膀胱副神经节瘤诊治特点分析

目的 探讨膀胱副神经节瘤的临床诊治特点和病理特点.方法 回顾性分析1985年6月至2010年3月11例确诊为膀胱副神经节瘤患者的临床资料.男5例,女6例.年龄14～66岁,平均39岁.9例有间断发作的用力排尿后的阵发性高血压、心悸及晕厥,11例均有出冷汗、头痛及头晕等症状.11例均行24 h尿儿茶酚胺水平测定,5例行甲襞微循环监测;11例均行B超、CT和(或)MRI检查;131I-间位碘代苄胍(MIBG)显像阳性4例,奥曲肽显像阳性7例,PET-CT阳性发现者1例.UICC膀胱肿瘤分期T11例、T25例、T34例、T41例.结果 11例行膀胱部分切除、腹腔镜和经尿道膀胱肿瘤电切等手术治疗,1例术后接受131I-MIBG治疗.在内镜下切除肿瘤时血压升高8例.术后儿茶酚胺水平恢复正常10例.病理学检查主要特征性为巢状细胞排列成"细胞球",周围包绕血窦.有盆腔淋巴结及肝、结肠转移1例.11例随访3～291个月,平均45个月.复发3例,再次切除,1例接受MIBG治疗;多发浸润及远处转移死亡1例.结论 40岁以下患者有头痛及排尿性晕厥、冷汗、心悸和血尿典型四联症的表现,应高度考虑膀胱副神经节瘤可能.131I-MIBG和奥曲肽显像有助于定位诊断.进展性T3以上、多灶性肿瘤以及CgA阳性表达是复发和转移的高危因素.肿瘤无法切除者用化疗药物和131I-MIBG治疗可控制高血压和延缓疾病进展.

Abstract：

Objective To discuss the clinical and pathological manifestations of paragangliomas of the urinary bladder (PUB) and improve the preoperative diagnosis and surgical treatment of PUB.Methods Clinical data of 11 patients treated for PUB from June 1985 to March 2010 were analyzed.All cases had mild-paroxysmal hypertension, palpitation, sweating, and 9 cases occasionally showed headache and micturition syncope during straining urination. Twenty-four hour urine catecholamine (CA) levels were estimated in all cases. B-ultrasound and CT and/or MRI scanning were used in the imaging of all cases. Nailfola microcirculation inspection was carried out in 5 cases. Scintigraphy 131I-MIBG in 4 patients was positive expression, 7 of 111 In-DTPA-Octreotide scintigraphy and I of PETCT were also positive expression. UICC bladder tumor classification was T1 (1 case), T2 (5 cases), T3(4 cases), T4 (1 case). Results All the patients underwent partial cystectomy, laparoscopy or TURBT. One patient received 131 I-MIBG therapy. Histopathological diagnosis was confirmed by HE staining in all of the removed tumors. The tumors consisted of discrete aggregates of zellballen cells separated by a network of vascular channels. Follow-up ranged from 3 to 291 months (mean, 45 months). There were 3 recurrences following surgery. One patient died after developing pelvic lymph nodes, liver and colon metastasis. Conclusions PUB should be suspected in patients below 40 years of age if the clinical manifestations of typical tetrad symptoms: headache and micturition syncope,sweating, palpitation and hematuria are present. Advanced classification, multifocal tumors and CgA (+) are risks of recurrence and metastasis. In those patients with unresectable multiple or recurrent tumors, chemotherapy and 131I-MIBG therapy may be helpful for controling hypertension and delaying progress.     

