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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.

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.

Lipid-rich foamy macrophages intermingled with lymphocytes, plasma cells, neutrophils, and eosinophils are the hallmark of xanthogranulomatous inflammation. Due to its capacity for mass formation and destructiveness, it is an uncommon entity that mimics neoplasm. Rarely, it is detected in the female genital system; however, it is most frequently seen in the kidney and gallbladder. It is associated with endometriosis, uterine leiomyoma, ovarian hemangioma, and can occur secondary to diverticulitis, chronic bacterial infections, ineffective antibiotic treatment, and uterine artery embolization. The annual incidence of pheochromocytoma is 0.8 per 100,000 person-years and it is a catecholamine-secreting tumor that arises from chromaffin cells in the adrenal medulla. Synchronous presentation of xanthogranulomatous salpingo-oophoritis (XSO) with pheochromocytoma has not been described so far. Hence, we report a series of two such cases mimicking malignancy and have discussed the pathogenesis of the above association. [ABSTRACT FROM AUTHOR]

We aim to present an adult case of a rare cause of scrotal swelling i.e., xantho-granulomatous orchitis (XGO). The patient presented to us with a history of swelling and pain in the left scrotum for one month. There were no other associating symptoms and sexual and traumatic history were not present. Incision and drainage were planned for the patient under our care, however, because of ruptured tunica albuginea and dispersion of seminiferous tubules, left orchiectomy was performed and the specimen was sent for histopathological analysis. Xantho-granulomatous inflammation (XGI) is a relatively benign and infrequent condition and usually affects the 3rd-7th decade of life. The lipid-laden macrophages replace the destructed and necrotic normal tissue. The findings that are the hallmark of granulomatous diseases are necrosis and multinucleated giant cells which are not present in cases of XGI. It affects the testis, epididymis, spermatic cord, and bladder among genital organs.

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. [ABSTRACT FROM AUTHOR]

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Purpose: Xanthogranulomatous pyelonephritis (XGP) is a rare form of pyelonephritis more commonly seen in females and diabetics. Frequently associated with renal tract calculi, it is often difficult to diagnose, as it can clinically and radiologically mimic other disorders. Most cases are treated with antibiotics and nephrectomy. The aim of our review is to summarise and analyse the current evidence focusing on management. Methods: A literature search was conducted to identify papers relating to xanthogranulomatous pyelonephritis in adults. Studies containing ten or more patients with XGP were included for descriptive analysis, and a meta-analyses of cohort studies conducted comparing open and minimally invasive nephrectomy undertaken. Other papers were included for narrative review. Results: 52 studies were identified, 20 were included for narrative review and 32 retrospective observational studies containing 868 patients were included for descriptive analysis. 99.8% of patients underwent nephrectomy, about one-third laparoscopically. The most commonly cultured organisms were Escherichia coli and Proteus mirabilis. 60% of patients, where reported, underwent preoperative drainage. Seven studies containing 211 patients were included for meta-analysis which found that postoperative complications, length of stay and transfusion requirements were all significantly reduced in those who underwent minimally invasive surgery. Conclusion: The mainstay management of XGP is antibiotic therapy and nephrectomy. Some studies highlight a role for preoperative upper urinary tract drainage, but evidence supporting this is limited. We present the first meta-analyses examining operative approach for patients undergoing nephrectomy for XGP. Though limited by the data available, our meta-analysis indicates minimally invasive nephrectomy for XGP provides better postoperative outcomes. [ABSTRACT FROM AUTHOR]

We present the pathognomonic image of the «bear paw sign» 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.

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., Competing Interests: I acknowledge on behalf of all the authors that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials discussed in this manuscript., (© 2024 Published by Elsevier Inc.)

Xanthogranulomatous pyelonephritis (XGP) is a rare type of chronic bacterial nephritis, which rarely involves the invasion of adjacent organs or the formation of fistulas due to tissue-destructive granulomatous reactions. Although the invasions of various adjacent organs have been reported in several cases of XGP, MRI data on their features are limited. MRI has a better soft-tissue resolution than CT. Thus, it can identify the extent of extrarenal involvement in advanced XGP, and the findings can be used in treatment planning. Herein, we report a rare case of XGP with nephropleural fistula formation diagnosed using CT and MRI.

