Navigating diagnostic challenges in xanthogranulomatous cholecystitis: A case report.

Xanthogranulomatous cholecystitis (XGC) presents a diagnostic challenge due to its rarity and varied clinical manifestations and nonspecific radiological findings. We here describe a 67-year-old man with right hypochondriac pain, where imaging revealed irregular thickening of the gallbladder wall, prompting consideration of various differential diagnoses including gallbladder malignancy, adenomyomatosis, and complicated cholecystitis. With inconclusive lab results, cholecystectomy with potential extended hepatectomy was advised. Intraoperatively, an inflamed gallbladder was observed. Histopathological examination confirmed XGC, stressing histological verification. Complete cholecystectomy is standard, with partial resection an option. Our case details the complexity in diagnosis and management of XGC.

[18F]FDG PET/CT in Benign and Malignant Diseases Involving the Biliary Tract.

Role of FDG PET/CT in evaluation of biliary tract diseases remains relatively unexplored. PET/CT with FDG helps in evaluation of both infective / inflammatory as well as neoplastic diseases as increased glucose utilization is observed in both the conditions. In this article, we describe the spectrum of FDG PET/CT findings in various diseases affecting the biliary tract. Role of FDG PET/CT in neoplastic diseases involving the biliary duct has been described at the time of staging and response evaluation; in characterization of the intrahepatic mass (abscess v/s cholangiocarcinoma). In addition, we have discussed about the false positive FDG uptake along the biliary duct stent, which interfere with scan interpretation. Few of the benign conditions described are Langerhans cell histiocytosis and IgG4 related disease involving the biliary duct and adenomyomatosis and Xanthogranulomatous cholecystitis involving the gall bladder.

Gallbladder cancer masquerading as xanthogranulomatous cholecystitis: a case report and literature review.

Xanthogranulomatous cholecystitis (XGC) is a rare type of cholecystitis that, despite being benign poses diagnostic challenges due to its low prevalence and need for consensus on diagnostic criteria. Consequently, distinguishing XGC from gallbladder cancer (GBC) is challenging, leading to clinical misdiagnoses. This article presents a case where a patient initially diagnosed with GBC was later found to have XGC.

Dual-energy CT in differentiating benign gallbladder wall thickening from wall thickening type of gallbladder cancer.

OBJECTIVE: To evaluate the performance of dual-energy computed tomography (DECT) in differentiating non-acute benign from malignant gallbladder wall thickening (GBWT). METHODS: This prospective study comprised consecutive adults with GBWT who underwent late arterial phase (LAP) and portal venous phase (PVP) DECT between January 2022 and May 2023. The final diagnosis was based on histopathology or 3-6 months follow-up imaging. DECT images in LAP and PVP were assessed independently by two radiologists. The demographic, qualitative, and quantitative parameters were compared between two groups Multivariate logistic regression was performed to determine the association between the aforementioned factors and malignant GBWT. RESULTS: Seventy-five patients (mean age 56 ± 12.8 years, 46 females) were included. Forty-two patients had benign, and 33 had malignant GBWT. In the overall group, female gender (p = 0.018), lymphadenopathy (p = 0.011), and omental nodules (p = 0.044) were significantly associated with malignant GBWT. None of the DECT features differed significantly between benign and malignant GBWT in overall group. In the xanthogranulomatous cholecystitis (XGC, n = 9) vs. gallbladder cancer (GBC) (n = 33) subgroup, mean attenuation value at 140 keV LAP VMI was significantly associated with malignant GBWT [p = 0.023, area under curve 0.759 (95%CI 0.599-0.919)]. CONCLUSION: DECT-generated quantitative parameters do not add value in differentiating non-acute benign from malignant GBWT. However, DECT may have a role in differentiating XGC from GBC in a selected subgroup of patients. Further, larger studies may be necessary to confirm these findings. CLINICAL RELEVANCE STATEMENT: In patients with non-acute gallbladder wall thickening in whom there is suspicion of xanthogranulomatous cholecystitis (XGC), DECT findings may allow differentiation of XGC from wall thickening type of gallbladder cancer. KEY POINTS: Differentiation of benign and malignant gallbladder wall thickening (GBWT) at CT is challenging. Quantitative dual energy CT (DECT) features do not provide additional value in differentiating benign and malignant GBWT. DECT may be helpful in a subgroup of patients to differentiate xanthogranulomatous cholecystitis from gallbladder cancer.

