膀胱副神经节瘤诊治特点分析

摘要

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.%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.