Xanthomas or xanthelasmas are rare benign tumours characterised by localised lipid deposits within an organ system which can be an important sign of systemic disease. These tumours have a predilection for skin and subcutaneous tissue, the most common visceral site being the Gastrointestinal (GI) tract. Xanthoma in female genital tract is a rare finding and should be considered as a differential diagnosis for abdominal pain in a reproductive age group. This is an unusual case of xanthoma of fallopian tube in a 27-year-old female presenting with lower abdominal pain. Ultrasonography (USG) revealed right hydrosalpinx. The histopathological examination of fallopian tube showed sheets of foamy macrophages with peripherally placed nucleus and abundant vacuolated cytoplasm in the lamina propria which was positive for Cluster of Differentiation 68 (CD68) and negative for Cytokeratins (CK). A final diagnosis of xanthoma of right fallopian tube was made. Fallopian tube xanthomas must be distinguished from xanthogranulomatous salpingitis, which is associated with an inflammatory cell infiltrate, often including giant cells and plasma cells.

Recurrent bladder perforation due to xanthogranulomatous cystitis with malakoplakia is rare entity and can lead to spontaneous bladder perforation. A 15 years girl presented with sudden pain abdomen with reduced urine output. Her exploratory laparotomy revealed, perforation of 2 cm at the dome of bladder with unhealthy margins. Excisional bladder biopsy and repair of bladder perforation by 3-0 polyglactin suture was done. The histopathology showed xanthogranulomatous cystitis with malakoplakia. Her records revealed the same histopathology in bladder perforation at age of 9 with lost follow-up till age of 15. Exploratory laparotomy and bladder repair should be done to save the patient.

Xanthogranulomatous inflammation is a rare inflammatory reaction, characterized by lipid-laden macrophages, known as xanthomas, in histopathologic examination. Aggressive xanthogranulomatous inflammation often manifests as local infiltration but does not affect distant organs unless combined with rare systemic diseases. We report a case of focal xanthogranulomatous pyelonephritis (XGP) associated with severe xanthogranulomatous cholecystitis. Focal XGP was suspected in radiologic examination that showed a cystic lesion with an infiltrative margin, which were surgically resected and confirmed in pathologic examination. To our knowledge, this is the first report of focal xanthogranulomatous pyelonephritis associated with xanthogranulomatous cholecystitis. Moreover, we found peripheral hypointensity around the cystic lesion in the T2-weighted image, probably reflecting hemorrhage and fibrosis of the xanthogranulomatous inflammation.

Granulomatous prostatitis is a rare condition that is diagnosed only by histopathological examination. Though rare, the condition was reported to have different presentations (mimicking prostate cancer or prostatitis and prostatic abscess) and to have different etiologies which classified it into three main entities; nonspecific (idiopathic), post-surgery, and specific. Specific granulomatous prostatitis is further sub-classified to infective, xanthogranulomatous, Malacoplakia and associated with systemic granulomatous disease and allergy. We hereby report a rare case of xanthogranulomatous prostatitis that presented with persistent urinary tract infection.

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., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Varghese et al.)

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., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 The Authors. Published by Elsevier Inc.)

Abstract Xanthogranulomatous endometritis is a rare benign pathology mimicking endometrial carcinoma.

Xanthogranulomatous endometritis is a rare benign pathology mimicking endometrial carcinoma. [ABSTRACT FROM AUTHOR]

Abstract Xanthogranulomatous prostatitis as mimicker of prostate adenocarcinoma can cause a diagnostic dilemma, as presented in this case. Therefore, alongside histopathology analysis, multiparametric magnetic resonance imaging (mpMRI) would be useful in this situation by identifying and characterizing suspicious prostatic lesions before biopsy thereby supporting current recommendations on the use of mpMRI.