Xanthogranulomatous cholecystitis: Diagnostic dilemma and surgical solution in geriatric patients: A case report.

INTRODUCTION: Xanthogranulomatous Cholecystitis (XGC) is a rare inflammatory condition characterized by the presence of xanthogranulomas within the gallbladder wall, often mimicking gallbladder carcinoma (GBC). Diagnosis is challenging and may require biopsy. Once GBC is excluded, an open cholecystectomy is recommended, although laparoscopic cholecystectomy is increasingly being performed with great caution. This case report aims to evaluate clinical and radiological features, surgical outcomes, and treatment approaches for XGC. CASE PRESENTATION: A 70-year-old patient presented with right hypochondrial pain and a palpable gallbladder. A CT scan revealed a distended lithiasic gallbladder with a thickened irregular wall and hepatic nodules. A hepatic MRI suggested xanthogranulomatous cholecystitis. A CT-guided biopsy of the liver nodule showed no signs of malignancy. An open cholecystectomy with a trans-cystic drain was performed. Histological examination confirmed chronic xanthogranulomatous cholecystitis. The patient was discharged on postoperative day 10. A clinical and radiological follow-up at 6 months postoperatively showed no abnormalities. CLINICAL DISCUSSION: XGC presents diagnostic challenges due to its resemblance to GBC. Imaging aids in diagnosis, but biopsy may be necessary. Open cholecystectomy is the recommended surgical treatment due to excessive local inflammation and the risk of concomitant malignancy. CONCLUSION: Managing XGC demands a holistic approach that integrates all clinical insights and mandates close collaboration among a multidisciplinary team of surgeons, radiologists, and pathologists. Further research is needed to refine diagnostic and therapeutic strategies for this rare condition, especially in geriatric patients.

Machine learning-based diagnostic model for preoperative differentiation between xanthogranulomatous cholecystitis and gallbladder carcinoma: a multicenter retrospective cohort study.

Background: Xanthogranulomatous cholecystitis (XGC) and gallbladder carcinoma (GBC) share similar imaging and serological profiles, posing significant challenges in accurate preoperative diagnosis. This study aimed to identify reliable indicators and develop a predictive model to differentiate between XGC and GBC. Methods: This retrospective study involved 436 patients from Zhejiang Provincial People's Hospital and The Affiliated Lihuili Hospital of Ningbo University. Comprehensive preoperative imaging, including ultrasound, Computed Tomography (CT), Magnetic Resonance Imaging (MRI), and blood tests, were analyzed. Machine learning (Random Forest method) was employed for variable selection, and a multivariate logistic regression analysis was used to construct a nomogram for predicting GBC. Statistical analyses were performed using SPSS and RStudio software. Results: The study identified gender, Murphy's sign, absolute neutrophil count, glutamyl transpeptidase level, carcinoembryonic antigen level, and comprehensive imaging diagnosis as potential risk factors for GBC. A nomogram incorporating these factors demonstrated high predictive accuracy for GBC, outperforming individual or combined traditional diagnostic methods. External validation of the nomogram showed consistent results. Conclusion: The study successfully developed a predictive nomogram for distinguishing GBC from XGC with high accuracy. This model, integrating multiple clinical and imaging indicators, offers a valuable tool for clinicians in making informed diagnostic decisions. The findings advocate for the use of comprehensive preoperative evaluations combined with advanced analytical tools to improve diagnostic accuracy in complex medical conditions.

