腹膜后化学感受器瘤诊断和治疗

腹膜后化学感受器瘤较为少见,占全身化学感受器瘤的30.4％,占腹膜后肿瘤的7.4～10.6％.由于无特征性临床表现,术前诊断困难,误诊率很高.为此本文讨论此病的诊断和治疗. 腹膜后化学感受器瘤来源于腹膜后交感神经节中或周围的嗜铬细胞,故又称为腹膜后肾上腺外嗜铬细胞瘤或神经节瘤.1980年世界卫生组织将副神经节瘤分为嗜铬细胞瘤,交感性副神经节瘤及副交感性副神经节瘤.非嗜铬性副神经节瘤系指无高血压症状的交     

腹膜后副神经节瘤一例报道

副神经节瘤(paraganglioma,PGL)又称异位嗜铬细胞瘤或肾上腺外嗜铬细胞瘤,是一种少见的神经内分泌肿瘤,起源于神经嵴细胞,可发生于头颈部、胸部、腹部、盆腔[1-2].腹膜后副神经节瘤约占所有副神经节瘤的10％,相对于其他部位的副神经节瘤而言,其少见且具有更高的转移倾向.我们收治腹膜后副神经节瘤1例,现报道如...     

手术治疗对嗜铬细胞瘤/副神经节瘤患者血糖影响的研究

目的:对嗜铬细胞瘤/副神经节瘤患者手术前后血糖变化进行研究分析,探讨嗜铬细胞瘤/副神经节瘤与患者高血糖之间的关系,促进围手术期安全。方法:对2014年1月~2015年12月我院收治的136例嗜铬细胞瘤/副神经节瘤患者做回顾性分析,全部患者均通过定性及定位检查,诊断嗜铬细胞瘤或副神经节瘤明确,16例患者因各种原因未接受手术治疗,120例接受手术的患者术后病理证实为嗜铬细胞瘤或副神经节瘤。结果:120例患者中65例诊断为继发性高血糖,其中糖尿病者25例,糖耐量减低者40例;65例患者中59例术后血糖不同程度下降,其中45例降至正常。结论:继发性血糖升高在嗜铬细胞瘤或副神经节瘤患者中常见,手术治疗后绝大多数患者血糖显著下降,积极的手术治疗有助于此类患者血糖恢复正常。     

肾上腺外腹膜后嗜铬细胞瘤(附六例报告)

嗜铬细胞瘤(pheochromocytoma,PHOE)是指发生在副神经节嗜铬细胞的产生儿茶酚胺(catecholamine,CA)的神经内分泌肿瘤,起源于肾上腺外的副神经节肿瘤被称为副神经节瘤或肾上腺外PHOE.肾上腺外PHOE约占PHOE的17％ ～24％,恶性者也较多,约占15％ ～ 35％[1].自2000年3月至2007年12月,我们共收治肾上腺外腹膜后PHOE 6例,均经手术切除、病理诊断证实,现就有关问题作一讨论.     

腹腔镜嗜铬细胞瘤切除术围手术期的处理

2004年世界卫生组织（WHO）将嗜铬细胞瘤定义为来源于肾上腺髓质产生儿茶酚胺的嗜铬细胞肿瘤;将交感神经和副交感神经节来源者定义为肾上腺外副神经节瘤。目前比较统一的认识是嗜铬细胞瘤特指起源于肾上腺髓质的嗜铬细胞肿瘤,而传统概念上10％发生在肾上腺外或异位的嗜铬细胞瘤统称为副神经节瘤。约95％以上的副神经节瘤位于腹腔和盆腔,常见部位为腹主动脉旁、肾门附近、     

肾上腺外嗜铬细胞瘤的诊断和治疗

广义的嗜铬细胞瘤（pheochromocytoma，PHOE）是指发生在副神经节嗜铬细胞的产生儿茶酚胺（catecholamine，CA）的神经内分泌肿瘤，但是一般只有起源于肾上腺髓质的副神经节肿瘤被称为PHOE，而起源于肾上腺外的副神经节肿瘤被称为副神经节瘤或肾上腺外PHOEE。     