Xanthogranulomatous pyelonephritis (XGP) is a rare variant of chronic pyelonephritis. It is characterized by progressive parenchymal destruction caused by chronic renal obstruction due to calculus, stricture, or rarely tumor, resulting in kidney function loss. Herein, we describe the case of a 36-year-old female who presented with left loin pain, left lower limb pain, and dysuria. On contrast-enhanced computed tomography (CECT), multiple abscesses and an obstructive staghorn calculus were depicted in the left kidney with the classical appearance of “Bear Paw Sign.” An abscess with calculi was also present within the left psoas muscle. Though psoas muscle abscess in association with XGP was described, a ureteric fistula and calculi within the psoas muscle have not yet been reported in the literature. Left nephrostomy was performed, which came out to be positive for E. coli on culture. The patient underwent left nephrectomy, and the histopathological report of the surgical specimen confirmed XGP.

We report a case of cooccurrence of xanthogranulomatous and emphysematous pyelonephritis along with necrotizing fasciitis (NF) of scrotum and premium in a patient was nondiabetic and the history of kidney stones. This case a unique presentation of NF due to two pyelonephritis causing fournier gangrene. Surgical management and antibiotics provide an effective management of the disease.

OBJECTIVE: In this study, we aimed to compare development of complications, malignancy and confusion rates in the preliminary diagnosis in patients with xanthogranulomatous cholecystitis identified. METHODS: In this study, 2803 patients undergone cholecystectomy between January 2010 and December 2016 were retrospectively evaluated. Patients with xanthogranulomatous cholecystitis identified in the histopathological examination were classified as Group 1 and patients with cholelithiasis, cholecystitis, and malignancy detected were classified as Group 2. RESULTS: Forty-five patients with xanthogranulomatous cholecystitis were classified as group 1 and 2758 patients as group 2. of group 1, 18 were male and group 2 consisted of 2758 patients with 707 (26%) being male (p=0.04). In the ultrasonographic examination, the wall thickness was increased in 40 patients in Group 1 and 662 patients in Group 2 (p<0.0001). The operation was converted to the open type in 24 patients in Group 1 and 61 patients in Group 2 (p<0.0001). Five patients in Group 1 and 32 patients in Group 2 developed complications in the postoperative period (p<0.0001). CONCLUSION: Xanthogranulomatous cholecystitis should be considered for the differential diagnosis and the operation should be performed, especially by carefully exposing the anatomy in these patients. [ABSTRACT FROM AUTHOR]

Abstract Xanthogranulomatous pyelonephritis (XPN) is an unusual and severe form of chronic inflammatory lesion of the kidney, characterised by the destruction of the renal parenchyma and the presence of multinucleated giant cells and lipid-laden macrophages, inflammatory infiltration and intensive renal fibrosis. There are a few cases in the literature which describe the disease in children. The pathomechanism of XPN is poorly understood. Renal obstruction with concomitant urinary tract infection is the most commonly associated pathological finding. The process is typically unilateral and may be focal or diffuse. In both cases, the perirenal infiltration is possible and can be mistaken for common renal neoplasm or inflammatory process. The symptoms are non-specific. Diagnostic imaging techniques with clinical suspicion have enabled XPN to be diagnosed and differentiated from malignancy with a high degree of confidence. Computed tomography (CT) is the mainstay of diagnostic imaging. The definitive diagnosis of XPN is based on pathological assessment after nephrectomy. We review and illustrate the clinical, radiological, surgical and pathological characteristics of XPN in children. All cases shown are surgically and histopathologically proven. Teaching Points • XPN can present different clinical manifestations. • CT is the mainstay of diagnostic imaging in XPN. • Focal type of XPN should be included in the differential diagnosis of children with a renal mass. • There are no clear guidelines on the management of XPN. • Conservative and surgical treatments should be considered for each individual case. • Histopathological examination confirms the diagnosis and excludes other benign and malign diseases.

