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 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.

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.

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.

UNASSIGNED: Xanthogranulomatous pyelonephritis (XPGN) is a rare pathology of the kidneys occurring in 0.6 to 1% of all cases of renal infections, in both men and women. It is characterized by severe inflammation of the renal parenchyma leading to formation of granulomatous tissue containing lipid-laden macrophages. This condition may mimic less aggressive or benign conditions but may worsen or be fatal if not treated aggressively.
METHODS: Our patient is a 54 year old Caucasian female who presented with five days of left flank pain, hematuria, chills, nausea and vomiting. Imaging and biopsy results showed that the patient had XPGN.
UNASSIGNED: XPGN is a difficult condition to diagnose as the symptoms are non-specific relative to renal cell carcinoma or other common renal infections. Definitive diagnosis is made with a biopsy; however, clues in various imaging modalities are used to make a tentative diagnosis. It is unclear whether earlier surgical intervention would have improved overall patient outcomes. Currently, a partial or complete nephrectomy is the only effective treatment.
CONCLUSIONS: Aggressive management including early diagnosis, antibiotics and nephrectomy appears to be critical in preventing progression and complications of XPNG.

Xanthogranulomatous endometritis is a rare benign pathology mimicking endometrial carcinoma.

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.