Xanthogranulomatous Appendicitis: A Rare Pathological Finding Associated With Delayed Appendectomy.

Xanthogranulomatous inflammation of the appendix is a rare pathological finding associated with appendicitis and chronic inflammation. Its clinical significance is not fully understood, and diagnosis is primarily based on the histopathological review as imaging findings with CT and ultrasound are non-specific. Here, we present a case of a 64-year-old female with recurrent appendicitis who underwent an appendectomy with final pathological findings consistent with xanthogranulomatous appendicitis (XGA). We discuss the higher reported incidence of XGA in interval appendectomy specimens compared to emergency appendectomies, and how this relates to its proposed pathophysiology. We found that XGA is associated with a more challenging operative field and the need to convert from a laparoscopic to an open procedure, increasing the potential risks of surgical complications. The potential development of XGA should be considered when planning an interval appendectomy as it may impact operative planning, although there is no clear consensus on its clinical significance.

Xanthogranulomatous osteomyelitis of pubic bone mimicking neoplasm: a case report and literature review.

BACKGROUND: Xanthogranulomatous osteomyelitis (XO) is a rare disease characterized radiologically by an osteolytic lesion with cortical expansion or disruption. Differentiating this condition from other osteolytic diseases such as primary or metastatic bone neoplasms is imperative. Several case reports have been published on XO, with previous reports predominantly identifying bacteria such as Pseudomonas or Staphylococcus as causative organisms. However, fungal infection-induced XO has not yet been reported. CASE PRESENTATION: We present the case of a 23-year-old woman with a tumor-like osteolytic lesion in the pubic bone. The patient had experienced pelvic pain and intermittent febrile episodes for 2 months. Plain radiography revealed an osteolytic lesion in the right pubic tubercle. Magnetic resonance imaging suggested a cystic bone tumor or tubercular infection. Surgical intervention included curettage of the lesion and irrigation with normal saline. Histopathological examination of the specimen revealed abundant foamy histiocytes with inflammatory infiltrates consistent with XO. Culture of the osteolytic lesion confirmed an Aspergillus species infection and antifungal treatment was initiated. At 1-year follow-up, no evidence of local recurrence was observed. CONCLUSIONS: Although rare, XO requires differentiation from similar conditions and is treated with surgical intervention and targeted medical therapy based on the identified organisms. Clinicians should be mindful that XO can also be induced by fungal infections and that combination antifungal treatments may be beneficial in such cases.

Stage III xanthogranulomatous pyelonephritis with sarcomatoid degeneration.

Xanthogranulomatous pyelonephritis (XGP) is an uncommon chronic condition characterized by destructive granulomatous disease of the kidney with uncertain etiology. Significant risk factors for XGP are represented by the coexistence of history of nephrolithiasis, diabetes mellitus, recurrent urinary tract infections and other immunocompromised conditions. It is also associated with higher risk of malignancy, reported in up to 11% of patients. We report a case of a 76-year-old female who presented to the emergency department with an insidious onset of abdominal and right lower back pain. She had a history of renal stones and diabetes mellitus. On physical examination, a painful fistulous orifice in skin on the right lumbar region was found. CT images showed a nonfunctioning right kidney replaced by multiple necrotic cavities with inflammatory involvement of the right hepatic lobe and a nephron-cutaneous fistula. These CT findings were strongly suggestive of XGP (III state). CT images obtained before and after the administration of intravenous contrast material showed also a hyper-vascularized renal mass with irregularly thickened walls confirmed by a targeted CEUS examination and suspicious for malignancy. Pathologic examination confirmed the chronic pyelonephritis and revealed evidence of a concomitant sarcomatoid lesion. This case underlines the central role of a multimodality imaging approach in the emergency department and how this affects the correct management and treatment of patients. In fact, MDCT is considered the current gold standard for the diagnosis and the staging of XPG but the contrast-enhanced ultrasound (CEUS) in selected patients can increase the diagnostic accuracy in the uncertain small renal masses detected on CT scans.

Xanthogranulomatous Pyelonephritis and Plummer-Vinson Syndrome: A Case Report Exploring Potential Connections in a Single Patient.

Xanthogranulomatous pyelonephritis (XGP) and Plummer-Vinson Syndrome (PVS) are two rare disorders that pose considerable diagnostic difficulties mainly because their signs overlap and are multifaceted. XGP is a severe form of pyelonephritis imitating cancer, whereas PVS is defined by dysphagia, iron deficiency anemia, and esophageal webs. This article presents the case of a 53-year-old female with a previous history of renal calculi and multiple transfusions who presented with dysphagia, flank pain, and hematuria. Findings from investigations showed severe anemia, a renal lesion suggesting malignancy, and GI findings pointing to the presence of PVS. The coexistence of XGP and PVS in this patient highlights the need for careful differential diagnosis and the importance of a multidisciplinary approach in managing patients with rare overlapping syndromes. Furthermore, this example shows how chronic infection, malnutrition, and their potential for leading to neoplasms connect. In summary, one should recognize that the possibility these two conditions can coincide is paramount for accurate diagnosis and effective management. Lastly, this case demonstrates that comprehensive assessment can only be achieved through coordinated care addressing both direct effects as well as secondary complications due to such uncommon diseases.

Case report: Xanthogranulomatous pyelonephritis successfully treated with antibiotics.

Xanthogranulomatous inflammation (XGI) is an uncommon benign inflammatory disease process characterized by aggregation of lipid laden foamy macrophages, among other inflammatory cells. XGI affecting the kidney and renal pelvis is otherwise known as xanthogranulomatous pyelonephritis (XGP), which has been reported to be present in approximately 5% of all reported xanthogranulomatous cases. The clinical relevance of this disease is that it often mimics malignancy, and preoperative imaging is unable to differentiate between the two. Our case report demonstrated a renal lesion that mimicked malignancy and ultimately required histopathological examination for the diagnosis of XGP. Current consensus remains that surgery is the mainstay form of treatment. However, this case report showed that use of antibiotics alone can be successful as the patient showed significant improvement and resolution on interval imaging.

[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.

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.

Xanthogranulomatous Pyelonephritis: A pooled quantitative analysis of published cases.

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.

[Xanthogranulomatous pyelonephritis : bear paw sign]. / L'image du mois. Pyélonéphrite xanthogranulomateuse : signe de la patte d'ours.

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.

Fatal nephrobronchial fistula arising from xanthogranulomatous pyelonephritis: a case report.

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.

Xanthogranulomatous pyelonephritis in a patient with polycystic kidney disease without underlying risk factors: a case report.

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.

Xanthogranulomatous Pyelonephritis in a Young Adult Without Any Pre-existing Conditions.

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.

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.

Xanthogranulomatous epithelial tumors/keratin-positive giant cell-rich tumors involving the head and neck: report of seven cases and review of the literature.

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).

Xanthogranulomatous Pyelonephritis in Peritoneal Dialysis.

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.

An Interesting Finding of Xanthogranulomatous Pyelonephritis Revealed Concomitant Incidental Squamous Cell Carcinoma (SCC) of the Renal Pelvis.

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).

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.

Xanthogranulomatous pancreatitis: A rare entity in the spectrum of pancreatic lesions, a case report.

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.