The presenting symptoms of RCC (renal cell carcinoma) overlap closely with those of XGP (Xanthogranulomatous pyelonephritis) and can clinically as well as pathologically be confused with XGP which is an unusual inflammatory disorder. Most of the patients present with varied symptoms from the constellation of flank pain, fever, malaise, weight loss, and hematuria. We hereby report here a case of 52 year old male who presented with right flank pain, diagnosed erroneously as XGP on preoperative CT guided biopsy from the renal mass, on two occasions which eventually turned out to be clear cell variant of renal cell carcinoma. Right radical nephrectomy was done where the final histopathological diagnosis was of clear cell variant of RCC.

Hypophysitis is classified into primary and secondary. Xanthomatous hypophysitis is one of the rare types of primary hypophysitis. A 55-year-old female presented with headache, vomiting, and blurring of vision. She also had endocrine dysfunction in the form of low serum T3, T4, and low cortisol levels. MRI scan showed a sellar expansile lesion suggestive of pituitary macroadenoma. Microscopy showed pituitary tissue replaced by inflammatory infiltrate made up of foamy histiocytes arranged in sheets along with lymphoplasmacytic infiltrate. Interspersed areas of fibrosis, hyalinization, few congested and sclerosed blood vessels were seen. Compressed residual pituitary tissue was identified at the periphery. Xanthomatous hypophysitis is a rare entity which can mimic as pituitary adenoma both clinically and radiologically. Accurate diagnosis at an early stage with postsurgical steroid therapy may help to prevent permanent pituitary damage.

Xanthogranulomatous endometritis is an uncommon benign lesion characterized by the destruction of endometrium and replacement by the sheets of foamy histiocytes, lymphocytes, plasma cells, multinucleated giant cells, fibrosis, calcification, and accompanying polymorphonuclear leukocytes. It is commonly mistaken for malignancy clinicoradiologically, and therefore, histopathological examination is of utmost importance. We report the case of a 61-year-old postmenopausal female who presented with pyometra, bulky uterus, and cervical stenosis, and histopathology revealed XGE.

Adult xanthogranulomatous disease of the orbit is a rare and incompletely understood entity. It can be limited to the orbit or be associated with systemic disease. While routine systemic surveillance is the standard of care for patients with orbital xanthogranulomatous disease, there is no universally accepted protocol for managing periorbital and orbital involvement. The authors report a case of adult orbital xanthogranuloma, without systemic disease, who, after 10 years of observation, demonstrated sustained radiographic regression of the lesions. To the authors' knowledge, this represents one of the first reports of spontaneous regression of untreated orbital xanthogranuloma, and supports observation of lesions that do not compromise ocular function. We present our case in the context of a major review of various treatment strategies described in the literature, including surgical resection, radiotherapy, plasmapheresis, corticosteroids, multiple immunomodulatory medications. [ABSTRACT FROM AUTHOR]

Aim: Thick-walled gallbladder is difficult to characterize on conventional imaging. 18F-FDG PET was used to differentiate benign and malignant wall thickness and compared with histopathology. Methods: Thirty patients with gallbladder (GB) wall thickening (focal > 4 mm and diffuse > 7 mm), underwents uspected on ultrasound, or CT scan, and underwent 18F-FDG PET. Histopathology of the specimen was compared with imaging findings. Results: The mean age was 48.22 ± 31.33 years with a M:F 1:4 ratio. Twenty patients had diffuse and 10 had focal thickening. On 18F-FDG PET, lesion was benign in 12, malignant in 13, and indeterminate in 5. Histopathology was malignancy in 12; benign in 18-chronic cholecystitis in 11, xanthogranulomatous in 4, IgG4 related in 2, and polyp in 1. The mean GB wall thickness was 7.79 ± 3.59 mm (10.34 malignant and 6.10 in benign, p = 0.001). At a cutoff of 8.5 mm, the sensitivity and specificity of detecting malignancy was 94% and 67%. The mean SUV uptake was 7.46 (benign 4.51, malignant 14.26, p = 0.0102). At a cutoff of 5.95, the sensitivity and specificity of detecting malignancy was 92% and 79%. For 18F-FDG PET, overall sensitivity was 91%, specificity 79%, PPV 77%, NPV 92%, and diagnostic accuracy was 84%. Conclusion: 18F-FDG PET is a reliable method of differentiation between benign and malignant thickening of the gallbladder particularly when wall thickness and SUV value is taken into account. [ABSTRACT FROM AUTHOR]