Imaging and Clinical Findings of Xanthogranulomatous Inflammatory Disease of Various Abdominal and Pelvic Organs: A Pictorial Essay.

Xanthogranulomatous (XG) inflammatory disease is a rare benign disease involving various organs, including the gallbladder, bile duct, pancreas, spleen, stomach, small bowel, colon, appendix, kidney, adrenal gland, urachus, urinary bladder, retroperitoneum, and female genital organs. The imaging features of XG inflammatory disease are nonspecific, usually presenting as a heterogeneous solid or cystic mass. The disease may also extend to adjacent structures. Due to its aggressive nature, it is occasionally misdiagnosed as a malignant neoplasm. Herein, we review the radiological features and clinical manifestations of XG inflammatory diseases in various organs of the abdomen and pelvis.

Usefulness of MRI Scoring System for Differential Diagnosis between Xanthogranulomatous Cholecystitis and Wall-Thickening Type Gallbladder Cancer.

Purpose: To define an MRI scoring system for differentiating xanthogranulomatous cholecystitis (XGC) from wall-thickening type gallbladder cancer (GBC) and compare the diagnostic performance of the scoring system with the visual assessment of radiologists. Materials and Methods: We retrospectively analyzed 23 and 35 patients who underwent abdominal MRI and were pathologically diagnosed with XGC and wall-thickening-type GBC after surgery, respectively. Three radiologists reviewed all MRI findings. We defined a scoring system using these MRI findings for differentiating XGC from wall-thickening type GBC and compared the area under the curve (AUC) of the scoring system with the visual assessment of radiologists. Results: Nine MRI findings showed significant differences in differentiating the two diseases: diffuse gallbladder wall thickening (p < 0.001), mucosal uniformity (p = 0.002), intramural T2-high signal intensity (p < 0.001), mucosal retraction (p = 0.016), gallbladder stones (p < 0.001), T1-intermediate to high-signal intensity (p = 0.033), diffusion restriction (p = 0.005), enhancement pattern (p < 0.001), and phase of peak enhancement (p = 0.008). The MRI scoring system showed excellent diagnostic performance with an AUC of 0.972, which was significantly higher than the visual assessment of the reviewers. Conclusion: The MRI scoring system showed better diagnostic performance than the visual assessment of radiologists to differentiate XGC from wall-thickening-type GBC.

Distinguishing characteristics of xanthogranulomatous cholecystitis and gallbladder adenocarcinoma: a persistent diagnostic dilemma.

BACKGROUND: Xanthogranulomatous cholecystitis (XGC) is an uncommon variant of chronic cholecystitis which can resemble gallbladder adenocarcinoma (GAC) on preoperative imaging and present technical challenges in the performance of cholecystectomy. We examined our experience with each pathology to identify distinguishing characteristics that may guide patient counseling and surgical management. METHODS: A retrospective review of all pathologically confirmed cases of XGC and GAC following cholecystectomy between 2015 and 2021 at a single institution was performed. Clinical, biochemical, radiographic, and intraoperative features were compared. RESULTS: There were 37 cases of XGC and 20 cases of GAC. Patients with GAC were older (mean 70.3 years vs 58.0, p = 0.01) and exclusively female (100% vs 45.9%, p < 0.0001). There were no significant differences in accompanying symptoms between groups (nausea/vomiting, fevers, or jaundice). The mean maximum white blood cell count was elevated for XGC compared to GAC (16.4 vs 8.6 respectively, p = 0.044); however, there were no differences in the remainder of the biochemical profile, including bilirubin, liver transaminases, CEA, and CA 19-9. The presence of an intraluminal mass (61.1% vs 9.1%, p = 0.0001) and lymphadenopathy (18.8%. vs 0.0%, p = 0.045) were associated with malignancy, whereas gallbladder wall thickening as reported on imaging (87.9% vs 38.9%, p = 0.0008) and gallstones (76.5% vs. 50.0%, p = 0.053) were more often present with XGC. Cases of XGC more often had significant adhesions/inflammation (83.8% vs 55.0%, p = 0.03). CONCLUSION: Clinical features that may favor benign chronic cholecystitis over gallbladder adenocarcinoma include younger age, male gender, current or prior leukocytosis, and the absence of an intraluminal mass or lymphadenopathy. Laparoscopic cholecystectomy is a safe surgical option for equivocal presentations. Intraoperative frozen section or intentional staging of more extensive procedures based upon final histopathology are valuable surgical strategies.

