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Xanthogranulomatous Pyelonephritis (XGP) is a serious and rare inflammatory disease of unknown etiology. This systematic review analyzes XGP cases. We performed a literature search for "Pyelonephritis, Xanthogranulomatous." The primary composite outcome was recovery with post-surgery complications, partial recovery, death, or chronic kidney disease. The secondary outcome was any presentation or treatment complication. Predictor variables consisted of demographics, history, symptoms, and diagnosis/management. Among the 251 patients, the mean age was 36.1 years, and 57.4% were female. The most common symptom and finding were fever (55.0%) and renal stones (53.8%), respectively. There were 15.5% with the composite outcome. There were 51.0% with any presentation or treatment complication. Multivariate logistic regression analysis for the composite outcome showed that kidney of both/horseshoe (OR:3.86, 95% CI:1.01, 14.73, p = 0.048), dialysis required (OR:8.64, 95% CI:2.27, 32.94, p = 0.002), and operative treatment of nephrostomy or nephrostomy followed by nephrectomy (OR:4.57, 95% CI:1.58, 13.17, p = 0.01) were each significantly associated with increased odds. Fever (OR:3.04, 95% CI:1.63, 5.67, p <0.001) and renal stones (OR:2.55, 95% CI:1.35, 4.81, p = 0.004) were each significantly associated with increased odds for any presentation/treatment complication. In conclusion, XGP patients with involvement of both or horseshoe kidneys, dialysis requirements, or treatment of nephrostomy or nephrostomy followed by nephrectomy may require aggressive treatment to mitigate poor patient outcomes.

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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.

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We present the pathognomonic image of the «bear paw sign¼ of xanthogranulomatous pyelonephritis, a rare form of chronic pyelonephritis typically occurring in association with obstructive uropathy (especially with staghorn calculi) and recurrent urinary tract infections.

Nous présentons l'image pathognomonique du «signe de la patte d'ours¼ de la pyélonéphrite xanthogranulomateuse, forme rare de pyélonéphrite chronique survenant classiquement sur un terrain associant une uropathie obstructive (en particulier sur lithiase coralliforme) et des infections urinaires à répétitions.

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The timely diagnosis of xanthogranulomatous pyelonephritis (XGP), a rare and chronic kidney condition, along with its appropriate management, is a must to spare the kidney from end-stage renal disease (ESRD). The main hurdle in early diagnosis of most medical conditions, including XGP, is the absence of specific and characteristic symptoms, which, if present, would make the patient seek medical aid earlier and tempt the clinician to think of variable differential diagnosis. We hereby report a case of a 20-year-old male patient who had no specific symptoms suggestive of a renal pathology, which delayed him from considering consulting a healthcare professional, thereby making his condition diagnosed as XGP at a time when his involved kidney was hardly salvageable. Through this case report, we wish to humbly request clinicians all across the globe to kindly broaden their range of differential diagnoses while dealing with patients with nonspecific symptomatology, in order to have a better prognosis.

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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.

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Introduction: Nephrobronchial fistula is an exceptionally rare complication of renal infections, including the uncommon xanthogranulomatous pyelonephritis. Existing literature is limited to a few case reports, with antibiotic therapy and nephrectomy being the preferred treatments. Case: We present the case of a 63-year-old woman with a history of recurrent xanthogranulomatous pyelonephritis in her right kidney, requiring drainage through lumbotomy. She presented with a chronic dry cough and weight loss, without other noticeable symptoms. Imaging suggested a pulmonary abscess and nephrobronchial fistula. Despite antibiotic treatment and surgical intervention, her condition progressed fatally. Conclusion: Nephrobronchial fistulas are extremely complications of renal infections, often presenting with nonspecific symptoms. This case highlights their significant impact on morbidity and mortality, especially in resource-limited settings, and underscoring the urgent need for prompt diagnosis and treatment.

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Xanthogranulomatous epithelial tumor (XGET) and HMGA2::NCOR2 fusion keratin-positive giant cell-rich tumor (KPGCT) are recently described morphologically overlapping rare neoplastic entities characterized by HMGA2::NCOR2 fusions, low-grade biological behavior, and a strong predilection for young females. To date, 47 cases have been reported with only four occurring in head and neck anatomic locations. In this study, we describe the clinicopathologic, immunohistochemical, and molecular findings of seven XGET/KPGCTs occurring in the head and neck region. The patients were six females and one male, aged 3.5-59 years old (median, 25 years). The tumors involved the ear, vocal cord, skull, neck soft tissue, and sinonasal cavity. Tumor sizes ranged from 1.5 to 6.7 cm. Histologically, the tumors were characterized by xanthogranulomatous histiocytes, osteoclast-like giant cells, and keratin-positive epithelioid cells. The XGET/KPGCTs involving the ear was remarkable for more cytologic atypia than previously described. Four cases had the HMGA2::NCOR2 fusion identified by NGS and three had HMGA2 gene locus alterations by FISH. Follow-up information was available for 3 of 7 patients (range 6-46 months). The patient with a vocal cord XGET/KPGCTs developed a local recurrence treated with excision. This study illustrates that XGET/KPGCTs involves the head and neck region as well, where it may be unexpected and hence under-recognized, and expands the anatomic locations of involvement to include unreported sites (ear, vocal cord, and sinonasal tract).