Endoscopic ultrasonography (EUS) has greater spatial resolution than other diagnostic imaging modalities. In addition, if gallbladder lesions are found and gallbladder cancer is suspected, EUS is an indispensable modality, enabling detailed tests for invasion depth evaluation using the Doppler mode and ultrasound agents. Furthermore, for gallbladder lesions, EUS fine-needle aspiration (EUS-FNA) can be used to differentiate benign and malignant forms of conditions, such as xanthogranulomatous cholecystitis, and collect evidence before chemotherapy. EUS-FNA is also useful for highly precise and specific diagnoses. However, the prevention of bile leakage, an accidental symptom, is highly important. Advancements in next-generation sequencing (NGS) technologies facilitate the application of multiple parallel sequencing to EUS-FNA samples. Several biomarkers are expected to stratify treatment for gallbladder cancer; however, NGS can unveil potential predictive genomic biomarkers for the treatment response. It is believed that NGS may be feasible with samples obtained using EUS-FNA, further increasing the demand for EUS-FNA.

Background: Xanthogranulomatous prostatitis is a rare benign inflammatory process of the prostate. Clinical, biochemical and imaging similarities make xanthogranulomatous prostatitis a difficult differential diagnosis of prostatic adenocarcinoma. Case presentation: We report the case of a 62 year-old diabetic patient, with 3 months of lower urinary tract symptoms. On digital rectal examination, the prostate was hard and nodular. Initial prostate-specific antigen (PSA) was 43.97 ng/mL, with urine analysis and transabdominal ultrasonography not showing any alterations. A multiparametric magnetic resonance imaging (mpMRI) of the prostate was performed and showed two foci that were classified as PI-RADS 4 lesions. A transrectal ultrasound guided prostate biopsy was then realized. After an initial suspicion of prostatic adenocarcinoma, on clinical, biochemical and radiological grounds, histopathological examination revealed a suppurated xanthogranulomatous prostatitis, with no evidence of malignancy. Conclusions: Xanthogranulomatous prostatitis is an uncommon benign inflammatory condition of the prostate that can clinically, biochemically and even radiologically simulate prostatic adenocarcinoma. We advise that if an older male with low urinary tract symptoms and a hard nodular prostate on digital rectal examination presents, the first diagnosis that one should think of is prostate adenocarcinoma, especially if PSA is high. However, only histopathological examination can differentiate with certitude between these two pathologies and is therefore essential for the diagnosis of xanthogranulomatous prostatitis.

Xanthogranulomatous pyelonephritis (XGPN) is an atypical long-term pyelonephritis with destruction of renal parenchyma and a long-term inflammatory infiltrate of macrophages. Reported presentations of transitional cell carcinoma (TCC) are different. A 73-year-old woman presented with loin pain, prostration, and fever. Computed tomography scan revealed poor cortical enhancement of the kidney, but some of the images bore resemblance to the characteristic “bear's paw” sign, consistent with XGPN with a 7-cm perinephric collection. She was provisionally diagnosed as severe acute pyelonephritis, possibly XGPN, with abscess. In view of the poor clinical condition, decision was made to perform nephrectomy. Histology revealed a G3pT4 high grade TCC with perineural and vascular invasion and reactive xanthogranulomatous inflammatory response. There are few reports of concomitant XGPN and TCC affecting the kidney. However, there has not been any mention of XGPN and TCC presenting as acute pyelonephritis and perinephric abscess so far.

