for itching and general fatigue. Blood examination showed elevated hepatobiliary enzyme levels. Various imaging studies showed dilation of the bilateral intrahepatic bile duct and wide stenosis from the proximal bile duct to the right and left hepatic duct. They also showed the enlargement of multiple lymph nodes in the hepatoduodenal ligament, periaorta, and mesocolon. Based on endoscopic retrograde cholangiopancreatography-directed brush cytology, we diagnosed this patient with hilar cholangiocarcinoma and performed left trisegmentectomy of the liver. The pathology results showed that the wall from the bilateral hepatic duct to the proximal bile duct had thickened irregularly with dense fibrosis and a marked formation of lymph follicles. The mucosal epithelia did not have malignant findings. The diagnosis was follicular cholangitis. This case indicates that follicular cholangitis should be considered as a differential diagnosis of hilar biliary stricture. [ABSTRACT FROM AUTHOR]

Abstract: IgG4-related disease involving the liver and biliary tree exhibits variable morphological changes. The most common manifestation is sclerosing cholangitis characterized by pipe stem-like fibrosis of the wall and extensive inflammation. IgG4-related sclerosing cholangitis sometimes exhibits pseudo-tumourous exuberance around the hilar bile duct (inflammatory pseudotumour), radiological features of which are reminiscent of hilar cholangiocarcinoma. Another minor manifestation is IgG4-related chronic active hepatitis. Consistent with other manifestations of IgG4-related disease, pathological features include diffuse lymphoplasmacytic infiltration, irregular fibrosis typically in a storiform pattern, and moderate tissue eosinophilia. On immunostaining, not only the absolute number of IgG4-positive plasma cells but also the IgG4/IgG-positive ratio is dramatically increased. Pathologists need to be aware of this condition, as the diagnosis requires a multi-disciplinary approach, in which pathological examination has a critical role. [Copyright &y& Elsevier]

We experienced a case of follicular cholangitis that was positive on fluorodeoxyglucose-positron emission tomography ( 18 F-FDG-PET). A 70-year-old man was admitted for jaundice. Endoscopic retrograde cholangiography showed stenosis of the middle to upper choledocus. 18 F-FDG-PET depicted a localized hot spot at the stenotic lesion (maximum standardized uptake value = 8.2). Although no malignant findings were found in the cytology or on a bile duct biopsy, malignancy could not be excluded, so surgical treatment was performed. Follicular cholangitis is a new, rare disease that causes severe biliary stricture. Only 11 cases of follicular cholangitis have been reported, including the present case.