原发性腹膜后肾上腺外嗜铬细胞瘤的诊断与治疗探讨（附5例报告）

目的　探讨原发性腹膜后肾上腺外嗜铬细胞瘤的诊断和治疗经验。方法　回顾性分析5例原发性腹膜后肾上腺外嗜铬细胞瘤诊治经过。均行手术治疗 ,4例术中切除瘤体 ,并安置银夹 ,其中 1例在美蓝注入瘤体血管染色指导下手术 ,1例 2次手术。结果　 1例术前诊断不明确 ,准备不充分 ,术中死亡。后 4例术前诊断明确 ,准备充分 ,术中血压稳定 ,完整切除肿瘤 ,其中美蓝染色指导下 1例手术时间缩短 ,出血少。随访中 ,1例无症状患者行CT检查时于银夹标记部位发现肿瘤复发 ,及时 2次手术 ,病理提示恶性。结论　重视原发性腹膜后肾上腺外嗜铬细胞瘤的术前定性、定位诊断 ,术中美蓝注入瘤体血管染色能指导手术 ,安置银夹标记值得采用和推广。     

机器人辅助腹腔镜术治疗压迫大血管的巨大嗜铬细胞瘤和副神经节瘤6例报告

目的:评价机器人辅助腹腔镜术治疗压迫大血管的嗜铬细胞瘤和副神经节瘤的可行性和安全性。方法:回顾性分析2012年5月~2013年10月间所行4例机器人辅助腹腔镜肾上腺嗜铬细胞瘤切除术和2例机器人辅助腹腔镜副神经节瘤切除术患者的临床资料:所有患者术前均通过内分泌检查和影像学检查定性定位。手术采取侧卧位经腹腔途径。通过记录患者的人口学数据、肿瘤大小、手术时间(operating time,OT)、失血量(estimated blood loss,EBL)、术中血压波动次数(intraoperative blood fluctuation,IBF)、住院天数(hospital stay,HS)及术后内分泌随访情况来分析手术效果。结果:所有手术均成功完成,中位肿瘤直径、OT、EBL、IBF和HS分别为73mm、102.5min、120ml、3次和4.5天。术后1个月随访时,所有患者术前升高的血变肾上腺素(metanephrine,MN)或血变去甲肾上腺素(normetanephrine,NMN)均恢复正常。结论:采用机器人辅助腹腔镜手术治疗压迫大血管的肾上腺嗜铬细胞瘤或副神经节瘤是安全可行的。     

腰椎椎管内副神经节瘤一例

副神经节瘤是指体内散在分布的、与交感或副交感神经有联系的嗜铬或非嗜铬的神经上皮样细胞团,是一类少见的软组织神经内分泌肿瘤,属APUD瘤,起源于神经嵴细胞[1]。1908年Alezai’S和Pey-ron首先报道了一组副神经节瘤病例。1912年Pick建议将肾上腺内嗜铬细胞瘤命名为嗜铬细胞瘤(pheochromocytoma),而肾上腺外嗜铬性肿瘤称为     

双侧肾上腺肿瘤的诊治

目的 探讨双侧肾上腺肿瘤诊断和治疗。方法 分别对17例嗜铬细胞和2例原发性醛固醇增多症的临床特征、定性和定位检查及手术径路进行分析。结果 17例双侧肾上腺嗜铬细胞瘤在临床特征、生化、内分泌检查和术前准备方面与单侧或肾上腺外嗜铬细胞瘤类似,术前应注意合并肾上腺外嗜铬细胞瘤可能。经腹切口有利于一期切除双侧肾上腺肿瘤。结论 对双侧肾上腺肿瘤应注意定性分析,I^131-MIBG和NP59有益于肾上腺髓质和皮质肿瘤的鉴别诊断,双侧肋缘下弧形切口有利于切除双侧肾上腺肿瘤。对小的、无功能肾上腺肿瘤应予以随访。     

腹腔镜下切除无症状、生化正常的肾上腺嗜铬细胞瘤12例报告

目的:探讨无症状、生化检查正常的肾上腺嗜铬细胞瘤的诊治原则。方法:回顾分析12例无症状、生化检查正常的肾上腺嗜铬细胞瘤患者的临床资料,术前口服酚苄明1～2周,充分扩容,行腹腔镜肾上腺肿瘤切除术,其中经腰2例,经腹10例。结果:12例患者术中挤压肿瘤时血压均波动明显,手术一期完成,未发生大出血、心脑血管意外等严重并发症,无一例中转开放手术。术后病理均诊断为肾上腺嗜铬细胞瘤。随访6～36个月,血压正常,无复发。结论:无症状、生化检查正常的肾上腺肿瘤临床多见,术前按嗜铬细胞瘤进行准备,充分扩容后行腹腔镜手术是安全、有效的。     

