13例无功能型肾上腺嗜铬细胞瘤临床分析

目的 探讨无功能型肾上腺嗜铬细胞瘤的诊断和治疗方法.方法 回顾性分析13例无功能型肾上腺嗜铬细胞瘤的临床资料.患者均为偶然发现,无高血压和典型的嗜铬细胞瘤临床表现,4例为体检时发现,8例出现腰区、上腹部不适或疼痛,2例出现发热.血浆肾上腺素升高1例(7.7%),去甲肾上腺素升高2例(15.4%).24 h尿儿茶酚胺(CA)及尿香草基扁桃酸(VMA)测定均正常.12例术前给予哌唑嗪降压或全血扩容治疗,经腰第十一肋切口肿瘤切除术12例,腹腔镜肾上腺及肿瘤切除术1例,术中血压平稳无波动.结果 术后病理检查均证实为嗜铬细胞瘤.随访11个月至6.5年,患者血压正常,无肿瘤复发及转移.结论 无功能型肾上腺嗜铬细胞瘤患者无高血压等典型症状,多为体检时发现或表现为上腹部或腰区疼痛,由于其分泌CA及其代谢产物水平较低,为定性诊断带来困难.为防止隐匿功能型嗜铬细胞瘤所致围手术期严重并发症,对于具有典型影像学表现的无功能型肾上腺嗜铬细胞瘤患者术前也应进行常规的降压及扩容治疗,降低手术危险和并发症发生率.     

嗜铬细胞瘤25例临床特点分析

目的总结分析嗜铬细胞瘤的临床特点,提高嗜铬细胞瘤的诊治水平。方法对25经病理证实为嗜铬细胞瘤患者的病例资料进行回顾性分析。结果非意外瘤20例,高血压、血或尿去甲肾上腺素升高为其主要特点,意外瘤5例,临床表现不典型,部分患者存在高血压;腹部B超异常14/15例,CT异常25/25例,所有患者均行手术治疗,良性嗜铬细胞瘤21例,恶性嗜铬细胞瘤4例;肾上腺原发肿瘤24例,肾上腺外肿瘤1例。结论血、尿儿茶酚胺检测、影像学检查仍是嗜铬细胞瘤首选的诊断方法,手术为根本的治疗手段;对于高血压患者,可行腹部B超以提高非典型嗜铬细胞瘤的检出率。     

18例肾上腺外嗜铬细胞瘤的诊断与治疗的研究

目的探讨肾上腺外嗜铬细胞瘤的诊断和治疗方法.方法回顾性分析18例本病患者的临床资料.结果肾上腺外嗜铬细胞瘤18例,占同期嗜铬细胞瘤的26.1%,15例有高血压的症状,无症状3例.手术切除肿瘤17例,无严重手术并发症.结论血和尿儿茶酚胺及其代谢产物测定对本病定性和监测有很高的诊断价值,定位诊断通过B超和CT检查.术前使用肾上腺素能受体阻滞剂控制血压和心率,并扩充血容量,是非常重要的.     

尿儿茶酚胺和3-甲氧基-4-羟基苦杏仁酸联合检测对诊断嗜铬细胞瘤的临床价值

目的 研究尿儿茶酚胺和3-甲氧基-4-羟基苦杏仁酸(VMA)联合检测对诊断嗜铬细胞瘤的临床价值.方法 采用高效液相-电化学法检测60例嗜铬细胞瘤患者和60例健康体检者的尿儿茶酚胺和VMA水平.结果 嗜铬细胞瘤患者的肾上腺素、去甲肾上腺素、多巴胺和VMA水平明显高于健康体检者(P＜0.01),60例嗜铬细胞瘤患者中尿儿茶酚胺升高的有41例(68.3%),VMA升高的有45例(75.0%).结论 用高效液相-电化学法同时测定肾上腺素、去甲肾上腺素和VMA,大大提高了嗜铬细胞瘤的诊断水平.     

胸腔异位嗜铬细胞瘤2例报道(附国内14例文献复习)

目的 探讨胸腔异位嗜铬细胞瘤的临床特点及治疗方法.方法 回顾性分析近期收治的胸腔异位嗜铬细胞瘤患者2例,并总结国内相关文献14例.结果 肿瘤位于左侧纵隔5例,右侧纵隔10例,中纵隔1例.恶性者3例.同时合并肾上腺嗜铬细胞瘤者3例,术前确诊5例.术中挤压肿瘤均出现血压急剧升高,切除肿瘤后血压均急剧下降,其中肺水肿、心脏停跳1例.手术完整切除肿瘤者14例,剖胸探查者2例.结论 胸腔异位嗜铬细胞瘤罕见,术前误诊率高,术中血压波动剧烈,术前充分准备、术中血压的控制是减少并发症的关键.     

