Xanthogranulomatous

黄色肉芽肿
  • 文章类型: Case Reports
    及时诊断黄色肉芽肿性肾盂肾炎(XGP),一种罕见的慢性肾病,连同其适当的管理,是使肾脏免于终末期肾病(ESRD)的必要条件。大多数疾病早期诊断的主要障碍,包括XGP,是没有特定和特征性的症状,which,如果存在,会使患者更早地寻求医疗援助,并诱使临床医生考虑可变的鉴别诊断。我们在此报告一例20岁的男性患者,没有特定的症状提示肾脏病理,这耽误了他考虑咨询医疗保健专业人员,因此,在他的受累肾脏几乎无法挽救时,他的病情被诊断为XGP。通过这个案例报告,我们希望谦卑地要求世界各地的临床医生在处理非特异性症状的患者时,扩大他们的鉴别诊断范围,以便有更好的预后。
    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.
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  • 文章类型: Case Reports
    我们报告了一名患者,该患者最初因诊断为Erdheim-Chester病(ECD)的视力丧失而就诊于眼科诊所。
    ECD是一种罕见的非朗格汉斯细胞组织细胞增生症,其特征是多系统器官受累和预后不良。在出现任何全身症状之前,我们的患者由于突出的眼眶受累而完全视力丧失。该病例表现出不同的ECD临床表现。
    对类固醇治疗反应差的无痛性双侧眼球突出应提示考虑ECD和系统评估。此外,在没有典型临床表现的情况下,对活检的全面评估对于准确诊断至关重要。
    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.
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  • 文章类型: Case Reports
    垂体炎是一种极其罕见的炎症性疾病,可以模仿垂体腺瘤的临床和放射学特征。在这个案例报告中,我们描述了一名45岁女性,患有继发性黄色肉芽肿性垂体炎(XGH),表现为垂体大腺瘤.病人抱怨头痛,视力障碍,闭经-溢乳综合征.体检正常。实验室调查显示促肾上腺皮质激素,促甲状腺激素,和促性腺激素缺乏。她的右眼视力也很低,视野也改变了。垂体磁共振成像显示鞍内和鞍上肿块13×11×16mm,伴有出血性坏死,这对患者的视交叉和垂体柄有离散的质量影响。患者接受了氢化可的松和左甲状腺素治疗,然后转到神经外科进行全蝶窦切除。肿瘤的组织学检查允许对重塑的Rathke囊囊肿进行XGH诊断。系统性疾病,如结核病,结节病,排除其他肉芽肿性疾病。XGH的病因仍然不明确,但它可能是淋巴细胞性垂体炎的进行性形式或重塑的Rathke囊囊肿。筛查自身免疫性病理学和全身性疾病对于指导适当的管理至关重要。
    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.
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  • 文章类型: Case Reports
    黄色肉芽肿性肾盂肾炎是一种罕见的疾病,由慢性炎症和肾实质感染引起。它通常是阻塞性慢性肾盂肾炎的结果。原发性肾盂鳞状细胞癌是一种独特的病理,在临床实践中非常罕见,与黄色肉芽肿性肾盂肾炎有明确的联系。作者介绍了一名57岁的女性,患有慢性肾盂肾炎,并伴有黄色肉芽肿特征。随后的检查显示,没有怀疑,原发性肾盂鳞状细胞癌。有了这个案子,作者打算强调和加强需要警惕,由于其诊断,两种罕见疾病之间的罕见关联,治疗性的,和预后影响。
    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.
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  • 文章类型: Case Reports
    黄色肉芽肿性卵巢炎是一种罕见的,慢性和非肿瘤性病症,其中重度泡沫组织细胞炎性浸润与中性粒细胞混合,浆细胞,多核巨细胞,成纤维细胞和坏死灶引起广泛的组织损伤和器官破坏。胆囊和肾脏只是表现出类似黄色肉芽肿改变的组织学变化的不同器官的两个例子。本案涉及一名40岁的女性,她表现为卵巢肿块,最终被诊断为黄色肉芽肿性肾炎,尽管最初的临床放射学怀疑恶性肿瘤。黄色肉芽肿性肾炎是一个重要的实体,因为,临床和影像学检查,它类似于卵巢肿瘤,取决于仔细的组织病理学分析以确定诊断。
    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.
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  • 文章类型: Case Reports
    黄色肉芽肿性膀胱炎(XC)是一种非常罕见的膀胱疾病,病因不明。它可能模仿膀胱恶性肿瘤;因此,组织病理学评估是诊断的关键.我们报告了一例38岁的女性,无痛性血尿,临床和膀胱镜检查强烈考虑膀胱恶性肿瘤。然而,关于组织病理学评估,做出了罕见的XC诊断。她接受了一个疗程的抗生素治疗,随访四个月后仍无症状。据我们所知,这是尼日利亚和非洲首例XC病例。
    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.
