膀胱嗜铬细胞瘤的诊断及治疗(附6例报告)

目的：探讨膀胱嗜铬细胞瘤的诊断及治疗方法。方法：回顾性分析６例膀胱嗜铬细胞瘤患者临床资料,并复习相关文献。结果：６例均经术后病理证实,术前确诊５例,行膀胱部分切除５例,肿瘤剜除１例。随访８个月￣１０年,血压正常,无肿瘤复发,结论：膀胱嗜铬细胞瘤的典型性症状是排尿时或排尿后血压升高、头痛、头晕、心悸、出汗等。Ｂ超、ＣＴ、ＭＲＩ及＾１３１Ｉ－ＭＩＢＧ等对诊断均有较高的价值。手术切除是最有效的治疗方法。     

腹腔镜膀胱嗜铬细胞瘤切除术

目的:探讨应用腹腔镜技术治疗膀胱嗜铬细胞瘤的可行性及疗效.方法:回顾分析为1例膀胱嗜铬细胞瘤患者施行腹腔镜膀胱嗜铬细胞瘤切除术的临床资料.术前均行B超、CT、MRI等检查,以明确诊断并定位,肿瘤43mm×32mm×30mm大.结果:手术顺利完成,手术时间100min,术中出血80ml,围手术期无并发症发生,术后住院7d...     

Laparoscopic treatment of hypertension after micturition: Bladder pheochromocytoma.

Bladder pheochromocytomas are extremely rare and constitute less than 5% of bladder tumors. The initial symptoms of the patient are mostly nonspecific caused by hypertension. The postmicturition hypertensive crisis is the typical warning sign of this disease. In this article, we present a 29-year-old female having hypertensive attacks following micturition. Radiological imaging techniques revealed a 3 x 3 x 4-cm bladder tumor that was hormonally active. This is the first case reported of bladder pheochromocytoma that was laparoscopically treated without using the adjunct transurethral resection. The postoperative follow-up of the patient confirms the success of the surgical procedure.     

超声及CT诊断膀胱嗜铬细胞瘤

目的探讨超声及CT诊断膀胱嗜铬细胞瘤的价值。方法分析经病理证实的6例膀胱嗜铬细胞瘤的超声及CT表现。结果灰阶超声（n=6）示膀胱嗜铬细胞瘤呈中低回声团块,形态较规则,边界清晰,其内部回声不均匀;彩色多普勒示病灶内部血供丰富;CEUS（n=4）示膀胱嗜铬细胞瘤呈高强度灌注,并且与膀胱壁同步,灌注较均匀,未见明显充盈缺损。CT（n=5）示膀胱嗜铬细胞瘤呈软组织密度影,密度较均匀,边界清晰,增强后明显强化。结论膀胱嗜铬细胞瘤超声及CT表现具有一定特征性,有助于其临床诊断。     

肾上腺外嗜铬细胞瘤34例报告

目的 总结肾上腺外嗜铬细胞瘤的诊治经验.方法回顾性分析34例肾上腺外嗜铬细胞瘤患者的临床资料.就诊原因:高血压27例,血压170～230/110～155 mm Hg(1 mm Hg=0.133 kPa),病程3个月～15年;腹部疼痛10例,病程5个月～6年;间歇性肉眼血尿2例,病程1周和3个月.34例实验室检查,血、尿儿茶酚胺升高20例,尿3-甲氧-4-羟苦杏仁酸升高24例.B超检查34例,肿瘤准确定位31例,阳性率91.2%.CT扫描25例,24例准确定位,阳性率96.0%.MRI检查6例,均准确定位.15例经腹、3例经耻骨上、16例经腰手术,其中32例完整切除肿瘤,1例行包膜下摘除术,1例行术中活检.结果 肿瘤位于肾门旁12例、左肾下极2例、下腔静脉后方1例、腹主动脉和下腔静脉之间3例、腹主动脉前方肠系膜根部2例、右髂总动脉前方1例、肝门部1例、胰腺后方1例、膀胱内2例、降结肠旁1例、多发肿瘤8例(2～3枚).良性嗜铬细胞瘤22例,恶性12例.恶性嗜铬细胞瘤患者中肿瘤单发7例,多发5例.手术切除瘤体直径2～15 cm.术后30例随访6个月～13年.术前27例有高血压症状者,22例血压于术后3 d～6个月恢复正常,5例仍有高血压者中3例舒张压恢复正常,收缩压波动于150～170 mm Hg;2例血压较术前稍有下降.1例膀胱良性嗜铬细胞瘤患者术后6个月肿瘤复发,行膀胱全切.12例恶性嗜铬细胞瘤患者中9例获随访,术后1年内均复发、转移,其中2例为术中未能完整切除肿瘤者,6例术后6个月～3年内死亡,其中3例死于脑溢血,3例死于肿瘤广泛转移;3例经131Ⅰ-间位碘代苄胍(MIBG)治疗后病情稳定,荷瘤生存.结论 肾上腺外嗜铬细胞瘤定位较为困难,易漏诊,CT扫描定位准确可靠.手术切除肿瘤是最佳治疗方法.恶性肾上腺外嗜铬细胞瘤术后可给予131Ⅰ-MIBG治疗.     

