Metastatic xanthogranulomatous pyelonephritis managed with immunosuppression

A male aged in his mid-60s was diagnosed with xanthogranulomatous pyelonephritis after a left nephrectomy for a renal mass that was detected during the investigation of weight loss and drenching night sweats. Past medical history includes type 2 diabetes mellitus, transient ischaemic attack, hypertension, non-alcoholic fatty liver disease, dyslipidaemia, osteoarthritis and active smoking. Three years after the initial diagnosis, the patient represented with abdominal pain. CT imaging demonstrated new pulmonary and pancreatic lesions, which were histologically confirmed to be xanthogranulomatous disease. The patient was too unstable for surgical intervention so was commenced on glucocorticoids with marked improvement in his clinical condition with resolution of inflammatory markers and radiographic improvement. Weaning of prednisolone led to a relapse of disease, which was managed with re-introduction of high-dose prednisolone and the initiation of azathioprine. The patient is now 2 years post initiation of immunosuppressive therapy with stable renal function and no active inflammation.     

黄色肉芽肿性肾盂肾炎诊断与治疗

目的探讨黄色肉芽肿性肾盂肾炎的诊断及治疗方法。方法对2002年～2010年收治的黄色肉芽肿性肾盂肾炎10例患者的临床资料进行回顾性分析,结合文献复习,就其临床表现、影像学特征、诊断及鉴别诊断、治疗等问题进行讨论。结果 9例患者术前误诊,1例患者经B超引导下肾穿刺活检确诊,所有病例均经病理确诊为黄色肉芽肿性肾盂肾炎,1例予以单纯抗感染治疗,症状消失,9例行手术治疗,其中3例行根治性肾切除术,4例行肾切除术,2例行肾部分切除术。术后效果良好,随访5例,至今无1例复发。结论黄色肉芽肿性肾盂肾炎临床表现复杂多样,缺乏特异性,极易误诊。B超引导下肾穿刺活检,是确诊的良好手段。抗感染及手术是治疗的主要手段。     

Diffuse xanthogranulomatous pyelonephritis in a patient with myotonic dystrophy and cerebral palsy

Xanthogranulomatous pyelonephritis (XGP), a morphological and clinical variant of chronic pyelonephritis, is an uncommon disease in children. It is characterized by the destruction of the renal parenchyma and replacement by granulomatous tissue containing foamy lipid-laden macrophages and is classified into diffuse and focal XGP. We present a case of diffuse XGP in a child with myotonic dystrophy complicated by cerebral palsy and discuss the importance of correct diagnosis and preoperative management to reduce inflammation and improve malnutrition associated with the disease.     

Renal tuberculosis simulating xanthogranulomatous pyelonephritis with contagious hepatic involvement

Xanthogranulomatous pyelonephritis (XGPN) is a chronic renal infection typically associated with nephrolithiasis and a non-functioning kidney. Renal tuberculosis is a major cause of morbidity in developing countries. Despite recent advances in diagnosis, it is sometimes difficult to differentiate renal tuberculosis preoperatively from XGPN. We present herewith a case report of a patient who was preoperatively diagnosed with a right non-functioning kidney due to renal calculus with stage 3 XGPN and adjacent liver abscess on computed tomography. Subsequent histopathological examination of the nephrectomised specimen revealed renal tuberculosis. To our knowledge this is the first case of renal tuberculosis spreading to the liver and causing liver abscess formation which was misdiagnosed as XGPN preoperatively.     

Staghorn calculi and xanthogranulomatous pyelonephritis associated with transitional cell carcinoma

Untreated staghorn calculi can cause xanthogranulomatous pyelonephritis (XGP), diminished renal function, and renal malignancy. Squamous cell carcinoma (SCC) of the upper urinary tract is associated with kidney stones and chronic infection, but their association with transitional cell carcinoma (TCC) has not been proven and has rarely been reported in literature. We present a rare case of staghorn calculi and XGP associated with TCC.     