膀胱副神经节瘤诊治特点分析

目的 探讨膀胱副神经节瘤的临床诊治特点和病理特点.方法 回顾性分析1985年6月至2010年3月11例确诊为膀胱副神经节瘤患者的临床资料.男5例,女6例.年龄14～66岁,平均39岁.9例有间断发作的用力排尿后的阵发性高血压、心悸及晕厥,11例均有出冷汗、头痛及头晕等症状.11例均行24 h尿儿茶酚胺水平测定,5例行甲襞微循环监测;11例均行B超、CT和(或)MRI检查;131I-间位碘代苄胍(MIBG)显像阳性4例,奥曲肽显像阳性7例,PET-CT阳性发现者1例.UICC膀胱肿瘤分期T11例、T25例、T34例、T41例.结果 11例行膀胱部分切除、腹腔镜和经尿道膀胱肿瘤电切等手术治疗,1例术后接受131I-MIBG治疗.在内镜下切除肿瘤时血压升高8例.术后儿茶酚胺水平恢复正常10例.病理学检查主要特征性为巢状细胞排列成"细胞球",周围包绕血窦.有盆腔淋巴结及肝、结肠转移1例.11例随访3～291个月,平均45个月.复发3例,再次切除,1例接受MIBG治疗;多发浸润及远处转移死亡1例.结论 40岁以下患者有头痛及排尿性晕厥、冷汗、心悸和血尿典型四联症的表现,应高度考虑膀胱副神经节瘤可能.131I-MIBG和奥曲肽显像有助于定位诊断.进展性T3以上、多灶性肿瘤以及CgA阳性表达是复发和转移的高危因素.肿瘤无法切除者用化疗药物和131I-MIBG治疗可控制高血压和延缓疾病进展.     

膀胱副神经节瘤

膀胱副神经节瘤临床少见 ,本文总结近年国内外相关报道 ,论述了膀胱副神经节瘤的病因、临床表现、影响改变、病理特点及其治疗与预后。     

膀胱副神经节瘤的诊断和治疗

目的：探讨膀胱副神经节瘤的临床特点及诊治疗效。方法：对5例膀胱副神经节瘤患者的临床资料进行回顾性分析。结果：5例患者,男2例,女3例,平均46（36～77）岁。经尿道膀胱肿瘤电切（TURBT）2例,开放膀胱部分切除术3例。5例患者随访1～7年,1例术后3年肿瘤复发行TURBT,术后病理诊断为移行细胞癌1～2级。1例术后6年复发,伴有左肾重度积水,左肾无功能,行根治性膀胱切除加左肾输尿管切除加右输尿管皮肤造口。3例患者未见肿瘤复发及转移。结论：膀胱副神经节瘤临床少见,其临床表现有一定特点,对于排尿时伴有相应副神经节瘤临床表现的患者应除外膀胱副神经节瘤的可能,治疗方法主要以手术为主。     

膀胱副神经节瘤1例

膀胱副神经节瘤是一种罕见的肿瘤疾病,因多位于黏膜下层和肌层之间,且具有潜在恶性,故极易误诊为膀胱尿路上皮癌。本例报道1例27岁女性膀胱副神经节瘤患者。该患者因“大量血尿”入院,彩超、CT、MRI均提示膀胱占位。全身麻醉下行经尿道膀胱肿瘤电切手术,术后组织病理及免疫组织化学提示副神经节瘤。出院后2周再次发生无痛性肉眼血尿,全身麻醉下行膀胱部分切除术,术后痊愈出院。正确地诊断膀胱副神经节瘤具有重要的临床价值,尤其是影像学检查对诊断至关重要。手术是膀胱副神经节瘤最有效的治疗方法,而膀胱部分切除术是膀胱副神经节瘤的主要手术方式。     

膀胱副神经节瘤4例诊治分析及文献复习

目的探讨膀胱副神经节瘤的临床特点及诊治经验。方法回颐性分析我院2010年5月至201i年7月诊治的4例膀胱副神经节瘤患者的临床资料。其中女3例,男I例,年龄41～63岁。临床表现为高血压者2例,间歇性无痛肉眼血尿者1例,无症状者1例。4例中仅l例患者血去甲肾上腺素值明显高于正常。4例均行CT及膀胱镜检查。术前均按嗜铬细胞瘤做准备。结果3例行膀胱部分切除术,1例行肿瘤剜除术。术前2例高血压者术后血压恢复正常。术后随访6～20个月,未见肿瘤复发。结论膀胱副神经节瘤临床表现常缺乏特异性,膀胱非上皮肿瘤鉴别诊断时应考虑本病可能。血儿茶酚胺测定对膀胱副神经节瘤的定性诊断价值有限。CT和膀胱镜检查在肿瘤定位、定性诊断中起重要作用。膀胱部分切除术是首选的治疗方法,疗效确切,术前应充分扩容。     