Xanthogranulomatous pyelonephritis may, rarely, occur as a renal cystic mass. We report a case report of a 50-year-old with a history of medically treated renal lithiasis, who consults for left low back pain. Imaging findings concluded to a Bosniak type-3 hemorrhagic cystic mass of the left kidney. The diagnosis of xanthogranumolatous pyelonephritis on its focal form was made histologically. The diagnosis of xanthogranulomatous pyelonephritis is often difficult even with surgical findings and frequently a histological surprise. This points out the importance of identifying it in pre-operative staging; the diagnosis may be suggested by the association of chronic pyelonephritis, renal stones and hypovascular renal tumor syndrome without specificity at sonography and CT. [ABSTRACT FROM AUTHOR]

Xanthogranulomatous inflammation of female genital tract is uncommon and is usually seen in endometrium. Only a few cases involving the ovary have been reported. Its clinical manifestations, imaging modalities, and gross features can mimic ovarian malignancy. Thus, a preoperative diagnosis of this entity is important to avoid radical surgical treatment. We report a case of xanthogranulomatous oophoritis in a 24 years female, which was clinically and radiologically misdiagnosed as ovarian neoplasm. The approach to such a case and the differential diagnosis has also been discussed.

Hypophysitis is an extremely rare inflammatory disease that can mimic the clinical and radiological features of a pituitary adenoma. In this case report, we describe a 45-year-old woman with secondary xanthogranulomatous hypophysitis (XGH) who presented with signs of a pituitary macroadenoma. The patient complained of headaches, visual impairment, and amenorrhea-galactorrhea syndrome. Her physical examination was normal. Laboratory investigation revealed corticotropin, thyrotropin, and gonadotropin deficiencies. She also had low visual acuity in her right eye and an altered visual field. Pituitary magnetic resonance imaging revealed an intra and suprasellar mass measuring 13 × 11 × 16 mm, with hemorrhagic necrosis, that was having a discrete mass effect on the patient's optic chiasm and pituitary stalk. The patient was treated with hydrocortisone and levothyroxine, and then transferred to the Neurosurgery department for total transsphenoidal resection of the mass. Histological examination of the tumor permitted a diagnosis of XGH of a remodeled Rathke's pouch cyst to be made. Systemic conditions such as tuberculosis, sarcoidosis, and other granulomatous diseases were excluded. The etiopathogenesis of XGH remains poorly characterized, but it may be a progressive form of lymphocytic hypophysitis or a remodeled Rathke's pouch cyst. Screening for autoimmune pathology and systemic diseases is essential to guide appropriate management., Competing Interests: Declaration of conflicting interestThe authors declare that there is no conflict of interest.

Xanthogranulomatous pyelonephritis (XGP) is an uncommon and distinct type of chronic infective pyelonephritis causing destruction of the kidney, severely affecting the renal function. The perinephric adipose tissue and peritoneum is not uncommonly involved. The study was undertaken to decipher the clinicopathologic spectrum of XGP. Forty cases of XGP were diagnosed on histopathology over a period of 13 years (2005-2017). Relevant clinical details and radiological findings were recorded from the case files. Out of a total of 40 cases, 26 were female and 14 were male with a mean age of 39.5 ± 13.6 years. Flank pain was the most common presenting symptom. All the patients had unilateral disease and underwent nephrectomy for a nonfunctional kidney. Gross examination showed enlarged kidney with replacement of cortico-medullary tissue by yellow nodular areas of fatty tissue and dilatation of the pelvicalyceal system. Thirty-six (90%) cases had nephrolithiasis. Histologically, the characteristic feature was the existence of lipid-laden foamy macrophages. Renal parenchymal involvement was diffuse in majority (31, 77.5%). Two (5.0%) of the patients had coexisting carcinoma in the same kidney. Histopathologic examination gives the definitive diagnosis of XGP which relies on the characteristic morphology. Surgical intervention in the form of nephrectomy is the treatment of choice and offers good treatment outcomes. [ABSTRACT FROM AUTHOR]

Article: Predictors of Mortality in Korean Patients with Pyogenic Liver Abscess: A Single Center, Retrospective Study (Korean J Gastroenterol 2016;67:238-244)

Xanthogranulomatous prostatitis as mimicker of prostate adenocarcinoma can cause a diagnostic dilemma, as presented in this case. Therefore, alongside histopathology analysis, multiparametric magnetic resonance imaging (mpMRI) would be useful in this situation by identifying and characterizing suspicious prostatic lesions before biopsy thereby supporting current recommendations on the use of mpMRI. [ABSTRACT FROM AUTHOR]