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Xanthogranulomatous pyelonephritis (XGP) is an extremely rare, chronic granulomatous inflammatory condition thought to arise secondary to a combination of obstruction, recurrent bacterial infection and an incomplete immune response although the etiology of XGP is more complex. We would like to report a case of XGP occurring in a patient with polycystic kidney disease (PCKD), which has not been previously documented in etiology. A 29-year-old woman presented to our hospital with right upper quadrant pain for 5 days. She had experienced a low-grade fever, generalized weakness, and myalgia throughout her body for 2 weeks. She had no history of renal stones or recurrent UTIs. Contrast-enhanced CT revealed a well-enhancing large septated cystic mass in the right kidney and numerous cysts in the liver and both kidneys. Open right radical nephrectomy was performed due to the suspicion of renal cell carcinoma, as there was no response to antibiotics over 7 days. Gross specimen demonstrated architectural distortion due to xanthomatous nodules and a dilated pelvico-calyceal system filled with pus and blood. Microscopic examination revealed infiltration of neutrophils and lipid-laden macrophages. The patient is currently being followed up in the outpatient clinic without recurrence of XGP. This is the first reported case of XGP in a patient with underlying PCKD. Physicians should consider PCKD as a potential underlying cause of XGP.

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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.

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Introduction: Xanthogranulomatous pyelonephritis (XGP) is a rare illness that consists of a destructive chronic inflammatory process of the renal parenchyma associated with recurrent infection and obstructions of the urinary tract. Peritoneal dialysis (PD) is a form of renal replacement therapy used in advanced kidney disease. PD patients demonstrate a systemic inflammatory state, secondary to the increase in uremic toxins, decreased filtration of proinflammatory cytokines, as well as constant exposure to bioincompatible dialysis solutions or a foreign body reaction from the catheter, among other factors, as peritoneal infections. Case Presentation: We present the clinical case of a 74-year-old woman, with a history of recurrent urinary tract infections associated with nephrolithiasis and stage 5D chronic kidney disease, on a PD program. The patient presented a non-specific 3-month state of progressive asthenia, with increased inflammatory parameters in the analytical controls. After presenting multiple negative urine cultures and peritoneal fluid cultures, she was hospitalized to study the constitutional syndrome. The imaging test revealed bilateral staghorn lithiasis with severe dilatation of the right renal pelvis and great cortical thinning. Given the suspicion of XGP, it was decided to perform right renal nephrectomy, which was confirmed after the anatomopathological study. Prior to the intervention, she was transferred to hemodialysis. Over the following months, significant clinical and analytical improvement was observed. Conclusion: The systemic inflammatory state and the risk of infections in PD can mask the diagnosis of XGP in PD patients. There are no reported cases of XGP in patients in PD.

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Squamous cell carcinoma, originating in the renal pelvis, is an infrequent form of kidney malignancy. The occurrence rate remains below 1% for all neoplasms in this specific area. Most of these carcinomas are moderately or poorly differentiated, and diagnosis typically occurs at an advanced stage. Xanthogranulomatous pyelonephritis is an uncommon form of severe chronic infection that affects the parenchyma of native kidneys. We present the case of a 34-year-old male with a history of end-stage renal disease secondary to recurrent pyelonephritis, which was incidentally diagnosed as renal squamous cell carcinoma (SCC).

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Purpose: We report a patient who initially visited the ophthalmology clinic for a vision loss diagnosed with Erdheim-Chester Disease (ECD). Observations: ECD is a rare non-Langerhans cell histiocytosis characterized by multisystemic organ involvement and poor prognosis. Our patient had complete vision loss due to prominent orbital involvement before any systemic symptoms appeared. This case demonstrates variable clinical manifestations of ECD. Conclusions and importance: Painless bilateral proptosis with poor response to steroid treatment should prompt consideration for ECD and systemic evaluation. In addition, in the absence of typical clinical manifestations, a thorough evaluation of the biopsy can be crucial for an accurate diagnosis.

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Xanthogranulomatous pleuritis is an extremely rare pathological entity, characterized by the infiltration of foamy cells and multinucleated giant cells within the pleural space. This condition often mimics infectious and neoplastic processes, presenting significant diagnostic challenges. This report details the first documented case of xanthogranulomatous pleuritis induced by recurrent biliothorax due to a biliopleural fistula, presenting a unique clinical scenario. We describe the clinical presentation, diagnostic hurdles, and both the surgical and medical management of this case. The discovery of biliothorax, evidenced by pleural fluid bilirubin levels that exceed serum bilirubin levels, underscores the importance of considering biliothorax in the differential diagnosis of recurrent pleural effusions, particularly in patients with a history of trauma. This case emphasizes the need for heightened awareness and a multidisciplinary approach in the diagnosis and treatment to effectively manage this complex condition and prevent recurrence.