膀胱功能性副神经节瘤诊治的临床研究

目的 探讨膀胱功能性副神经节瘤（paraganglioma of urinary bladder,PUB）的临床特点及诊治。方法 回顾性分析2003-2020年山东大学附属省立医院收治的5例功能性PUB患者的临床资料。5例患者中男2例,女3例;年龄26～61岁,平均年龄（40.0±13.7）岁。临床表现为排尿诱发的阵发性高血压、心悸,发作时血压高达240/120 mmHg (1 mmHg=0.133 kPa),术前均行超声、CT或磁共振成像检查,其中合并膀胱肿瘤1例。术前扩容准备后采用膀胱部分切除术治疗,行开放手术2例,行腹腔镜手术3例,其中合并膀胱肿瘤者同期行经尿道膀胱肿瘤切除术（transurethral resection of bladder tumor,TURBT）。结果 5例均顺利完成手术,患者术中血压平稳。术后病理证实为PUB, 4例行免疫组织化学显示嗜铬粒蛋白A (chromogranin A,CgA)、突触素（synapsin,Syn）阳性,增殖指数Ki-67表达1%～15%。随访33～153个月,平均随访（81.25±53.78）个月。仅1例（Ki-67为15%）合...     

膀胱原发性副神经节瘤8例诊治分析

目的:分析膀胱副神经节瘤的临床诊疗方法,以提高其诊疗技术水平。方法:回顾性分析2011年6月—2021年8月徐州市中心医院和蚌埠医学院第一附属医院收治的8例膀胱副神经节瘤患者的临床资料和随访结果。结果:所有患者术后病理结果均提示膀胱副神经节瘤,免疫组织化学染色标志物突触素(Syn)、瘤细胞嗜铬素A(CgA)均为阳性。术后随访3～126个月,平均59.3个月,8例患者均存活,术后无明显并发症,7例无复发,1例复发。结论:膀胱副神经节瘤临床易诊断,结合临床表现、影像学、膀胱镜可初步诊断,病理诊断可确诊。手术是治疗膀胱副神经节瘤的有效手段,术后并发症少,不易复发,能有效提高患者生活质量。     

泌尿系统副神经节瘤

副神经节瘤为发生在肾上腺外的嗜铬细胞瘤,其发生率约5%-10%,从颈部到盆腔的器鼢官均有可能发生,其分布与自主神经支配区域一致.近年来,发生在泌尿系统的副神经节瘤陆续有人报告,本文就其在泌尿系统的临床、病理及影像学表现等作一综述.     

泌尿系统副神经节瘤

副神经节瘤为发生在肾上腺外的嗜铬细胞瘤,其发生率约5％～10％,从颈部到盆腔的器官均有可能发生,其分布与自主神经支配区域一致。近年来,发生在泌尿系统的副神经节瘤陆续有人报告,本文就其在泌尿系统的临床、病理及影像学表现等作一综述。     

膀胱小细胞癌的病理组织学特点及其诊治

目的:探讨膀胱小细胞癌病理组织学特点及其诊断与治疗。方法:回顾分析6例膀胱小细胞癌患者的临床和病理资料,6例患者行经尿道膀胱肿瘤电切术2例,膀胱部分切除术1例,行膀胱全切术2例,单纯化疗1例。5例患者接受2～6疗程化疗。结果:6例随访3个月～3年,1例患者术后生存2年至今,5例均因肿瘤转移死亡,平均生存时间14.8个月。结论:膀胱小细胞癌分化程度低、恶性程度高、易早期转移、预后差,主要依靠病理组织学检查确诊,现有治疗方法不足以将其治愈,手术联合化疗是目前主要的治疗方法,分子靶向治疗是未来治疗的主要手段。     

膀胱肉瘤样癌的诊断与治疗（附2例报告）

目的 提高对膀胱肉瘤样癌的认识和诊治水平。方法 分析2例膀胱肉瘤样癌患者的临床资料并结合文献进行讨论。2例患者均有肉眼血尿或伴有膀胱刺激症，膀胱镜检查为实体瘤，表面有坏死组织；病理表现，肿瘤主要由移行上皮癌细胞和恶性间叶细胞（梭形或多形性细胞）组成，之间可见移行过渡；免疫组化CK（＋）、CEA（＋）、SMA（＋）。结果 2例Ⅰ期治疗均行经尿道膀胱肿瘤电切，术后常规膀胱灌注，1例术后5个月死于肿瘤复发及多处转移；1例半年后复发改行部分膀胱切除，至今健在。结论 膀胱肉瘤样癌是一种高度恶性、预后差的肿瘤。确诊需依赖病理和免疫组织化学检查，早期诊断和采用膀胱部分切除或根治性切除，是改善预后的关键。     