黄色肉芽肿性肾盂肾炎的误诊原因分析

目的提高黄色肉芽肿性肾盂肾炎的诊治水平.方法总结18例黄色肉芽肿性肾盂肾炎患者的诊治资料.男5例,女13例.平均年龄47岁.左侧6例,右侧12例.术前误诊为肾结石伴积水4例,输尿管结石伴肾重度积水3例,肾结核3例,肾癌8例.结果18例患者术中快速冰冻切片7例,诊断为黄色肉芽肿性肾盂肾炎,另11例肾切除术后病理检查确诊.病理报告：病灶处有大量炎性细胞,胞质内有大量含类脂的泡沫细胞,核较小且一致,无核分裂及异形性.18例术后随访6～124个月,未见复发.结论黄色肉芽肿性肾盂肾炎术前诊断困难,尿液涂片有泡沫细胞、CT值为低密度值以及抗炎治疗无效等为本病的临床特点,综合分析临床资料,提高对此病的临床认识是避免误诊的关键.     

Xanthogranulomatous pyelonephritis: our experience with review of published reports

BACKGROUND: The aim of this study was to analyse the cases of xanthogranulomatous pyelonephritis with review of published reports. METHODS: We retrospectively reviewed all nephrectomy specimens during February 1995 to January 2006. We found 26 cases of xanthogranulomatous pyelonephritis. We prepared a chart of these cases consisting of preoperative symptoms, laboratory findings, radiological imaging results and preoperative diagnosis. Open nephrectomy was carried out in all cases. RESULTS: Age group of the patients was 6-65 years and male : female ratio was 1.6:1. Twenty-two patients presented to us with intermittent high-grade fever, 21 with flank pain, 18 with loin mass, 2 with haematuria and 1 was detected during screening of vague gastrointestinal symptoms. Twenty-five patients had pyuria and only 10 had sterile urine culture. In all patients, only one kidney was affected. All patients had renal calculi 10-42 mm in size with bilaterally enlarged kidneys. Ipsilateral kidney was enlarged because of hydronephrosis or pyonephrosis in all cases and contralateral kidney was enlarged because of compensatory hypertrophy in 13 cases. Ipsilateral kidney had severely compromised renal function in all cases. Associated psoas abscess was present in one and tuberculosis in another. CONCLUSION: Xanthogranulomatous pyelonephritis is a relatively rare entity that is associated with obstruction, stones and infection of the urinary tract. Late presentation leads to loss of renal parenchyma. It cannot be differentiated preoperatively with renal tumours (renal cell carcinoma and Wilms' tumour), pyonephrosis, infected hydronephrosis and renal lymphoma. Nephrectomy and antibiotics are the treatment of choice.     

Xanthogranulomatous pyelonephritis with a renocolic fistula caused by a parapelvic cyst

Fistula formation between the upper urinary tract and bowel is an uncommon complication in urogenital diseases. We present a rare case of focal xanthogranulomatous pyelonephritis with a renocolic fistula. This is the first case where a parapelvic cyst obstructs the caliceal outflow and leads to the formation of a renocolic fistula in renal inflammatory disease. It is difficult to make a preoperative diagnosis of focal xanthogranulomatous pyelonephritis with widespread involvement that is caused by non-calculous urinary tract obstruction.     

Xanthogranulomatous pyelonephritis: a systematic review of treatment and mortality in more than 1000 cases

Objectives

To systematically review the current demographics, treatment and mortality rate associated with xanthogranulomatous pyelonephritis (XGP) and to test the hypothesis that the weighted pooled peri-operative mortality rate will be <10%.

Methods

Searches were performed of the Cochrane, Embase and Medline databases and the grey literature for studies published during the period 1 January 2000 to 30 August 2021. Eligible studies reported cohorts of ≥10 predominantly adult patients with XGP and described either average patient age or mortality rate.

Results

In total, 40 eligible studies were identified, representing 1139 patients with XGP. There were 18 deaths, with a weighted pooled peri-operative mortality rate of 1436 per 100 000 patients. The mean age was 49 years, 70% of patients were female and 28% had diabetes mellitus. The left kidney was more commonly affected (60%). Four patients had bilateral XGP, and all of whom survived. Renal or ureteric stones were present in 69% of patients, including 48% with staghorn calculi. Urine culture was positive in 59% of cases. Fistulae were present in 8%. Correct preoperative diagnosis occurred in only 45% of patients. Standard treatment continues to comprise a short cause of antibiotics and open radical (total) nephrectomy. Preoperative decompression occurred in 56% of patients. When considered at all, laparoscopic nephrectomy was performed in 34% of patients. Partial nephrectomy was conducted in 2% of patients.

