UNASSIGNED: We report a patient who initially visited the ophthalmology clinic for a vision loss diagnosed with Erdheim-Chester Disease (ECD).
UNASSIGNED: 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.
UNASSIGNED: 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.

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.

Xanthogranulomatous pyelonephritis is a rare disease resulting from chronic inflammation and infection of the renal parenchyma. It usually arises as a consequence of obstructive chronic pyelonephritis. Primary squamous cell carcinoma of the renal pelvis is a distinct pathology, very rare in clinical practice, with a well-established association with xanthogranulomatous pyelonephritis. The authors present the case of a 57-year-old woman with chronic pyelonephritis containing xanthogranulomatous features. Subsequent workup revealed a concomitant, unsuspected, primary squamous cell carcinoma of the renal pelvis. With this case, the authors intend to emphasize and reinforce the need to be alert to an uncommon association between two rare diseases due to its diagnostic, therapeutic, and prognostic implications.

Xanthogranulomatous oophoritis is a rare, chronic and non-neoplastic condition in which a heavy foamy histiocyte inflammatory infiltrate admixed with neutrophils, plasma cells, multinucleated giant cells, fibroblasts and foci of necrosis causing extensive tissue damage and organ destruction. The gallbladder and kidney are just two examples of the different organs that exhibit histological changes resembling xanthogranulomatous alteration. The present case involved a 40-year-old female who presented with a tuboovarian mass and was ultimately diagnosed with xanthogranulomatous oophritis, despite initial clinicoradiological suspicions for malignancy. Xanthogranulomatous oophritis is a significant entity because, clinically and radiographically, it resembles tumours of the ovary and hinges on a careful histopathological analysis to establish a diagnosis.

OBJECTIVE: To present the results of our case series on laparoscopic nephrectomy in xanthogranulomatous pyelonephritis (XGP).
METHODS: A retrospective study was conducted that included 143 patients treated with laparoscopic nephrectomy for non-functioning kidney, of whom 15 had XGP, within the time frame of 2011 to 2019. The demographic and clinical data were collected, along with the intraoperative results, complications, and days of hospital stay.
RESULTS: Transperitoneal laparoscopic nephrectomy was successfully performed on 15 patients with XGP, with no need for conversion. Mean intraoperative time was 124.4 minutes (range 70-240) and intraoperative blood loss was 148.5 ml (range 30-550), with no blood transfusion required. No intraoperative complications occurred but there was one postoperative complication (6.6%), classified as Clavien-Dindo I (surgical wound infection). Mean hospital stay was 2.85 days (range 2-7).
CONCLUSIONS: Nephrectomy is the definitive management for XGP, and the laparoscopic approach should be considered a treatment modality, despite the fact that the pathology involves a severe chronic inflammatory process. Its benefits are reduced surgery duration, less blood loss, a lower complication rate, and fewer days of hospital stay, when performed by a skilled and experienced surgeon.
OBJECTIVE: Presentar los resultados de nuestra serie de nefrectomía laparoscópica en pielonefritis xantogranulomatosa (PXG).
UNASSIGNED: Se realizó un estudio retrospectivo que incluyó 143 pacientes tratados con nefrectomía laparoscópica por exclusión renal, de los cuales 15 fueron por PXG, en el periodo comprendido de 2011 a 2019. Se recolectaron datos demográficos y clínicos, resultados transoperatorios, complicaciones y días de estancia hospitalaria.
RESULTS: Se realizó nefrectomía laparoscópica transperitoneal de forma exitosa en 15 pacientes con PXG, sin necesidad de conversión. El tiempo transoperatorio promedio fue de 124.4 minutos (rango: 70-240). El sangrado transoperatorio fue de 148.5 ml (rango: 30-550), sin requerimiento de transfusión sanguínea. No se reportaron complicaciones transoperatorias; se presentó una complicación en el posoperatorio (6.6%) clasificada como Clavien-Dindo I (infección de la herida quirúrgica). La estancia hospitalaria promedio fue de 2.85 días (rango: 2-7).
CONCLUSIONS: El manejo definitivo de la PXG es la nefrectomía, y el abordaje laparoscópico debe ser considerado como una modalidad de tratamiento a pesar de ser una patología que presenta un proceso inflamatorio grave y crónico, obteniéndose beneficios como disminución en el tiempo quirúrgico, menor sangrado, menor tasa de complicaciones y menos días de estancia hospitalaria cuando es realizado por un cirujano experimentado.