原发性膀胱小细胞癌诊治体会

目的 探讨膀胱小细胞癌的临床及病理特点.方法 回顾性分析9例膀胱小细胞癌的基本资料.男6例,女3例.年龄45～79岁,平均62岁.临床表现为肉眼血尿7例,排尿困难及下腹部疼痛2例.肿瘤直径0.5～7.0 cm,平均2.0 cm;多发2例,单发5例,全膀胱弥漫性生长2例.7例行尿脱落细胞学检查,阳性4例.9例均行手术治疗,其中4例术前诊断为浅表性者行TURBt,术后均定期行吡柔比星膀胱灌注,1例化疗3个周期;膀胱部分切除2例,均定期行吡柔比星膀胱灌注,1例化疗2个周期;根治性膀胱全切3例,静脉化疗2例.结果 术后病理检查:肿瘤细胞体积小,呈圆形,胞质稀少,核浓染,缺乏巢状结构.免疫组化染色:嗜铬素A(+)、神经元特异性烯醇化酶(+).诊断为小细胞癌,其中1例含移行细胞癌成分,1例合并前列腺癌.1例术前检查显示高血钙(3.15 mmol/L)和低血磷(0.61 mmol/L),术后1个月血钙及血磷恢复正常.保留膀胱者随访4例,3例分别于术后4、9、25个月死于转移,1例术后化疗者随访24个月未见复发及转移.膀胱全切3例中2例分别于术后2、28个月死亡,1例术后随访32个月未见肿瘤复发及转移.结论膀胱小细胞癌恶性程度高,预后差,根治性膀胱全切加全身化疗是主要的治疗方法,保留膀胱的手术应配合全身化疗.决定预后的是肿瘤的临床分期及治疗方法.

Abstract：

Objective To investigate the clinical and pathological features of small cell carcinoma of the urinary bladder. Methods The pathological and clinical data of 9 cases of small cell carcinoma were analyzed retrospectively. There were 6 males and 3 females, ages 45 to 79 years (mean age, 62 years). Clinical manifestations of 7 cases included gross hematuria and dysuria, the other 2 cases experienced lower abdominal pain. The mean tumor size was 2.0 cm (ranged, 0.5 to 7.0 cm). Two cases had multiple tumors and 5 cases had single tumors. The growth pattern in 2 cases was diffuse growth in the whole bladder. In 4 cases tumor cells were found in urine cytology. All 9 patients underwent surgical treatment, including TURBt. Four patients were diagnosed as superficial tumors before operation. All the patients underwent regular theprubicine irrigation in the bladder. One case underwent additional intravenous chemotherapy for 3 cycles. Partial cystectomy was performed in 2 cases, with regular theprubicine irrigation in bladder and 1 case underwent intravenous chemotherapy for 2 cycles. Radical cystectomy was performed in 3 cases, with 2 cases undergoing intravenous chemotherapy after operation. Results Pathological findings showed that tumor cells were small and round in shape. These hyperchromatic nuclei showed limited cytoplasm with lack of nesting characters. CgA and NSE were positive in immunohistochemistry. The final diagnosis was small cell carcinoma, with 1 case accompanied with transitional cell carcinoma and 1 case accompanied with prostate cancer. One case showed high preoperative serum calcium (3.15 mmol/L) and low serum phosphate (0.61 mmol/L), which returned to normal 1 month after operation. Four cases who′s bladder was preserved were followed up, 3 cases were alive for 4, 9 and 25 months after operation. The 1 case who underwent intravenous chemotherapy was followed up for 24 months and there was no sign of relapse or metastasis. In all the 3 cases with radical cystectomy, 2 cases died 2 and 28 months postoperativly. Another case with adjuvant chemotherapy was followed up for 24 months without recurrence or metastasis. Conclusions Small cell carcinoma of the urinary bladder is highly malignant with poor prognosis. Radical cystectomy in combination with systemic chemotherapy has better efficacy. Retained bladder surgery with systemic chemotherapy is an alternative choice. The most important factors which influence the prognosis of the tumor are clinical stage and therapeutic methods.     

Villous adenoma of the urinary bladder

Abstract:   Villous adenomas arising in the urinary tract are an uncommon occurrence. They have been identified in the urachus, urethra, prostate, and throughout the bladder. Villous adenomas arising in the bladder are rare tumors that have been described as isolated cases and a few case series. We report a new case of a large villous adenoma arising in the bladder that was treated by transurethral resection.     