Successful Treatment of Xanthogranulomatous Cholecystitis Following Total Knee Replacement.

Xanthogranulomatous cholecystitis (XGC) is a rare form of chronic gallbladder inflammation that most commonly presents as acute cholecystitis and is often mistaken for carcinoma of the gallbladder. This case details the hospital course and follow-up of a 77-year-old male who developed suspected acute acalculous cholecystitis (AAC) resulting in severe sepsis after elective left total knee arthroplasty (TKA). Histopathological findings after elective cholecystectomy later revealed XGC as the underlying etiology.

Xanthogranulomatous cholecystitis in a patient with ulcerative colitis and primary sclerosing cholangitis: A case report.

Chronic gallbladder disease due to xanthogranulomatous cholecystitis is uncommon, and its symptoms are generally vague. While there is no firm evidence to link xanthogranulomatous cholecystitis to primary sclerosing cholangitis or ulcerative colitis. The patient is a 41-year-old male with a history of ulcerative colitis, primary sclerosing cholangitis, and biliary stenting who complained of symptoms of anorexia, jaundice, and pruritus. In the initial ultrasound exam, there was evidence of intrahepatic and extra-hepatic bile duct dilation along with a significant and mass-like circumferential thickening of the gallbladder wall. Magnetic resonance cholangiopancreatography was performed for further evaluation, which indicated increased gallbladder wall thickness, containing multiple T2 hyper-signal nodules while the mucosal layer was intact. There was also a filling defect in the common bile duct's distal portion. These findings matched a xanthogranulomatous cholecystitis diagnosis and a possibly malignant lesion in the distal of the common bile duct. The patient ultimately had a cholecystectomy, and pathology findings confirmed the diagnosis of xanthogranulomatous cholecystitis. Biopsy specimens obtained from the distal of the common bile duct lesion were microscopically identified as intramucosal adenocarcinoma. In patients with a history of primary sclerosing cholangitis who present with nonspecific symptoms suggesting chronic gallbladder disease and radiologic evidence of circumferential gallbladder wall thickening containing intramural nodules and intact mucosa, xanthogranulomatous cholecystitis should be kept in mind.

Xanthogranulomatous cholecystitis with histologic features suggestive of IgG4 related cholecystitis - A morphologic overlap with IgG4 related disease.

AIMS: Both xanthogranulomatous cholecystitis (XGC) and IgG4-related cholecystitis (IgG4-CC) are rare chronic fibroinflammatory tumefactive diseases of the gallbladder, which cause a strong confusion with resectable malignancy in view of their mass forming tendency with extension into the liver. We aim to study the histopathologic features of xanthogranulomatous cholecystitis with regard to IgG4-related cholecystitis in extended cholecystectomy specimens. METHODS AND RESULTS: Sixty cases of extended cholecystectomy with liver wedge resection, diagnosed as XGC on histopathology from January 2018 to December 2021 were retrieved from the archives. Representative sections were reviewed by two pathologists independently. Immunohistochemistry was performed for IgG4 and IgG4/IgG was derived. The cases were dichotomized in two groups on the basis of IgG4 positive plasma cells. Six cases with >50 IgG4 positive plasma cells had storiform fibrosis, IgG4/IgG ratio >0.40 and extra-cholecystic extension. Of these, 50 % had obliterative phlebitis and 66.7 % had perineural plasma cell wrapping. CONCLUSIONS: A small subset of XGC cases (~10 %) had morphologic overlap with IgG4-CC, but should not be overcalled as the diagnosis of IgG4-RD requires an integrative approach based on clinical, serologic and imaging criteria and not solely on histopathology.