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  • 文章类型: Case Reports
    黄色肉芽肿性肾盂肾炎(XPG)是一种已知的临床实体;然而,这种炎症病理进一步进展到邻近器官,包括输尿管,膀胱和尿道,极为罕见。输尿管黄色肉芽肿性炎症是一种慢性炎症状态,在固有层中可见泡沫巨噬细胞,多核巨细胞和淋巴细胞形成肉芽肿性炎症。这是良性的。根据其在计算机断层扫描(CT)扫描图像上的外观,很容易被误认为是恶性肿块,患者可能会接受可能导致并发症的手术。在这里,我们介绍了一名老年男性,其已知患有慢性肾脏疾病的病例,患有不受控制的2型糖尿病,并伴有发烧和排尿困难。经过进一步的放射学调查,患者有潜在的败血症,被发现有一个累及右输尿管和下腔静脉的肿块。活检和组织病理学检查,他被诊断为黄色肉芽肿性输尿管炎(XGU)。患者接受了进一步治疗并进行了随访。
    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.
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  • 文章类型: Case Reports
    背景:垂体黄色瘤病变与Rathke裂囊肿破裂或出血性有关。据报道,大多数病例在根治性切除术后消退。当复发发生时,没有既定的治疗方案。据报道,在一些已发表的病例中,大剂量糖皮质激素是有益的;然而,一旦停止治疗,它们的效果通常不会持续。
    方法:作者报告了一例青少年男性,尽管进行了两次手术干预,但还是出现了与Rathke囊肿相关的复发性黄色肉芽肿性垂体炎。他接受了短期地塞米松治疗,然后接受了塞来昔布和霉酚酸酯的维持治疗。该方案被证明是安全和耐受性良好的,它成功地阻止了他的黄色肉芽肿性垂体炎的另一次复发。
    结论:本病例证明了一种治疗复发性黄色肉芽肿性垂体炎的新的非手术方法。这表明保留皮质类固醇的免疫抑制和抗炎联合方案在其他难治性黄色肉芽肿性垂体炎病例中的潜在应用。
    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.
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  • 文章类型: Case Reports
    黄色肉芽肿性肾盂肾炎(XGP)是一种罕见的严重肾盂肾炎。它的特点是由慢性尿路梗阻和感染引起的进行性实质破坏,通常导致无功能的肾脏增大。它很少出现腰大肌脓肿,文献中只描述了少数情况。与XGP的典型症状不同,我们的病人出现了典型的腰痛症状.一旦诊断确定,给予抗生素并进行肾切除术.不幸的是,手术后,患者右下肢出现轻度单瘫,膝关节伸展肌肉力量下降,需要手杖支撑。尽管如此,延迟诊断可能是致命的.
    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.
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  • 文章类型: Journal Article
    目的:黄色肉芽肿性胆囊炎(XGC)是慢性胆囊炎的一种罕见变种。这种罕见的病理特征是胆囊壁严重和进行性纤维化以及富含脂肪的巨噬细胞浸润。
    方法:回顾性分析2011-2019年8213例胆囊切除术的最终病理报告,将病理结果报告为XGC的患者纳入研究。患者的人口统计学特征,病理结果,并对手术方法进行了评价。对中转开腹胆囊切除术的危险因素进行Logistic回归分析。
    结果:胆囊切除术中XGC的发生率为0.91%。患者的平均年龄为57.32岁。92%(n:69)的患者采用了腹腔镜胆囊切除术。这些患者在术前都没有怀疑癌症,但是在手术中发现10.6%的患者怀疑癌症。有了冷冻测试,这些患者避免了不必要的手术。开腹胆囊切除术的转化率为26.09%。转换为开腹胆囊切除术的最常见原因(66.7%)是强烈的纤维化。超声检查发现胆囊壁厚度增加和急性胆囊炎是有统计学意义的危险因素(p<0.05)。XGC病例的总并发症发生率为3.9%。
    结论:XGC是一种极其罕见的疾病,在胆囊切除术前难以诊断。特别是在术前USG中,在没有怀疑恶性肿瘤的情况下,但手术期间怀疑是恶性肿瘤,在大范围手术前使用冷冻方法进行组织病理学检查可以防止不必要的解剖和相关的并发症。
    OBJECTIVE: Xanthogranulomatous cholecystitis (XGC) is a rare variant of chronic cholecystitis. This rare pathology is characterized by severe and progressive fibrosis of the gallbladder wall as well as infiltration of fat-laden macrophages.
    METHODS: The final pathology report of 8213 cholecystectomies performed between 2011 and 2019 was evaluated retrospectively, and patients whose pathology result was reported as XGC were included in the study. Patients\' demographic characteristics, pathology results, and surgical methods were evaluated. Logistic regression analysis was performed for risk factors on conversion to open cholecystectomy.
    RESULTS: The rate of XGC among cholecystectomies was 0.91%. Mean age of the patients was 57.32 years. Laparoscopic cholecystectomy was applied to 92% (n: 69) of the patients. None of the patients had cancer suspicion in the preoperative period, but cancer suspicion was found in 10.6% of the patients during the operation. With the frozen test, unnecessary surgeries were prevented in these patients. Conversion rate to open cholecystectomy was found to be 26.09%. The most common reason for conversion to open cholecystectomy (66.7%) was intense fibrosis. Increased gallbladder wall thickness and acute cholecystitis were found to be statistically significant risk factors in ultrasonography (p <0.05). Total complication rate in XGC cases was 3.9%.
    CONCLUSIONS: XGC is an extremely rare disease and is difficult to diagnose before cholecystectomy. Especially in preoperative USG, in cases with no suspicion of malignancy, but with suspected malignancy during the operation, histopathological examination with frozen method before extensive surgery may prevent unnecessary dissection and related morbidities.
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