膀胱副神经节瘤

目的：总结膀胱副神经节瘤的临床、病理组织学特征及诊断与治疗方法。方法：报告５例膀胱副神经节瘤诊治情况,并复习有关文献。结果：５例膀胱副神经节瘤中４例为有功能性,１例为非功能性,病理均见肿瘤细胞排列成“细胞球”结构,周边血窦围绕形成器官样结构。３例行肿瘤切除术,２例行膀胱部分切除术,效果满意。结论：膀胱副神经节瘤是膀胱肿瘤中较少见的一种类型,有其特殊的临床表现与病理特点。     

膀胱副神经节瘤的临床病理分析

目的：探讨膀胱副神经节瘤的临床病理学特征,免疫表型和鉴别诊断。方法回顾4例膀胱副神经节瘤的临床资料,病理学形态和免疫学表型。结果男性2例,女性2例,年龄41-57岁,高血压患者2例,其中1例在排尿时血压明显升高,血尿1例,1例发现尿VMA升高。3例行部分膀胱切除,1例行膀胱镜下肿物切除术。镜下如肾上腺的嗜铬细胞瘤。免疫组化显示瘤细胞CgA、Syn、NES阳性,CK和Vim阴性,支持细胞S-100阳性。结论副神经节瘤除好发于肾上腺外,膀胱也是一个发病部位,但较少见,掌握其特征性的临床表现,典型的镜下特征和免疫表型特点,对以后的诊断有极大的帮助。     

膀胱副神经节瘤6例临床病理分析

目的探讨膀胱副神经节瘤（PUB）的临床病理特征及诊断治疗方法。 方法回顾性分析2007年11月至2018年7月我院收治的6例膀胱副神经节瘤患者临床资料,总结分析膀胱副神经节瘤的临床病理特点和诊治要点。 结果6例患者中男性1例,女性5例,平均年龄42（23～55）岁,所有患者均为无功能性肿瘤,1例临床表现为尿痛,5例为体检发现膀胱占位。CT增强扫描明显强化。肿瘤均为单发,平均直径1.6（0.8～2.2）cm。1例行腹腔镜膀胱部分切除术,1例行膀胱部分切除术,4例行经尿道膀胱肿瘤电切术。平均手术时间72（35～130）min,平均估计出血量22（10～50） ml。术后病理6例均有阳性表达神经内分泌标志物CgA、CD56和Syn,而上皮性标志物如CK、CEA等为阴性。术后随访8个月至11年,1例于经尿道膀胱肿瘤电切术（TURBT）术后5年复发,其余5例均无肿瘤复发或转移。 结论膀胱副神经节瘤临床罕见,可无明显症状,CT增强扫描结合免疫组化检测可明确诊断,经尿道膀胱肿瘤电切术安全、有效,术后需长期随访。     

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...     

膀胱副神经节瘤二例报告并文献复习

目的 总结膀胱副神经节瘤的临床病理学特征及诊疗方法.方法膀胱副神经节瘤2例.例1,女,35岁.临床表现排尿后心慌、头痛,B超及CT检查示膀胱左侧壁肿块2.6 cm×1.5cm,尿3-甲氧-4-羟苦杏仁酸(VMA)35.3～43.3 μmol/24 h(正常值10～35 μmol/24 h).例2,男,22岁.临床表现无痛性全程血尿,B超及CT检查示膀胱右前壁肿块2.5 cm× 2.0 cm,尿VMA 17.9～31.3 μmol/24 h.结果行经尿道膀胱肿瘤电切术1例,行膀胱部分切除术1例.病理诊断为膀胱副神经节瘤.免疫组化CgA、Syn、S-100蛋白和NSE(+).患者术后症状消失,行膀胱镜及B超分别随访3个月、3年肿瘤无复发.结论膀胱副神经节瘤为潜在恶性肿瘤,诊断需结合临床、病理及免疫组化结果判断,膀胱部分切除为主要治疗手段.