Xanthogranulomatous inflammation involving the gallbladder, bile duct, and pancreas: a case report.

Xanthogranulomatous inflammation (XGI) is a rare, benign condition that can affect several organs, including the gallbladder, kidney, skin, gastrointestinal tract, lymph nodes, and soft tissues. It is often misdiagnosed as a malignancy. In this report, we present the case of a 79-year-old male who presented with persistent jaundice for 11 months. Computed tomography and magnetic resonance imaging revealed pancreatic head enlargement, gallbladder thickening, and common bile duct thickening, leading to a preoperative diagnosis of malignant neoplasm of the pancreatic head. During surgery, dense adhesions were found around the portal vein, suggestive of mass invasion. To relieve obstruction, choledochojejunostomy was performed. Postoperative pathological examination revealed xanthogranulomatous cholecystitis (XGCc), xanthogranulomatous cholangitis (XGCg), and xanthogranulomatous pancreatitis (XGP). XGI affecting the bile ducts and pancreas is extremely rare, and there are no reported cases of simultaneous involvement of the gallbladder, bile duct, and pancreas by XGI. This study provides valuable insight into the differential diagnosis of XGI by presenting the imaging features of XGI patients.

Xanthogranulomatous cholecystitis mimicking gallbladder carcinoma: a case report.

Xanthogranulomatous cholecystitis (XGC) is an uncommon type of chronic cholecystitis. Clinical presentation, laboratory findings, and radiological analysis mimic gallbladder carcinoma. A definitive diagnosis is made by histological study. Cholecystectomy, along with adjuncts as required, is performed for management. Case Presentation: We present a case of a 67-year-old female who was planned for interval cholecystectomy for gallstone pancreatitis. Her clinical, laboratory and radiological findings were suggestive of cholelithiasis and was planned for laparoscopic cholecystectomy. Her intraoperative findings mimicked gallbladder carcinoma. The surgery was aborted, and a biopsy was sent for histopathological analysis. XGC was diagnosed, and the patient underwent laparoscopic cholecystectomy with no postoperative complications during the 6-month follow-up period. Discussion: XGC is a rare disorder resulting from chronic inflammation of the gallbladder. There is the presence of xanthogranuloma with predominant lipid-laden macrophages in the gallbladder wall along with fibrosis. Clinical presentation, laboratory findings, and radiological analysis mimic gallbladder carcinoma. Ultrasonography usually shows diffuse wall thickening of the gallbladder, intramural hypoechoic nodules, unclear liver and gallbladder interface, and the presence of gallstones. The final diagnosis is made by histopathological analysis. Laparoscopic or open cholecystectomy, along with adjuncts as required, is performed for management with a low postoperative complication rate. Conclusion: XGC is a rare, benign disease that is often confused with gallbladder cancer before histological analysis. XGC can be managed with laparoscopic cholecystectomy with minimal postoperative complications.

Xanthogranulomatous Cholecystitis Mimicking Gall Bladder Cancer: a Diagnostic Dilemma and Review of Literature.

Xanthogranulomatous cholecystitis (XGC) is one of the rare variants of chronic cholecystitis which is characterized by inflammation of gall bladder along with infiltration by acute and chronic inflammatory cells. Intramural accumulation of lipid laden macrophages in GB wall is the hallmark of the disease. XGC results in dense adhesion of gall bladder (GB) to surrounding structures, like duodenum, colon, and stomach. The intense GB inflammation results in gall bladder perforation and development of fistulous communication between gall bladder and surrounding structures. This may also lead to formation of inflammatory mass which closely mimic gall bladder malignancy. Often differentiation from carcinoma of GB (Ca GB) on the basis of clinical presentation and even on intra-operative and radiological findings is difficult, and the issue could only be resolved on final Histopathology (HPE). We review presentation and investigation of a patient, discuss our approach in managing dilemma in treating such cases of XGC, and review the literature.

