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Purpose: Adult-onset xanthogranuloma (AOX) of the corneoscleral limbus is a rare inflammatory condition of unknown aetiology. Similar to limbal juvenile xanthogranuloma (JXG), it presents as a growing mass at the corneoscleral junction. Limbal AOX and JXG can lead to sight-threatening complications if not managed in a timely manner. This systematic review summarises the main clinical and histopathological features of limbal AOX/JXG and discusses the management of this uncommon disease., Methods: We performed a literature search in the MEDLINE database for all historical entries, using the search terms "limbus", "limbal" and "xanthogranuloma", and retrieved all articles reporting on limbal xanthogranuloma. After refining the search to articles relevant to limbal AOX, we were able to identify ten adult cases of limbal AOX and compare those with all reported cases of limbal JXG., Results: Clinically, AOX usually presents as an isolated smooth, yellowish, dome-shaped nodule at the corneoscleral junction, similar to an ocular presentation of JXG, with which it also shares similar histopathological features., Conclusion: Limbal JXG and AOX may represent the same disease entity. Diagnosis relies on the clinical presentation, pathology and immunohistochemical profile. Spontaneous regression is unlikely, and thus prompt surgical intervention should be considered to prevent sight-threatening complications. Xanthogranuloma should be included in the differential diagnosis of corneoscleral limbal masses in patients of all age groups.

Adult xanthogranuloma presents most commonly as an orange-tan firm solitary nodule with no systemic manifestations. Recently, some cases have been reported in conjunction with lymphoproliferative disorders. Adult reticulohistiocytosis classically presents as red to yellow-red dermal nodules. In the multicentric form, lesions have a predilection for hands and elbows, with a classic coral bead periungual presentation, and are often associated with symmetric erosive arthritis, particularly of the hands and wrists. The presentation and course of Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy, can vary. The classic presentation is extensive, painless bilateral cervical lymphadenopathy, but some cases have been entirely extranodal., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Adult orbital xanthogranulomatous diseases are rare entities and encompass a group of disorders with varying manifestations that are poorly understood. Taken as a group, there are non-Langerhans histiocytic disorders (type II) that are diagnosed histologically by the presence of foamy histiocytes, Touton giant cells, and varying degrees of fibrosis. Based on the accompanying systemic associations, there are 4 main categories of adult xanthogranulomatous disease: adult-onset xanthogranuloma, adult-onset asthma and periocular xanthogranuloma, necrobiotic xanthogranuloma, and Erdheim-Chester disease. Herein, we discuss the etiopathogenesis, systemic associations, methods of diagnosis, and treatment options for these disorders., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Introduction: Xanthogranulomatous cholecystitis (XGC) is often mistaken for, and may predispose to, gallbladder carcinoma (GB Ca). This study reviews the worldwide variation of the incidence, investigations, management and outcome of patients with XGC., Methods: Data from 29 studies, cumulatively containing 1599 patients, were reviewed and results summarized by geographical region (Europe, India, Far East and Americas) with 95% confidence intervals (CIs) to present variability within regions. The main study outcomes were incidence, association with GB Ca and treatment of patients with XGC., Results: Overall, the incidence of XGC was 1.3-1.9%, with the exception of India where it was 8.8%. The incidence of GB Ca associated with XGC was lowest in European studies (3.3%) varying from 5.1-5.9% in the remaining regions. Confusion with or undiagnosed GB Ca led to 10.2% of patients receiving over or under treatment., Conclusions: XGC is a global disease and is associated with GB Ca. Characteristic pathological, radiological and clinical features are shared with GB Ca and contribute to considerable treatment inaccuracy. Tissue sampling by pre-operative endoscopic ultrasound or intra-operative frozen section is required to accurately diagnose gallbladder pathology and should be performed before any extensive resection is performed., (© 2013 International Hepato-Pancreato-Biliary Association.)

There were studied the peculiarities of the state of patients with xantogranulomatous cholecystitis (XGC) using clinical and instrumental diagnostics performed in preoperative period as well as morphological state of the wall of removed gall-bladders. It was established that the prevalence of XGC among patients after cholecystectomy because of biliary calculosis is 0,93%. The frequency of XGC in women is three times higher than in men. There is prevalence of patients older than 50 years. The clinical period of XGC in men is twice shorter than among women and a polimorbid pathology is more often found in men than in women. XGC has no specific clinical signs. Morphologically the xantogranulomes are the combination of xanthom cells, lymphocytes and plasmocytes. To detail this process it is necessary to determine subpopulations of T- lymphocytes presented in the xantogranulome.