Abstract：

Objective To study the clinical,histopathological,immunohistochemical features and the diagnosis and treatment of paraganglioma of urinary bladder. Methods Two cases of paraganglioma of urinary bladder were treated. The first case was a male with painless haematuria. The abdominal ultrasonography and CT scan showed a 2.5 cm× 2.0 cm mass in the right anterior wall of the bladder, and urine vanillylmandelic acid elevated to 17. 9- 31. 3 μmol/24 h (normal range 10- 35 μmol/24 h). The second case was a female who presented with 8 years history of headache and palpitation after voiding. Abdominal ultrasonography and CT scan showed a 2. 6 cm× 1.5 cm mass in the left wall of the bladder, and her urine vanillylmandelic acid was 35.3-43.3 μmol/24 h. Results One patient underwent transurethral resection (TURBT) and the other underwent partial cystectomy.The two cases were diagnosed as bladder paraganglioma by pathological examination. Immunohistochemically, the tumor cells were positive for CgA, Syn, NSE and S-100. No evidence of recurrence was detected during follow-up at 3 months and 3 years. Conclusions Paraganglioma of urinary bladder should be considered as a low grade malignancy. Partial cystectomy should be recommended. The diagnosis depends on clinical symptoms, pathological and immunohistochemical results.     

膀胱副神经节瘤二例报告并文献复习

目的 总结膀胱副神经节瘤的临床特点及诊疗方法。方法 报告2例膀胱副神经节瘤诊治情况,并复习国内外有关文献。结果 2例均为女性,1例为功能性副神经节瘤,予苯苄胺控制血压至正常后行膀胱部分切除术。术后血压正常,排尿无头痛等;1例为非功能性副神经节瘤,经常规术前准备,行经尿道膀胱肿瘤电切术。病理报告为副神经节瘤。术后效果均满意,分别随访2年和1年余,无复发。结论 功能性和非功能性膀胱副神经节瘤各有其特点,病理及免疫组化是确诊的唯一手段,手术治疗是主要治疗方法。     

膀胱副神经节瘤二例报告及文献复习

目的探讨膀胱副神经节瘤的临床病理特征及其诊治方法。 方法回顾性分析2例膀胱副神经节瘤患者的临床资料,分析该病的临床病理特征、诊断及治疗方法,并对相关文献进行回顾和总结。 结果病例1为无功能性肿瘤,体检发现膀胱占位,CT增强扫描明显强化,肿瘤为单发,行经尿道膀胱肿瘤电切术。病例2为功能性肿瘤,主要临床表现为头痛、心悸、排尿后血压一过性升高,MRI增强结节不均匀强化,肿瘤为单发,术前降压扩容后行腹腔镜膀胱部分切除术,术后症状消失。术后病理诊断均为膀胱副神经节瘤。随访9~12个月,均无复发或转移。 结论膀胱副神经节瘤临床罕见,可分为功能性和非功能性,术前需充分检查及准备,术中预防血压波动引起的高血压危象,经尿道膀胱肿瘤电切术及腹腔镜膀胱部分切除术均效果良好,术后需密切随访。     

膀胱副神经节瘤5例报告及文献复习

目的:探讨膀胱副神经节瘤的疾病特点及诊疗方法。方法:回顾性分析2012年3月～2019年1月在我院诊治的5例术后病理诊断为膀胱副神经节瘤患者的临床资料和随访结果。结果:5例患者均顺利完成手术治疗,术后病理组织表现为免疫组化不同程度的表达[嗜铬粒蛋白A(CgA)、突触素(Syn)、CD56等染色标记为阳性]。术后随访6～40个月,平均26.5个月,术前有血尿、头痛、心悸及排尿后血压升高等症状的患者于术后症状消失或逐渐缓解,5例患者术后均无复发或转移。结论:膀胱副神经节瘤在临床上非常罕见,且易误诊漏诊,需根据术后免疫组化进行明确诊断。其可分为功能性和非功能性,主要依据临床特征及内分泌检查进行鉴别。术前应进行定性和定位的辅助检查,目前膀胱副神经节瘤的治疗方案多样,手术治疗仍是首选方案,治疗后应长期规律随访。     