Abstract: Xanthogranulomatous pyelonephritis (XPN) is an unusual and severe form of chronic inflammatory lesion of the kidney, characterised by the destruction of the renal parenchyma and the presence of multinucleated giant cells and lipid-laden macrophages, inflammatory infiltration and intensive renal fibrosis. There are a few cases in the literature which describe the disease in children. The pathomechanism of XPN is poorly understood. Renal obstruction with concomitant urinary tract infection is the most commonly associated pathological finding. The process is typically unilateral and may be focal or diffuse. In both cases, the perirenal infiltration is possible and can be mistaken for common renal neoplasm or inflammatory process. The symptoms are non-specific. Diagnostic imaging techniques with clinical suspicion have enabled XPN to be diagnosed and differentiated from malignancy with a high degree of confidence. Computed tomography (CT) is the mainstay of diagnostic imaging. The definitive diagnosis of XPN is based on pathological assessment after nephrectomy. We review and illustrate the clinical, radiological, surgical and pathological characteristics of XPN in children. All cases shown are surgically and histopathologically proven.Teaching Points: • XPN can present different clinical manifestations.• CT is the mainstay of diagnostic imaging in XPN.• Focal type of XPN should be included in the differential diagnosis of children with a renal mass.• There are no clear guidelines on the management of XPN.• Conservative and surgical treatments should be considered for each individual case.• Histopathological examination confirms the diagnosis and excludes other benign and malign diseases. [ABSTRACT FROM AUTHOR]

Xanthogranulomatous oophoritis is an uncommon, non-neoplastic, chronic process in which the affected organ is destroyed by massive cellular infiltration of foamy histiocytes admixed with multinucleated giant cells, plasma cells, fibroblasts, neutrophils, and foci of necrosis. The etiology of this entity is unknown, but it shares histopathological findings similar to those of xanthogranulomatous change occurring in various organs, including the gallbladder and kidney. The current case was a 20-year-old female presenting with a tubo-ovarian mass with suspicion of malignancy on clinicoradiological findings and final diagnosis of xanthogranulomatous oophritis established on histopathology. Clinically and radiologically, xanthogranulomatous oophritis mimics tumor of the ovary and fallopian tube, thereby making it an important entity. A vigilant histopathological evaluation is important to diagnose the disease. [ABSTRACT FROM AUTHOR]

Aims: Xanthogranulomatous cholecystitis (XGC), an unusual histological variant of chronic cholecystitis, is characterised by mixed foamy histiocytic and lymphoplasmocytic infiltration and fibrosis. Radiologically, the poorly defined nodular growth pattern often leads to the misinterpretation of XGC as gallbladder cancer. In this study, we aimed to identify the relationship of XGC with IgG4‐related cholecystitis. Methods and results: We re‐evaluated 57 surgically resected XGCs and 104 conventional chronic cholecystitis cases, according to the histological features observed in IgG4‐related disease, including lymphoplasmocytic infiltration, storiform fibrosis, obliterative phlebitis, and IgG4‐positive plasma cells. XGCs contained a significantly increased mean number of IgG4‐positive plasma cells [34.8/high‐power field (HPF)] as compared with conventional chronic cholecystitis (4.8/HPF; P < 0.001), and 16 XGCs (28%) harboured >50 IgG4‐positive cells per HPF. Nine XGCs (16%), including one case with IgG4‐related autoimmune pancreatitis, showed ‘the histological features suggestive of IgG4‐related disease’, as described in the consensus statement regarding this condition. Extracholecystic inflammatory extension (seven cases, P = 0.009) and mass‐forming lesions (eight cases, P < 0.001) were more frequently seen in XGC cases with histological features suggestive of IgG4‐related disease than in cases without those microscopic changes. Conclusions: XGCs with IgG4‐positive cell infiltration are considered to be mimickers, as xanthogranulomatous inflammation generally contradicts a diagnosis of IgG4‐related disease and is weakly associated with other typical organ manifestations of IgG4‐related disease. However, XGC may be a concurrent condition, particularly in patients with IgG4‐related disease in other organs. [ABSTRACT FROM AUTHOR]

Xanthogranulomatous inflammation is a special type of inflammatory process with unknown etiology. Commonly involved organs are gallbladder and kidney. So far, only a negligible number of cases of xanthogranulomatous appendicitis (XA) have been reported in different literatures. Herein, we report a case of XA in a 49-year-old female patient, who presented with recurrent right iliac fossa pain. Clinically, appendicitis was diagnosed and appendectomy was performed. Histopathological examination revealed it as a case of XA. No other form of treatment was given and the patient is doing well 1 month postoperatively.