Association of urinary bladder paragangliomas with germline mutations in the SDHB and VHL genes

ObjectiveOur primary goal was to examine the clinical characteristics of a series of patients with urinary bladder paragangliomas (UBPGLs), focusing particularly on their genetic backgrounds.Materials and methodsWe analyzed the medical records of patients who presented to the National Institutes of Health with UBPGL from 2000 to 2013 to determine their clinical characteristics and outcomes, biochemical phenotype, tumor size, and genetic background.ResultsOf the 27 patients with UBPGLs who were identified, 17 (63%) had underlying genetic mutations. Overall, 14 (51.9%) patients had a germline mutation in the succinate dehydrogenase subunit B gene (SDHB), and 3 (11.1%) had mutations in the von Hippel-Lindau gene (VHL). Of the 21 patients who had biochemical data available before their first operation, 19 (90.5%) presented with a noradrenergic biochemical phenotype; 7 (33.3%) patients had tumors that also secreted dopamine. In addition, 1 patient (4.8%) had elevated metanephrine levels, and 2 (9.5%) had normal biochemical data. In total, 13 (48.1%) patients in the series were diagnosed with metastatic disease, at either first presentation or follow-up; 6 of these patients (46.1%) had SDHB mutations.ConclusionsUBPGLs typically present with a noradrenergic phenotype and are frequently associated with underlying germline mutations. Patients presenting with these rare neuroendocrine tumors should be screened for these mutations. In addition, patients with UBPGLs should be followed up closely for metastatic development regardless of genetic background, as almost half of the patients in this series presented with metastatic disease and less than half of them had SDHB mutations.     

Multivariate analysis of clinical parameters of synchronous primary superficial bladder cancer and upper urinary tract tumor

PURPOSE: We determined the incidence and characteristic of synchronous upper urinary tract tumors (UUTTs) in patients with primary superficial bladder carcinoma and evaluated the characteristics of bladder tumors related to UUTTs. MATERIALS AND METHODS: We performed a retrospective study of 1,529 patients with primary superficial bladder carcinoma who underwent initial examination of the upper urinary tract with excretory urography. Data were analyzed by multivariate analysis using logistic regression. Variables evaluated and related to the incidence of UUTT were multiplicity, carcinoma in situ, bladder tumor size, localization of tumor in the bladder, and tumor grade and stage. RESULTS: A total of 28 patients (1.8%) had simultaneous bladder tumor and UUTT. UUTTs showed no preferred location and 17.9% were multiple. Of UUTTs 46% were invasive and almost 87% were grade 2 or 3. The only significant variable related to UUTT was bladder tumor in the trigone (RR 5.8, 95% IC 2.18 to 15.9, p <0.0005). Of 147 tumors located in the trigone 11 (7.5%) were associated with UUTT, corresponding to 41% of the UUTTs first diagnosed. If multiplicity and tumors in the trigone (551 cases) had been considered, 66.7% of tumors would have been diagnosed. CONCLUSIONS: Synchronous UUTT and superficial bladder tumor are uncommon but 46% are invasive. Considering the possible examination of the upper urinary tract only in patients with tumor in the trigone or with multiple bladder tumors 41.4% or 69% of UUTTs, respectively, would have been diagnosed. Patients with tumor in the trigone are at almost 6-fold higher risk for a synchronous tumor in the upper urinary tract.     

膀胱非上皮性肿瘤

目的：提高膀胱非上皮性肿瘤的诊治效果。方法：回顾性分析1990／2002年收治的11例膀胱非上皮性肿瘤的临床资料。结果：膀胱平滑肌瘤、血管瘤及副神经节瘤各1例,行膀胱部分切除,术后无复发;膀胱小细胞癌3例,其中1例仅作探查活检术,术后行髂内动脉插管化疗,于术后18月死亡;另外2例作膀胱部分切除术,分别于术后3月和7月死亡;膀胱横纹肉瘤2例,1例仅作根治性膀胱全切 原位回肠膀胱术,于术后6月死亡,另1例行膀胱部分切除术后失访;恶性副神经节瘤1例,行膀胱部分切除术后9年出现广泛转移;肉瘤样癌2例,1例仅作探查活检术,另1例仅作膀胱部分切除术,均于术后6月内死亡。结论：膀胱非上皮性肿瘤少见,良性肿瘤预后好;恶性肿瘤预后差,须行综合治疗,并作密切随访。