膀胱副神经节瘤1例报告并文献复习

目的提高膀胱副神经节瘤的诊疗水平。方法回顾性分析1例膀胱副神经节瘤患者的临床资料,分析总结该病的临床特征及诊疗经过。患者,男,51岁,排尿后头晕同时血压升高,血儿茶酚胺和尿香草苦杏仁酸明显升高,MRI见膀胱前壁33 mm×27 mm 肿块,膀胱镜检未发现肿瘤。术前考虑膀胱前壁副神经节瘤,完善扩容降压3周以上,行腹腔镜下膀胱部分切除术。结果手术顺利,切除范围包括瘤体及部分膀胱壁,术中生命体征平稳。术后10 d 拔除导尿管,未见排尿时血压升高及头晕。术后病理证实为膀胱副神经节瘤。术后3个月复查,血压正常,盆腔CT未见复发。结论膀胱副神经节瘤术前应做好充分准备,术中预防血压波动导致的高血压危象,腹腔镜下膀胱部分切除术治疗效果满意,术后需定期随访。     

膀胱副神经节瘤7例报告并文献复习

目的:探讨膀胱副神经节瘤(PUB)的疾病特点、病理特征、诊断及治疗方法.方法:收集2007年11月-2019年12月收治7例PUB患者的临床资料,对临床记录、手术记录、病理报告、随访记录等进行了回顾性分析.结果:7例患者中女6例,男1例,术前均无排尿后高血压、心悸、排尿晕厥、血尿等临床表现;治疗方案均采取手术切除肿瘤:...     

Malignant paraganglioma of the urinary bladder. A case report

Paraganglioma (pheochromocytoma) is probably the most fascinating of alltumors as it can present with a wide range of clinical manifestations.Paraganglioma of the urinary bladder is one of the rare tumors andconstitute less than 10% of all bladder tumors. The common presentationof paraganglioma of the urinary bladder is painless haematura, headache,palpitation and anxiety. Malignancy is uncommon and no histological featureis characteristic of malignancy. Invasion of adjacent organs or metastasisto the lymph nodes is the only criteria to suggest malignancy. In thepresence of proven metastasis radical cystectomy with pelviclymphadenectomy is recommended. In the present case the tumor wasarising from anterior wall of the bladder away from the bladder neck andthat in a large capacity bladder it was possible to get a wide margin forpartial cystectomy along with bilateral pelvic lymphadenectomy withoutviolating the oncological principles. At a follow up of two years there is noevidence of recurrence of tumor.     

Malignant non-functional paraganglioma of the bladder presenting with azotemia

Paragangliomas of the urinary bladder are rare tumors representing less than 1% of bladder tumors and are usually benign. Malignant paragangliomas are uncommon and are defined by their clinical behavior rather than the histologic features. We describe a patient with recurrent nonfunctioning paraganglioma of bladder presenting with hematuria and obstructive uropathy due to involvement of ureteroileal anastomoses. Treatment consisted of excision of recurrent lesion involving both lower ureters with the revision of ureteroileal anastomoses. Histology confirmed the presence of nests of tumor cells with abundant eosinophilic cytoplasm. Immunohistochemically the tumor cells were strongly positive for chromogranin A.     

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.     

Hemangiopericytoma arising from the wall of the urinary bladder

Hemangiopericytoma (HPC) arising from within the urinary bladder is exceptionally rare. A 45-year-old man having the symptoms of left groin pain, vague suprapubic discomfort and frequency was admitted to our clinic. Pelvic tomography revealed a tumor in the bladder wall measuring 4 × 3 cm and was not clearly distinct from the lower abdominal wall. Partial cystectomy was performed and the histopathological examination confirmed the hemangiopericytoma. Three thousand rad exterior beam irradiation was performed after operation. Partial cystectomy and adjuvant radiotherapy may be a simple and effective alternative operation for the patient with HPC.     

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.     

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.