Xanthogranulomatous cystitis (XC) is a very rare urinary bladder condition, of unknown etiology. It may mimic bladder malignancy; therefore, histopathologic assessment is crucial in diagnosis. We report a case of a 38-year-old female who presented with persistent, painless hematuria and a strong consideration of bladder malignancy clinically and on cystoscopy. However, on histopathologic evaluation, the rare diagnosis of XC was made. She received a course of antibiotics and has remained asymptomatic after four months of follow-up. To the best of our knowledge, this is the first reported case of XC in Nigeria and Africa.

Xanthogranulomatous pyelonephritis (XPG) is a known clinical entity; however, the further progression of this inflammatory pathology to adjacent organs, including the ureter, bladder and urethra, is extremely rare. Xanthogranulomatous inflammation of the ureter is a chronic inflammatory state where foamy macrophages are seen in the lamina propria along with multinucleated giant cells and lymphocytes forming a granulomatous inflammation, which is benign. Based on its appearance on computed tomography (CT) scan images, it can easily be misidentified as a malignant mass, and the patient can be subjected to surgery that can lead to complications. Here we present a case of an elderly male with a known case of chronic kidney disease with uncontrolled type 2 diabetes mellitus who presented with fever and dysuria. Upon further radiological investigations, the patient had underlying sepsis and was seen to have a mass involving the right ureter and inferior vena cava. Upon biopsy and histopathology, he was diagnosed with xanthogranulomatous ureteritis (XGU). The patient underwent further treatment and was followed up.

BACKGROUND: Xanthomatous lesions of the pituitary have been linked to ruptured or hemorrhagic Rathke\'s cleft cysts. Most cases are reported to resolve following radical resection. When recurrence does occur, there is no established treatment regimen. High-dose glucocorticoids have been reported to be beneficial in several published cases; however, their effects are often not sustained once therapy is discontinued.
METHODS: The authors report the case of an adolescent male who developed recurrent xanthogranulomatous hypophysitis associated with a Rathke\'s cleft cyst despite two surgical interventions. He was treated with a short course of dexamethasone followed by a maintenance course of celecoxib and mycophenolate mofetil. This regimen proved to be safe and well-tolerated, and it successfully prevented another recurrence of his xanthogranulomatous hypophysitis.
CONCLUSIONS: This case demonstrates a novel nonsurgical approach to the management of recurrent xanthogranulomatous hypophysitis. It suggests a potential application of a combined corticosteroid-sparing immunosuppressive and anti-inflammatory regimen in other cases of refractory xanthogranulomatous hypophysitis.

OBJECTIVE: 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.
CONCLUSIONS: 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.

Xanthogranulomatous pyelonephritis (XGP) is a rare form of severe pyelonephritis. It is characterized by progressive parenchymal destruction caused by chronic urinary tract obstruction and infection, typically resulting in a non-functioning enlarged kidney. Its presentation with a psoas abscess is infrequent, and only a few cases are described in the literature. Unlike the typical presenting symptoms of XGP, our patient presented classic symptoms of lumbago. Once the diagnosis was established, antibiotics were given and a nephrectomy was performed. Unfortunately, after the surgery, the patient developed mild monoparesis on the right lower limb with decreased knee extension muscle strength and needed a walking stick for support. Nonetheless, a delayed diagnosis could have been fatal.