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INTRODUCTION: Xanthogranulomatous appendicitis (XGA) is a rare condition involving chronic inflammation of the appendix that is often difficult to distinguish from malignancy using imaging because of the formation of a heterogeneous mass with indistinct borders. Herein, we present a case of XGA with unusual clinical manifestations. PRESENTATION OF CASE: A 78-year-old female patient underwent radical resection of hilar cholangiocarcinoma with extended right hepatic lobectomy and biliary reconstruction. Three years postoperatively, she presented with an irregular mass in the right lateral pelvis, which was observed on computed tomography. The patient had not experienced recent clinical symptoms and did not present with abdominal tenderness. Routine blood tests did not indicate an increased inflammatory response; however, carcinoembryonic antigen levels continued to increase. Although disseminated recurrence of hilar cholangiocarcinoma and appendiceal carcinoma were suspected, XGA was diagnosed via laparoscopic appendectomy. DISCUSSION: XGA generally presents with symptoms of acute or chronic appendicitis, and is diagnosed incidentally during surgery. Hilar cholangiocarcinoma has a high recurrence rate, even after radical resection, and disseminated recurrence usually requires chemotherapy. In the present case, XGA was not suspected preoperatively because of the lack of physical symptoms and increased levels of tumor markers during follow-up for hilar cholangiocarcinoma. There have been no reports of XGA with such a confusing clinical course, thus confirming the difficulty in preoperatively diagnosing XGA. CONCLUSION: The preoperative diagnosis of XGA is difficult to differentiate from malignancy because of its clinical and imaging findings. We diagnosed the patient with XGA using laparoscopic surgery.

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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.

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INTRODUCTION: Xanthogranulomatous pancreatitis (XGP) is a rare, benign, and idiopathic disease that often presents with non-specific symptoms and can mimic or coexist with other pancreatic diseases. Despite its infrequency, XGP is frequently misdiagnosed as a pancreatic neoplasm, with only 15 reported cases in the literature. The pathogenesis of XGP remains unclear. CASE REPORT: We present the case of a 34-year-old woman with no pathological history who experienced continuous abdominal pain and oral intolerance, without signs of cholestasis. An abdominal CT scan initially suggested a cystic neoplasm of the pancreas, leading to a laparotomic cephalic duodenopancreatectomy. The anatomopathological study and immunohistochemistry revealed XGP in association with a mucinous cystic neoplasm with mild to moderate atypia. The patient remained hospitalized for six days post-surgery without any complications. DISCUSSION: XGP may be induced by the inflammatory reaction secondary to the obstruction of the pancreatic duct by mucin. The etiology is unknown, but it is attributed to a combination of obstruction, hemorrhage, or ductal infection. Abdominal pain is the most common symptom. Differentiating XGP from malignant processes of the pancreatic gland is challenging. Surgical treatment typically involves the Whipple procedure; however, echoendoscopy with biopsy is now available for a more accurate and early differential diagnosis. CONCLUSION: XGP is a rare and challenging differential diagnosis for pancreatic neoplasms. Due to its potential to mimic malignant lesions, a high index of suspicion is necessary. Echoendoscopy with fine-needle aspiration biopsy should be considered a routine diagnostic tool before major surgery, such as the Whipple procedure.

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Renal vein thrombosis (RVT) is a relatively uncommon condition that is most frequently observed in individuals with nephrotic syndrome. While rare, pyelonephritis (PN) may serve as a predisposing factor for secondary RVT. In such cases, one should consider the possibility of RVT when patients fail to respond to appropriate antibiotic treatment. Typically, these patients require additional anticoagulation therapy for a duration of 3 to 6 months, with a generally favorable prognosis. In this report, we present the case of a 74-year-old female who developed RVT due to Klebsiella pneumoniae PN. Additionally, we reviewed 11 cases of PN complicated by RVT, which were documented in the PubMed database over a span of 40 years, emphasizing key elements in diagnostic and therapeutic approaches. Lastly, we elaborated upon the role of thrombo-inflammation, especially in the context of sepsis.

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Xanthogranulomatous osteomyelitis (XO) is a rare chronic inflammatory bone disease characterized by the presence of cholesterol-laden foam macrophages, histiocytes, and plasma cells. We report the case of a 41-year-old man with end-stage renal disease who had undergone deceased donor kidney transplantation 4 years earlier. He presented with a chest wall mass that he had first identified 2 weeks prior to admission. Computed tomography revealed a periosseous heterogeneously enhancing soft tissue mass adjacent to the sternal end of the left clavicle, accompanied by irregular and destructive osteolytic lesions on the left side of the sternal manubrium. A total mass resection, which included partial clavicle and sternum removal, was performed. Pathological examination revealed foamy histiocytes along with numerous lymphoplasmacytic cells, confirming the diagnosis of XO. This case underscores the potential for XO to develop following kidney transplantation.