原发性腹膜后副神经节瘤14例报告并文献复习

目的：探讨原发性腹膜后副神经节瘤的临床和病理特点、诊治方法及预后。方法：回顾性分析14例病理检查证实为腹膜后副神经节瘤患者的临床资料：男8例，女6例，年龄14～66岁。有高血压症状9例，其中尿香草扁桃酸（VMA）升高8例，尿儿茶酚胺升高9例。14例均接受手术治疗。术后随访13个月～5年。结果：术中见肿瘤位于肾门区4例，肾上极区5例，肾下极区2例，肾上腺前外上方3例。13例手术完全切除肿瘤，1例伴肝转移者行肿瘤大部切除及肝尾叶切除。病理检查诊断为良性肿瘤13例，恶性肿瘤1例。术后随访12例，9例术前高血压者有7例血压恢复正常；9例无瘤生存，1例复发，2例死亡。结论：手术完全切除肿瘤是腹膜后副神经节瘤最有效的治疗方法，充分的术前准备和围手术期处理是手术成功的关键。术后生存时间取决于肿瘤是否发生淋巴结或远处转移，复发病例可再次手术切除。     

Case of recurrent retroperitoneal malignant paraganglioma 25 years after surgery

A 41-year-old man was referred to our hospital for a left renal mass detected by screening computed tomography for hypertension. He had undergone surgery for left pheochromocytoma at 16 years of age in 1981, and the last follow-up was in 1986. Although a 131I-metaiodebenzylguanidine (MIBG) scintigram did not show abnormal uptake, elevated levels of serum cathecholamines were demonstrated. The left renal mass positioned in the upper pole and inside of the kidney had invaded the pancreas, colon and spleen. Surgical resection was done under a diagnosis of recurrent pheochromocytoma, and the tumor was removed with other organs, including normal left adrenal gland that was confirmed histologically. Histologically and immunohistochemically, the resectioned tumor was invasive malignant paraganglioma and diagnosed as the local recurrence or metastasis of previously resectioned retroperitoneal paraganglioma, which was believed to have grown slowly.     

Safe retroperitoneal endoscopic resection of pheochromocytomas

Although endoscopic adrenalectomy is advocated for small adrenocortical tumors, questions remain about the safety of endoscopic retroperitoneal resection of pheochromocytomas. In this study we evaluated the outcome of retroperitoneal endoscopic adrenalectomy for pheochromocytoma. Between June 1995 and September 1999 we performed 18 retroperitoneal endoscopic adrenalectomies for a pheochromocytoma or paraganglioma. All patients received adequate alpha-adrenergic blockade. The adrenal vein was ligated at the end of the procedure. Operative blood pressure values were recorded and evaluated. Altogether 15 patients (11 women, 4 men; mean age 47.2 years) were operated on for 17 pheochromocytomas and 1 extraadrenal tumor (4 right, 11 left, 3 bilateral). One female patient was operated on at 13 weeks' gestation. Hypertensive episodes at operation were seen in 4 (26.7%) patients, and tachycardia occurred in 5 (33%). Hemodynamic changes could be corrected in all cases using simple measures without morbidity or detrimental effects. The mean operating time was 125 minutes (80-180 minutes), and the conversion rate was 5.6% (1/18). The median hospital stay was 5 days (3-28 days). Morbidity was 20% (3/15). Endoscopic retroperitoneal adrenalectomy for pheochromocytoma is safe and effective, and it is associated with limited morbidity.     

Alterations in Plasma Norepinephrine Concentration During Surgical Resection of Pheochromocytoma

Using a sensitive and specific radioenzymatic assay, the plasma norepinephrine (NE) concentration was measured in seven patients with pheochromocytoma, one patient with bilateral adrenal medullary hyperplasia, one patient with a retroperitoneal paraganglioma, and two patients undergoing bilateral adrenalectomies for palliation of metastatic breast carcinoma. Surgical manipulation of the pheochromocytomas resulted in striking increases in plasma NE concentration with concomitant increases in blood pressure. There were either small changes or no changes in the patients' plasma NE and blood pressure during resection of the normal adrenal glands, the adrenal glands with medullary hyperplasia, or the retroperitoneal paraganglioma. Plasma dopamine-beta-hydroxylase (DBH) was measured in one patient with pheochromocytomas and the patient with medullary hyperplasia. There was no change in plasma DBH in either patient, supporting the concept that exocytosis is not the primary mechanism for catecholamine secretion from pheochromocytomas. It was also noted that enflurane is an excellent general anesthetic for the resection of pheochromocytomas, and that sodium nitroprusside (rather than phentolamine) may be the agent of choice for the management of the hypertensive episodes that occur during surgical manipulation of pheochromocytomas.     