Pheochromocytoma in children and adolescents: a clinical spectrum

Purpose

The purpose of the study was to identify the spectrum of disease characteristics of pheochromocytoma in children.

Methods

Records of 21 consecutive children diagnosed with pheochromocytoma were reviewed. Patients' age, sex, presentation, associated conditions, diagnostic and imaging modalities used, preoperative preparation, operative details, outcome, and follow-up were recorded.

Results

The study included 21 children. Patients were diagnosed clinically and confirmed by biochemical tests. Tumors were localized by imaging studies and all were confirmed pathologically. Patients included 17 with adrenal and 4 with extra-adrenal tumors including 1 in the urinary bladder. Seventeen had sporadic and 4 had familial pheochromocytoma. Associated manifestations were the predominant features in 1 of the 4 patients with familial pheochromocytoma. The patient with bladder pheochromocytoma presented with gross hematuria. Hypertension and visual disturbances were prominent findings in the other patients with sporadic form. Two patients (1 sporadic and 1 familial) had malignant pheochromocytoma. One patient with benign pheochromocytoma had multiple recurrences in chromaffin-containing sites. All patients were treated surgically. Seventeen patients were treated preoperatively with alpha-adrenergic blockade. Two patients continued to have significant visual disturbances. One patient with malignant pheochromocytoma died of the disease, and 1 with recurrent pheochromocytoma had neurologic consequences.

Conclusions

Pheochromocytoma in children has unique characteristics. To our knowledge, this series is one of the largest reports of adrenal pheochromocytoma in children. It also reflects the spectrum of pheochromocytoma in this age group.     

Distinct microsatellite alterations in upper urinary tract tumors and subsequent bladder tumors

PURPOSE: Although synchronous and/or metachronous tumor development is common in urothelial cancer, genetic and biological differences in upper urinary tract and bladder tumors are unclear. We compared the genetic alteration pattern in multifocal disease in patients with upper urinary tract and subsequent bladder tumors, and those with recurrent bladder tumor. MATERIALS AND METHODS: Using 21 microsatellite markers on the 8 chromosomal arms 2q, 4p, 4q, 8p, 9p, 9q, 11p and 17p we analyzed 34 tumors from 15 patients with upper urinary tract and subsequent bladder disease, and 70 tumors from 22 with recurrent bladder disease. RESULTS: Judging from the patterns of genetic alterations multifocal tumors were considered to have derived from an identical progenitor cell in 7 of 13 evaluable patients (54%) with upper urinary tract and subsequent bladder tumors, and 16 of 19 (84%) who were evaluable with recurrent bladder tumor. These data confirm the view that seeding or intraepithelial spread is a major mechanism for the multifocal development of urothelial cancer in general. However, a discordant microsatellite alteration pattern in multifocal tumors was observed in 6 of 7 patients (86%) with upper urinary tract and subsequent bladder lesions but in 2 of 16 (13%) with recurrent bladder cancer (p <0.005). CONCLUSIONS: Our results imply that upper urinary tract neoplasms may be genetically more unstable than bladder neoplasms. The implantation of tumor cells from upper urinary tract to bladder may involve additional and diverse genetic alterations. Furthermore, a considerable number of multifocal upper urinary tract and subsequent bladder lesions may arise independently via field cancerization mechanism. Our study indicates that the factors contributing to multifocal development are different in the 2 groups.     