Conclusions

Xanthogranulomatous pyelonephritis has a lower mortality rate than historically reported. A typical patient is a woman in her fifth or sixth decade of life with urolithiasis. While open radical nephrectomy remains the most common treatment method, laparoscopic, and to a lesser degree partial nephrectomy, are feasible in well selected patients.     

Xanthogranulomatous pyelonephritis: presentation and management in the era of laparoscopy

OBJECTIVE

To report a contemporary series of patients with xanthogranulomatous pyelonephritis (XGP, an inflammatory condition of the kidney that has traditionally been treated with open nephrectomy, ON), managed with either ON or laparoscopic nephrectomy (LN), as early reports suggest that the benefits of LN do not extend to patients with XGP.

PATIENTS AND METHODS

The medical and procedural records of patients with pathologically confirmed XGP between December 1993 to February 2004 were reviewed retrospectively. During this period, 26 patients (mean age 43.5 years, range 17–85) had surgical management of XGP; information on the mode of presentation, surgical management, hospital course and complications were analysed.

RESULTS

Twelve (46%) and 14 (54%) patients had ON and LN, respectively. The LN group had significantly lower blood loss (P = 0.002), transfusion rates (P = 0.02), time to resumption of oral intake (P = 0.002) and length of hospital stay (P = 0.002) than the ON group. One (7%) LN was converted to ON due to failure to progress. The overall complication rates between the ON and LN groups were not significantly different (P = 0.95).

CONCLUSIONS

LN in the setting of XGP is often difficult and requires advanced laparoscopic skills. In highly selected patients this approach can be offered, with acceptable morbidity, allowing for lower blood loss and shorter convalesce times.     

黄色肉芽肿性肾盂肾炎的多层CT动态增强表现特征

目的 分析黄色肉芽肿性肾盂肾炎(XGP)的多层CT动态增强表现特点.方法 自2007年1月至2010年10月间,我院共5例经手术病理证实的XGP患者术前接受多层CT平扫及多期增强扫描.与病理组织学结果对照,本研究回顾性分析XGP的多层CT平扫及动态增强表现.结果 5例患者全部为单肾受累,左肾2例,右肾3例.CT表现分两型:(1)全肾弥漫性病变3例,全部合并肾结石及中-重度肾积水,表现为患肾肾盂肾盏显著扩张,腔壁增厚,并不均匀延迟强化;肾盏周围见串珠状低密度区,病理为多发微小脓肿;3例均见病灶内斑点或斑片状脂肪密度区,肾周脂肪间隙模糊及肾周筋膜增厚;1例腰大肌、竖脊肌及腹壁脓肿形成.(2)肾脏局灶性病变2例,1例为单发囊性低密度灶,夹杂斑片状脂肪密度区,壁薄,多期增强扫描囊壁呈延迟强化,囊腔无强化,囊壁周围散在蜂窝状低密度区,病理为微小脓肿;1例为单发囊性混杂密度灶,夹杂斑片状脂肪密度区,壁薄,多期增强扫描无强化.2例患者病灶周围肾周筋膜增厚,但均未发现结石征象.结论 CT能准确评价XGP肾脏受累程度及肾外炎性病变的浸润范围,肾结石、肾积水基础上病变区低密度脂肪沉积及肾盏周围肾实质内串珠状微小脓肿高度提示该病诊断.     