Xanthogranuloma (XG) is an uncommon benign disorder characterized by solitary or multiple yellow-red papulonodules on the skin, and occasionally, in other organs. It is predominantly a disease of infancy or early childhood, although adults may rarely be affected. To compare the clinicohistopathological featues of juvenile-type xanthogranulomas UXGs) and adult-type xanthogranulomas (AXGs) (>14 years) in Korea, 30 cases of JXGs and 15 cases of AXGs were compared clinically and histopathologically. Except for the fact AXGs were more often solitary and larger and showed neither other associated systemic diseases nor spontaneous regression, the clinical features such as color, mean number, or site of the lesions in AXGs were not statistically different from JXGs. Histologically, AXCs were not significantly different in amounts of foamy cells, giant cells including Touton cells, and inflammatory cells, although subcutaneous involvement was seen only in the two infant cases. In conclusion, in contrast to AXGs, JXGs need special attention to accompanying systemic diseases and do not need excisional procedures, considering their frequent spontaneous regression.

A 45-year-old man with spinal injury and diabetes mellitus who complained high fever and progressive enlargement of left intrascrotal mass visited to our hospital. Preoperative ultrasonography demonstrated epididymitis and abscess formation. Left high orchiectomy was performed because testicular tumor could not be denied. Epididymis was replaces by bright yellow mass associated with abscess and adhered to testis strongly. Histopathologically, the mass diagnosed xanthogranulomatous epididymitis consisted of foamy macrophages and chronic inflammatory cells. This is the first case in Japanese medical literature.

Xanthogranulomatous cystitis is a rare benign chronic inflammatory disease of unknown aetiology. We report a case of xanthogranulomatous cystitis. The patient is well more than 15 years after partial cystectomy. The relevant literature is reviewed.

Xanthogranulomatous pyelonephritis is a clinico-pathological entity that is gaining awareness amongst urologists worldwide. It is an unusual chronic inflammatory lesion involving the kidney that destroys renal parenchyma and may mimic renal carcinoma. It is usually seen in middle-aged women and is associated with urinary tract infections and urinary calculi. Most reports and reviews of this condition come from the West; this study reviews the incidence and presentation of the condition in a Malaysian population.

Clinicopathological and immunohistochemical studies were performed on 22 cases of xanthogranulomatous cholecystitis (XGC). Incidence of XGC was 3.6% of surgically resected gallbladder diseases. All cases of XGC were associated with cholelithiasis in which cholesterol gallstones were 6 times as many as pigment ones. XGC was frequently accompanied by incarceration of gallstones. In cholecystography, two-thirds of gallbladders were not visualized. Histologically, the granulomatous lesion of XGC consisted of accumulations of foam cells, lymphocytes, variable numbers of giant cells, granulocytes and fibroblastic cells. Although there had been no established theory in relation to the origin of foam cells, it is considered that foam cells were derived from monocyte/macrophages because they reacted variably with lysozyme, alpha 1-antichymotrypsin and alpha 1-antitrypsin, and almost invariably with OKM 1 and EBM 11 antibodies. Interspersed among macrophage foam cells, many T lymphocytes were identified. In terms of T cell subsets, CD 8 positive lymphocytes outnumbered CD 4 positive lymphocytes. Electron microscopy demonstrated intimate apposition of T lymphocytes to macrophages or macrophage foam cells. HLA-DR was expressed by most macrophages, macrophage foam cells and T lymphocytes. In conclusion, the results suggest that the features of XGC are those of granulomatous inflammation characterized by accumulation of macrophages, macrophage derived foam cells and activated T cells. It is suggested that delayed type hypersensitivity reaction of cell mediated immunity is operative in the pathogenesis of XGC.

Xanthogranulomatous Cholecystitis (XGC) is an uncommon inflammatory disease. In a retrospective analysis of 159 cholecystectomy specimens revealed 21 cases of diffuse and focal XGC with an overall incidence of 13.2 per cent. The age distribution was identical to those of traditional chronic cholecystitis with female predominance (M:F ratio 1:4). Gallstones were seen in 15 cases with marked thickening of the gall bladder on ultrasonography. In one case it falsely diagnosed as carcinoma on ultrasonographic examination, however, histopathologically it was turned out to be XGC. One case of XGC was associated with adenocarcinoma of gall bladder. The incidence of diffuse XGC was 5.66 per cent, whereas incidence of focal XGC was 7.54 per cent amongst chronic cholecystitis.