Spontaneous cholecystocutaneous fistula secondary to xanthogranulomatous cholecystitis: a case report.

BACKGROUND: Xanthogranulomatous cholecystitis, a rare variant of cholecystitis, may infrequently be complicated by spontaneous cholecystocutaneous fistula. CASE PRESENTATION: We report the case of a 75-year-old Saudi Arabian man who presented with "a painful area of redness" (cellulitis) over his right upper abdomen. Abdominal computed tomography revealed multiple collections, which were drained surgically. A discharging sinus was identified, and a fistulogram revealed cholecystocutaneous fistula during his follow-up visit. The patient underwent laparoscopic management and recovered uneventfully. Final histopathological evaluation confirmed acute-on-chronic xanthogranulomatous cholecystitis . CONCLUSIONS: Although rare, surgeons should consider cholecystocutaneous fistula in the differential diagnosis of anterior abdominal wall abscesses, particularly in patients with concurrent or background symptoms of gallbladder disease. We report the first case of laparoscopic management for cholecystocutaneous fistula in Saudi Arabia.

Differential diagnoses of gallbladder tumors using CT-based deep learning.

Background: The differential diagnosis between gallbladder cancer (GBC) and xanthogranulomatous cholecystitis (XGC) remains quite challenging, and can possibly lead to improper surgery. This study aimed to distinguish between XGC and GBC by combining computed tomography (CT) images and deep learning (DL) to maximize the therapeutic success of surgery. Methods: We collected a dataset, including preoperative CT images, from 28 cases of GBC and 21 XGC patients undergoing surgery at our facility. It was subdivided into training and validation (n = 40), and test (n = 9) datasets. We built a CT patch-based discriminating model using a residual convolutional neural network and employed 5-fold cross-validation. The discriminating performance of the model was analyzed in the test dataset. Results: Of the 40 patients in the training dataset, GBC and XGC were observed in 21 (52.5%), and 19 (47.5%) patients, respectively. A total of 61 126 patches were extracted from the 40 patients. In the validation dataset, the average sensitivity, specificity, and accuracy were 98.8%, 98.0%, and 98.5%, respectively. Furthermore, the area under the receiver operating characteristic curve (AUC) was 0.9985. In the test dataset, which included 11 738 patches, the discriminating accuracy for GBC patients after neoadjuvant chemotherapy (NAC) (n = 3) was insufficient (61.8%). However, the discriminating model demonstrated high accuracy (98.2%) and AUC (0.9893) for cases other than those receiving NAC. Conclusion: Our CT-based DL model exhibited high discriminating performance in patients with GBC and XGC. Our study proposes a novel concept for selecting the appropriate procedure and avoiding unnecessary invasive measures.

The incidental finding of xanthogranulomatous cholecystitis: a report of 10 cases.

There is insufficient clinical knowledge about xanthogranulomatous cholecystitis (XGC) due to biased reporting. This study aims to investigate the incidence of XGC and evaluate the clinical outcome regarding operative time, rate of conversion and intraoperative or postoperative complications. We included 1141 patients who underwent surgery for gallbladder disease between January 2019 and December 2021. Of 1141 patients who underwent cholecystectomy, XGC was seen in 10 (0.87%). The average age is 47 (24-81 years old) with a male to female ratio of 3:2. Biliary pancreatitis and obstructive jaundice are seen in two patients who did ERCP and stenting before surgery. One patient presented with a 4 cm necrotizing soft-tissue granuloma. The BMI was high, with class I obesity in five patients. Symptoms last from 1 to 12 weeks. One patient was only diagnosed preoperatively as XGC. Four out of 10 (40%) required more than 72 h of hospitalization. All patients underwent elective sitting surgery, with eight successfully managed by laparoscopy and one converted to open. The average operative time was 90 min (43-193 min), and a postoperative drain was inserted in four patients. The median follow-up is after 24 months (11-30 months), with no postoperative collection, bleeding, complication or readmission. XGC is a rare benign entity requiring no further action upon incidental discovery. Surgical resection is the cornerstone of management, with the laparoscopic approach considered feasible and safe. Four out of 10 patients might need more than 3 days of hospitalization. In the presence of mass, the frozen section can help guide the management.