Diffuse xanthogranulomatous pyelonephritis in a child with severe complications

Xanthogranulomatous pyelonephritis is a rare disease in childhood. Because the symptoms and signs are chronic and non-specific, preoperative diagnosis is usually difficult. We report an 8-year-old boy who had an abdominal mass and anemia for more than 6 months. Fever and dyspnea occurred 4 days prior to admission. Ultrasonography revealed an enlarged right kidney with multiple parenchymal hypoechogenic areas, absence of normal parenchymal structures, and perinephric thickening with multiple calcifications. An abdominal computed tomogram demonstrated an irregular, enlarged right kidney with multiple low-density round areas consistent with hydronephrosis and calculi. Diminished excretion of contrast media and a severe perinephric inflammatory reaction were present. Poor right kidney function was demonstrated by Tc^99m -diethylenetriamine penta-acetic acid split renal function examination. We diagnosed xanthogranulomatous pyelonephritis preoperatively based on the clinical and radiological features. The child first had drainage of an extrarenal abscess and antibiotic therapy, followed by definitive nephrectomy. The hospital course was complicated with pleural effusion, peritonitis, pelvic abscess, and sepsis. A two-stage nephrectomy requiring less radical resection and decreasing the surgical complications would have been preferable.     

肾盂脂肪瘤病合并膀胱黄色肉芽肿性膀胱炎一例并文献复习

目的探讨罕见的肾盂脂肪瘤病合并膀胱黄色肉芽肿性膀胱炎病例的发病机制、病理特点、临床表现、诊断和治疗。 方法回顾性分析1例我院收治的肾盂脂肪瘤病合并膀胱黄色肉芽肿性膀胱炎病例临床资料,复习相关文献并予以讨论。患者女,51岁,因"无痛性肉眼血尿6个月"入院,术前拟诊为左肾盂癌伴输尿管膀胱种植转移。 结果先后行膀胱镜检+活检术、经尿道膀胱肿物切除术、3D腹腔镜下左肾切除+左输尿管切除术,术中术后病理结果：左肾盂脂肪瘤病合并膀胱黄色肉芽肿性膀胱炎,术后随访6个月未见异常。 结论肾盂脂肪瘤病和膀胱黄色肉芽肿性膀胱炎均为临床上罕见的良性疾病,同一患者同时合并有这两种疾病的病例暂未见报道。该病需与泌尿系统的其他良恶性肿瘤相鉴别,以便正确诊断与及时治疗。     

黄色肉芽肿性肾盂肾炎的诊断与治疗(附8例报告)

目的　探讨黄色肉芽肿性肾盂肾炎 (XGP)的诊断及临床治疗方法。方法　总结 1995年至 2 0 0 2年收治的 8例XGP的临床表现、实验室检查结果、影像学特点及治疗方法。结果　CT扫描 8例 ,2例怀疑为XGP ;IVU及B超检查未做出特异性诊断提示。8例中行肾根治切除术 4例 ,肾切除 4例 ,全部获得治愈。结论　XGP缺乏特异性诊断方法 ,可根据临床及影像学表现做出初步诊断 ,必要时进行穿刺活检确诊。治疗上可根据临床分期、病变部位等采取肾切除术为主的治疗方式     

Xanthogranulomatous pyelonephritis with acquired cystic disease of the kidney in a haemodialysis patient

SUMMARY:   A 54-year-old-female patient who had received regular haemodialysis therapy for 12 years was referred to our hospital for evaluation of a left renal mass. Imaging examinations revealed acquired cystic disease of the kidney (ACDK) and a tumour-like lesion in the left kidney. Because of the preoperative diagnosis of the left renal cell carcinoma, the patient underwent a left nephrectomy. Pathological examination revealed xanthogranulomatous pyelonephritis. It was difficult to distinguish xanthogranulomatous pyelonephritis from renal cell carcinoma in our case, because it is very rare for xanthogranulomatous pyelonephritis to occur in ACDK.     

Focal xanthogranulomatous pyelonephritis associated with renal cell carcinoma.

A case of focal xanthogranulomatous pyelonephritis (XGP) with an associated renal cell carcinoma is presented. The tumor was discovered incidentally during a general surgical procedure. Subsequent evaluation showed a 3 cm mass in the lower pole of an otherwise normal kidney with no evidence of calculus or infection. Radical nephrectomy revealed classic findings of focal XGP with coexistent clear cell carcinoma. The literature describing the rare association between these conditions is reviewed.