原发性腹膜后副神经节瘤5例报告并文献复习

目的：探讨腹膜后副神经节瘤的临床和病理特点、诊治方法及预后.方法回顾性分析2013年3月至2015年7月收治的5例腹膜后副神经节瘤患者的临床资料.其中男3例,女2例,年龄32~68岁,平均46岁;有高血压症状者3例,无症状体检发现者2例.5例均接受手术治疗,术后随访6个月~3年.结果5例患者均在腹腔镜下手术完整切除肿瘤,术中见肿瘤位于肾上极区3例,肾门区1例,肾下极区1例.术后病理证实为副神经节瘤.4例患者随访期间均未见复发与转移.2例高血压患者术后血压下降但未降至正常值范围,患者口服降压药辅助降压.1例术后1．5年肿瘤复发再次行手术切除瘤体.结论腹膜后副神经节瘤临床罕见,尽早手术切除是其最有效的治疗方法,术中应尽可能的完整切除肿瘤组织,避免肿瘤组织的残留,预后主要取决于肿瘤是否复发与转移.     

Pheochromocytoma manifested by traumatic adrenal hemorrhage

The presentation of pheochromocytoma is quite variable. We report a case of previously undiscovered pheochromocytoma which was manifested by traumatic retroperitoneal hemorrhage. A 79-year-old man fell from his bicycle and was admitted to the emergency room complaining of right flank pain. Enhanced computed tomography (CT) revealed hemorrhage around the right adrenal gland. Since the plasma catecholamine levels were elevated, we suspected the presence of pheochromocytoma. After absorption of the hematoma, the tumor appeared clearly. The diagnosis of pheochromocytoma was confirmed through urine catecholamine testing and 131I-MIBG scintigraphy. Six months after the injury, the tumor was surgically resected. Traumatic hemorrhage of pheochromocytoma is extremely rare; only 3 cases have been reported in the literature.     

后腹腔镜手术切除肾上腺肿瘤(附80例报告)

目的:探讨应用后腹腔镜术切除肾上腺肿瘤的疗效和安全性.方法:应用后腹腔镜手术切除肾上腺肿瘤80例:功能性肾上腺肿瘤56例,非功能性肾上腺肿瘤24例.用自制的气囊扩张后腹腔,腰部3个Trocar穿刺入路,超声刀将肿瘤切除,用自制的标本袋取出肿瘤.结果:80例中73例完全由腹腔镜手术完成,7例因术中粘连、出血等改开放手术.术中出血量10～500 ml,平均70 ml.患者均未输血.76例随访3～48个月,平均8个月,未见肿瘤复发和转移,血电解质、儿茶酚胺、醛固酮、皮质醇等肾上腺内分泌检查均恢复正常;但12例仍有高血压.结论:应用后腹腔镜术切除肾上腺肿瘤安全、创伤小、恢复快,对肾上腺恶性肿瘤主张行开放手术.     

A case of vesical paraganglioma with intrapelvic multiplicity

A 35-year-old woman, who had a past history of hypertension due to paraganglioma of the urinary bladder and in the pelvis, was referred to us 12 years after the initial diagnosis of paraganglioma. Before coming to us and during the follow-up after enucleation of myoma uteri, she was again diagnosed as having a bladder tumor by a gynecologist. Cystoscopy revealed a non-papillary broad-based tumor, measuring 2 cm in diameter, on the trigone of the urinary bladder. Magnetic resonance imaging also showed two solid tumors, each measuring 2 cm in diameter, on the bilateral peri-vesical sides in the pelvis. Endocrinologic examination disclosed increased levels of serum and urinary catecholamines. Histopathologic examination revealed that the bladder tumor specimen obtained by transurethral resection was paraganglioma. She underwent partial cystectomy and resection of these intrapelvic tumors via a retroperitoneal approach. These tumors were histopathologically diagnosed as multiple paragangliomas. She has been followed up for 10 months after operation without any evidence of recurrence. Herein, we report this rare case of vesical paraganglioma and present a brief review of the relevant literature.