Xanthogranulomatous pancreatitis (XGP) is an extremely rare cause of a cystic pancreatic mass. The pathophysiology of this process is not entirely clear but likely results from a combination of duct obstruction, infection, and repeated hemorrhage. It is difficult to differentiate this inflammatory lesion from a cystic neoplasm and, therefore, in the majority of cases XGP is misdiagnosed as a neoplasm on preoperative imaging. In this report, we describe a case of XGP, the imaging characteristics of XGP, and a differential diagnosis for a cystic pancreatic lesion. [ABSTRACT FROM AUTHOR]

Objective: Unusual clinical course Background: Xanthogranulomatous pyelonephritis (XP) is an extremely rare, severe, atypical form of chronic renal parenchymal inflammation accompanied by hydronephrosis and/or urolithiasis. The pathomechanism of XP is not yet fully understood. Microscopically, XP is indicated by the presence of multinucleated giant cells and lipidladen macrophages, as well as inflammatory infiltration and intensive renal fibrosis. The lipid accumulation in kidney parenchyma may be secondary to the altered flow of low-density lipoprotein (LDL)-derived cholesterol particles inside the affected cells. Physiologically, the process of LDL-derived cholesterol transport from lysosomes to the sites of its esterification is dependent on vimentin, which is a molecule comprising the cytoskeleton in mesenchymal cells. Case Report: A 7-year old girl was hospitalized because of the finding of unexplained kidney lesions on an abdominal ultrasound examination (an enlarged and deformed collecting system of the right kidney with hyperechogenic, solid, staghorn lesions in the calyces). Three months earlier, the patient had experienced recurrent urinary tract infection. Based on the subsequent laboratory and imaging diagnostics, the final diagnosis of XP was established and the girl was qualified for right-sided nephrectomy Microscopic examination revealed numerous foci of granuloma formations with no evident exponents of dysplastic or neoplastic abnormalities. Significant CD68-positive cell infiltrations and scattered foam cells arranging the numerous foci of granuloma inflammation were noticed. Renal parenchyma, adjacent to granuloma lesions, presented a vimentin expression. Conclusions: Vimentin expression in XP may confirm a focal character of chronic granuloma formation and may suggest the complexity of XP pathogenesis involving not only macrophage and fibroblast activation but also local lipid deregulation and fibrosis. [ABSTRACT FROM AUTHOR]

Xanthogranulomatous inflammation is a specialform of chronic inflammation that is destruc-tive of affected organ. Xanthogranulomatous in-flammation of ovary and fallopian tube is an un-common entity and it is usually known to oc-cur in the kidney and gall bladder. Patients withxanthogranulomatous oophoritis present withlower abdominal or suprapubic pain, fever, men-orrhagia or vaginal bleeding, adnexal tendernessand a pelvic mass. This entity can be confusedwith ovarian tumour on clinical and radiologi-cal features. According to our search in the lit-erature 16 cases of xanthogranulomatousoophoritis have been reported.We report a 30 year old female, presented witha unilateral ovarian mass with clinical and ra-diological suspicion of ovarian tumour. Patientunderwent panhysterectomy, grossly the rightovary was enlarged and on cut section it wassolid with yellowish areas. Histopathologicalexamination revealed massive infiltration of theovarian tissue and fallopian tube by lipid-laden histiocytes admixed with mixed inflam-matory cell infiltrate suggestive ofxanthogranulomatous oophoritis. The case is ofinterest in the view of its rarity, also due to clini-cal and radiological resemblance to neoplasticlesion.