Rupture of urinary bladder secondary to bladder carcinoma with extensive abdominal gangrene: A case report

Introduction and importanceSpontaneous rupture of the urinary bladder is rare but potentially severe. It is unusually related to bladder tumours. The morbidity and mortality rate are very high in these groups of patients.Case presentationWe present a case of a 62-year-old man who was known to have a bladder tumour who presented with extensive gangrene of the anterior abdominal wall. Imaging showed an extraperitoneal urinoma extended to the anterior abdominal wall secondary to a bladder rupture with posterior bladder wall thickening suggesting a bladder tumour. After optimization of the patient’s condition, urinoma drainage and upper urinary tract drainage by bilateral nephrostomy, excision of all necrotic tissues and a biopsy of the bladder lesion was performed. At a multidisciplinary meeting, we opted for a transurethral resection of the bladder followed by palliative chemotherapy considering that the tumour was locally advanced and depending on the disease course and patient’s condition.Clinical discussionGangrene secondary to urinary bladder rupture caused by transitional cell carcinomas is a very rare disease with poor oncological and infectious prognoses. For these reasons, treatment is often palliative.ConclusionUrinary bladder rupture secondary to bladder carcinoma could rarely be complicated with abdominal gangrene. No standardized treatment is recommended seeing the extreme rarity of this disease and management should be discussed on a case-by-case basis.     

Partial Cystectomy of Paraganglioma of the Urinary Bladder Before Living Kidney Transplantation: Case Report

Background

Paraganglioma (extra-adrenal pheochromocytoma) of the bladder is a very rare disease, accounting for 0.06% of all bladder tumors. Optimal management of bladder paraganglioma before kidney transplantation is unknown. We report a case of partial cystectomy for urinary bladder paraganglioma before living kidney transplantation.

膀胱嗜铬细胞瘤的诊断和治疗

目的：提高膀胱嗜铬细胞瘤的诊断与治疗水平。方法：5例膀胱嗜铬细胞瘤患者均通过膀胱镜检查获得定位诊断并均经手术治疗，其中4例行膀胱部分切除，1例行全膀胱切除术。结果：5例获随访，时间2个月～9年。3例痊愈，2例分别于术后2个月、5年死于脑溢血。结论：膀胱镜是最主要的定位检查手段，术前准备与其他部位嗜铬细胞瘤同样重要，术式选择应依据病灶大小及部位。     

Clinical application of NMP22 and urinary cytology in patients with hematuria or a history of urothelial carcinoma

For evaluation of the clinical application of immunoassay for nuclear matrix protein 22 (NMP22 immunoassay) and urinary cytology for early diagnosis and detection of bladder cancer in patients with hematuria and/or a previous history of bladder cancer, 209 urine samples obtained from 137 patients presenting episodes of hematuria or a history of bladder cancer were assayed for NMP22 levels and/or prepared for cytology examination. Biopsy was performed when any visible tumor was identified during cystoscopy examination. The median NMP22 concentrations measured in samples taken from patients with active bladder cancer, from patients with a history of bladder cancer but no active disease, from patients with hematuria, and from healthy volunteers were 18.95, 5.45, 6.39, and 3.75 U/ml, respectively. The urinary NMP22 level recorded for patients with urothelial carcinoma was significantly higher than that noted for individuals without active disease. The sensitivity of the NMP22 assay and of urinary cytology in diagnosing bladder cancer was 69% and 67%, respectively. In contrast, the specificity of these two diagnostic modalities reached 72% and 93%, respectively. The NMP22 assay is slightly more sensitive but less specific than urinary cytology in detecting bladder cancer. This study indicates that determination of urinary NMP22 levels is a useful and noninvasive tool for the detection of bladder cancer because of its high sensitivity. The urinary NMP22 assay may be used as a first-line routine screening method; however, it cannot replace the use of urinary cytology because of its lower specificity.     