Machine Learning-Based Radiological Features and Diagnostic Predictive Model of Xanthogranulomatous Cholecystitis.

Background: Xanthogranulomatous cholecystitis (XGC) is a rare benign chronic inflammatory disease of the gallbladder that is sometimes indistinguishable from gallbladder cancer (GBC), thereby affecting the decision of the choice of treatment. Thus, this study aimed to analyse the radiological characteristics of XGC and GBC to establish a diagnostic prediction model for differential diagnosis and clinical decision-making. Methods: We investigated radiological characteristics confirmed by the RandomForest and Logistic regression to establish computed tomography (CT), magnetic resonance imaging (MRI), CT/MRI models and diagnostic prediction model, and performed receiver operating characteristic curve (ROC) analysis to prove the effectiveness of the diagnostic prediction model. Results: Based on the optimal features confirmed by the RandomForest method, the mean area under the curve (AUC) of the ROC of the CT and MRI models was 0.817 (mean accuracy = 0.837) and 0.839 (mean accuracy = 0.842), respectively, whereas the CT/MRI model had a considerable predictive performance with the mean AUC of 0.897 (mean accuracy = 0.906). The diagnostic prediction model established for the convenience of clinical application was similar to the CT/MRI model with the mean AUC and accuracy of 0.888 and 0.898, respectively, indicating a preferable diagnostic efficiency in distinguishing XGC from GBC. Conclusions: The diagnostic prediction model showed good diagnostic accuracy for the preoperative discrimination of XGC and GBC, which might aid in clinical decision-making.

Xanthogranulomatous cholangitis mimicking cholangiocarcinoma: Case report and review of literature.

INTRODUCTION: Xanthogranulomatous cholangitis is an extremely rare diagnosis and is believed to be an extension of xanthogranulomatous cholecystitis, a benign inflammatory process characterized by lipid-laden foamy macrophages (called "xanthoma cells") occurring in a background of chronic inflammation consisting of lymphocytes, plasma cells, and eosinophils. Here, we report a case of xanthogranulomatous cholangitis mimicking cholangiocarcinoma. CASE PRESENTATION: A 72 year old male with history of recurrent cholangitis had preoperative workup highly suggestive of intrahepatic cholangiocarcinoma. He underwent right hepatectomy and portal lymphadenectomy, with pathology showing xanthogranulomatous cholangitis, with no evidence of malignancy. Interestingly, the patient did not have xanthogranulomatous cholecystitis. DISCUSSION: We reviewed the current literature on xanthogranulomatous cholangitis, and identified only 14 previously reported cases. In our case series, there were six female and eight male patients. Among the 14 patients, 11 presented to the hospital with jaundice. Twelve patients had preoperative workup concerning for malignancy. The diagnosis of xanthogranulomatous cholangitis was confirmed through pathology in 13 patients, and through endoscopic ultrasound biopsy in one patient. In our review, seven patients had associated xanthogranulomatous cholecystitis, three patients had an isolated case of xanthogranulomatous cholangitis, and four patients had unknown status. Our patient is the fourth case of isolated xanthogranulomatous cholangitis without xanthogranulomatous cholecystitis. CONCLUSION: Xanthogranulomatous cholangitis is a very rare phenomenon that can lead to benign strictures of the bile ducts, especially in the setting of recurrent cholangitis. It can mimic malignancies, such as cholangiocarcinoma, and should be considered in the differential diagnosis.