Clinical guidelines for interstitial cystitis and hypersensitive bladder syndrome

A clinical guideline and algorism for interstitial cystitis and hypersensitive bladder syndrome has been developed by a group of East Asian urologists as a revised form of the Japanese guideline for interstitial cystitis. The guideline defines interstitial cystitis (IC) as a disease of the urinary bladder diagnosed by 3 requirements; 1) a characteristic complex of lower urinary tract symptoms, 2) bladder pathology such as Hunner's ulcer and bladder bleeding after overdistension, and 3) exclusions of confusable diseases. The characteristic symptom complex is termed as hypersensitive bladder syndrome (HBS), which is defined as bladder hypersensitivity, usually associated with urinary frequency, with or without bladder pain. For the definite diagnosis of IC, cytoscopy or hydrodistension is crutial; HBS is the diagnosis when IC is suspected but not confirmed by the 3 requirements. Numerous therapeutic options are available; however, most of them lack in high level of evidence, leaving a few as recommended therapies. Etiology of IC are multifactorial; the interaction among nervous, immune and endocrine factors forms a vicious cycle, provocating and maintaining inflammatory reactions in the bladder. The inclusion and efficacy criteria for clinical trials should be standardized to enhance the clinical research for this disabling disease, which has proved to be more prevalent than previously believed.     

A comparison of the pathology of transitional cell carcinoma of the bladder and upper urinary tract

OBJECTIVE: To clarify the histopathological patterns of upper and lower urinary tract transitional cell carcinomas (TCCs), as previous reports suggest that upper urinary tract TCCs have a greater tendency towards high-grade disease than bladder TCCs, of which most are low-grade and low-stage tumours. PATIENTS AND METHODS: All patients presenting with TCC of bladder or upper urinary tract between February 1991 and December 2001 at one institution were identified. Further patient information was obtained from the hospital database and case-note review. RESULTS: In all, 164 patients with upper urinary tract TCC and 2197 with bladder TCC were identified. There was a correlation between grade and stage of both upper urinary tract and bladder TCCs. 35% of the upper tract TCCs were classified as grade 2 and 44% as grade 3, while for bladder TCCs, 31% of lesions were classified as grade 2 and 35% as grade 3 (P = 0.003). Of the upper urinary tract lesions 33% were stage pT2-T4, compared with only 20% of bladder TCCs (P = 0.001). CONCLUSIONS: Upper urinary tract TCC is a higher grade and stage disease than bladder cancer, a finding that emphasizes the need for aggressive treatment of upper urinary tract TCC. If endourological management of upper urinary tract TCC is considered, histopathological determination of tumour grade before treatment is essential.     

Enterovesical fistula secondary to Crohn's disease manifested by inflammatory pseudotumor of the bladder

The authors describe a case of enterovesical fistulas in a patient with Crohn's disease that presented exclusively as urinary symptoms manifesting as bladder tumor. Ultrasound examination showed a normal upper urinary tract and a bladder tumor. Cystoscopy examination revealed a bladder tumor at the retrotrigonal region without showing the fistula orifice. Transurethral resection of the tumor revealed an inflammatory pseudotumor of the urinary bladder at the results of histological studies. Magnetic resonance imaging showed a normal upper urinary tract but a bladder tumor and a sigmoidovesical fistula. The results of barium contrast studies established the presence of numerous enterovesical fistulas. Partial resection of the ileum, ileostomy, colostomy and suture of the bladder fistulas was performed. Histological diagnosis of the affected ileum was Crohn's disease. The clinical features, natural history and treatment of enterovesical fistula secondary of Crohn's disease were discussed.     

Upper urinary tract tumors after primary superficial bladder tumors: prognostic factors and risk groups

PURPOSE: We evaluated the prognostic factors of primary superficial bladder cancer that may predict a metachronous upper urinary tract tumor. We also determined whether the incidence of upper urinary tract disease varies according to risk group based on primary superficial bladder tumor classification. MATERIALS AND METHODS: We studied disease evolution in a cohort of 1,529 patients with a primary superficial bladder tumor. To determine the prognostic factors of upper urinary tract cancer we performed multivariate analysis using Cox regression. Independent variables were grade, T stage, multiplicity, tumor size, carcinoma in situ association, previous or synchronous upper urinary tract tumor and intravesical instillation. We also performed the chi-square test and Kaplan-Meier survival analysis to assess the variable incidence of upper urinary tract tumors according to primary superficial bladder tumor risk group classification. RESULTS: The incidence of upper urinary tract cancer was 2.6%. The only factor prognostic for an upper urinary tract tumor was multiplicity (relative risk 2.7, 95% confidence interval [CI] 1.06 to 6.84). All patients with an upper urinary tract tumor had a previously recurrent primary superficial bladder tumor. In the low, intermediate and high risk groups the incidence of upper urinary tract cancer was 0.6% (relative risk 1), 1.8% (relative risk 3.1, 95% CI 0.4 to 23.9) and 4.1% (relative risk 8.3, 95% CI 1.1 to 61.6), respectively (chi-square and log rank tests p = 0.007 and p <0.05, respectively). CONCLUSIONS: A higher risk of upper urinary tract cancer must be expected in cases of multiple primary superficial bladder tumors. This finding supports the multicentricity theory of transitional cell carcinoma. Primary superficial bladder tumor classification by risk group is also useful for predicting the various risks of metachronous upper urinary tract cancer.     

核基质蛋白22检测在膀胱癌筛查中的应用

目的：探讨检测核基质蛋白22(NMP22)在膀胱癌筛查中的应用价值。方法：检测129例血尿患者的尿NMP22，并分为膀胱癌组和非肿瘤组进行比较。结果：膀胱癌组患者尿NMP22值高于非肿瘤组(P＜0．01)。结论：尿NMP22检测具有较高的敏感性和特异性，在膀胱癌筛查中可以选择应用。     

女性膀胱颈梗阻的诊治体会

目的：提高女性膀胱颈梗阻的诊治水平。方法：通过对26例女性膀胱颈梗阻患者经膀胱颈梗阻电切术，对其诊断和治疗进行回顾性分析。结果：26例膀胱颈梗阻患者(其中例合并腺性膀胱炎)全部进行电切术，术后疗效满意，无1例术后并发症。结论：膀胱镜检可明确诊断膀胱颈梗阻，膀胱颈梗阻部分电切术是治疗本病的最佳方法。     

Malacoplakia of the urethra

We report a case of urethral malacoplakia associated with disease of the bladder and bladder neck. Treatment consisted of fulguration of the bladder lesions and excision of the urethral lesion. Unlike most other cases of genitourinary malacoplakia, which are associated with chronic urinary tract infection, this patient had sterile urine and no history of urinary tract infections. Urethral malacoplakia is reviewed, and the pathogenesis and treatment of the disease are discussed.     

A case of emphysematous cystitis

Emphysematous cystitis is a rare lower urinary tract infection. Patients with diabetes mellitus, neurogenic bladder, and recurrent urinary tract infection are generally at higher risk of this disease. A 71-year-old woman with neurogenic bladder was referred from the internal medicine department because of urinary retention. Abdominal radiography and computed tomographic (CT) scanning revealed a characteristic accumulation of air in the wall and lumen of the urinary bladder. Emphysematous cystitis was improved by antibiotic therapy and urinary drainage. CT scan was a sensitive method for detecting early signs and confirming the diagnosis.     

Management options for sphincteric deficiency in adults with neurogenic bladder

Neurogenic bladder is a very broad disease definition that encompasses varied disease and injury states affecting the bladder. The majority of patients with neurogenic bladder dysfunction do not have concomitant intrinsic sphincteric deficiency (ISD), but when this occurs the challenges of management of urinary incontinence from neurogenic bladder are compounded. There are no guidelines for surgical correction of ISD in adults and most of the literature on treatment of the problem comes from treatment of children with congenital diseases, such as myelomeningocele. Our goal, in this review, is to present some of the common surgical options for ISD [including artificial urinary sphincters, bladder slings, bladder neck reconstruction (BNR) and urethral bulking agents] and the evidence underlying these treatments in adults with neurogenic bladder.