肾上腺嗜铬细胞瘤5例的围术期护理

嗜铬细胞瘤90%发生在肾上腺髓质,10%发生在肾上腺外的交感神经细胞,由于肿瘤能自主分泌儿茶酚胺包括肾上腺素、去甲肾上腺素和多巴胺,致心血管系统病变和代谢紊乱,临床主要表现:高血压和代谢紊乱[1].我院1999-08-2003-10共收住5例肾上腺嗜铬细胞瘤患者,通过充分术前准备、手术治疗、精心护理,均获得满意效果,现将护理体会介绍如下.     

不典型嗜铬细胞瘤42例临床分析

目的 提高不典型嗜铬细胞瘤的诊断和治疗水平.方法 回顾性分析42例不典型嗜铬细胞瘤患者的临床资料.结果 42例不典型嗜铬细胞瘤患者均无高血压,头痛、心悸、多汗等典型嗜铬细胞瘤的临床表现.尿香草扁桃酸升高占22％(9/41);影像学表现为回声、密度或信号均匀(直径≤2.0 cm)或不均匀(直径＞ 2.0 cm);13例行开放性手术,3例术中血压明显波动;29例行腹腔镜手术,1例术中血压明显波动.41例成功切除肿瘤,1例因肿瘤与肾、胰、脾等周围组织脏器粘连严重无法切除.所有患者均经术后病理证实为嗜铬细胞瘤.随访1～7年,1例复发,12例失访.结论 不典型嗜铬细胞瘤患者内分泌检查阳性率较低,定性诊断较困难,影像学检查的诊断帮助较大.怀疑嗜铬细胞瘤患者均应按嗜铬细胞瘤行术前准备,不能除外者,术中及术后加强监护.腹腔镜手术在减少术中高血压危象方面效果良好.     

嗜铬细胞瘤/副神经节瘤241例临床分析

目的 讨论嗜铬细胞瘤/副神经节瘤的诊断和治疗.方法 回顾性分析241例嗜铬细胞瘤/副神经节瘤患者的临床表现、生化及影像学检查资料、治疗方法和病理结果.结果 241例患者中,嗜铬细胞瘤223例(92.5％,223/241),副神经节瘤18例(7.5％,18/241).家族性嗜铬细胞瘤20例.有高血压症状193例,有头痛、心悸、多汗三联征130例,24 h尿香草扁桃酸(VMA)升高170例.241例患者中病理回报恶性倾向30例,恶性8例,行后腹腔镜手术治疗175例.随访1～7年,术前有高血压症状者中24例血压较术前明显下降,但仍稍偏高,口服降压药物可维持在正常范围内;其余患者血压恢复正常;8例恶性嗜铬细胞瘤者中复发2例,死亡1例. 结论 嗜铬细胞瘤早期诊断、及时手术治疗是非常必要的.术前药物扩容准备可以降低术中并发症的风险,后腹腔镜手术切除嗜铬细胞瘤已成为当前主要的和首选的治疗方式.     

胸腔异位嗜铬细胞瘤2例报道（附国内14例文献复习）

目的探讨胸腔异位嗜铬细胞瘤的临床特点及治疗方法。方法回顾性分析近期收治的胸腔异位嗜铬细胞瘤患者2例，并总结国内相关文献14例。结果肿瘤位于左侧纵隔5例，右侧纵隔10例，中纵隔1例。恶性者3例。同时合并肾上腺嗜铬细胞瘤者3例，术前确诊5例。术中挤压肿瘤均出现血压急剧升高，切除肿瘤后血压均急剧下降，其中肺水肿、心脏停跳1例。手术完整切除肿瘤者14例，剖胸探查者2例。结论胸腔异位嗜铬细胞瘤罕见，术前误诊率高，术中血压波动剧烈，术前充分准备、术中血压的控制是减少并发症的关键。     

经腹腔入路腹腔镜手术治疗肾上腺嗜铬细胞瘤18例

目的 探讨经腹腔入路腹腔镜下肾上腺嗜铬细胞瘤切除的安全性及手术经验和技巧.方法 对18例肾上腺嗜铬细胞瘤患者行经腹腔入路腹腔镜下嗜铬细胞瘤切除术,并对手术方法进行分析.结果 17例手术成功,中转开放手术1例,结肠损伤1例.手术时间70～ 145(100±25)min;术中失血量30～400(60±25) ml;术后住院时间5～9d,病理报告均为肾上腺嗜铬细胞瘤.全部病例获得随访6～48个月,患者术后血压正常,未见肿瘤复发.结论 经腹腔入路腹腔镜下肾上腺嗜铬细胞瘤切除术具有损伤小、安全有效、术后恢复快和住院时间短等优点,具有良好的临床应用前景,是直径＜6cm的肾上腺嗜铬细胞瘤的首选术式.     

Symptomatic pheochromocytoma with normal urinary catecholamine metabolites

A 61-year old female presented with paroxysmal hypertension and a 4.5cm left adrenal mass on CT scan. Repeated measurements of 24-hour urinary fractionated metanephrines, total catecholamines and vanillylmandelic acid (VMA) were within normal range. A further scintigraphic study with (131)I -metaiodobenzylguanidine ((131)I-MIBG) revealed selective concentration of the radiotracer, corresponding to the CT mass. After adequate preoperative treatment, successful surgical excision of the tumor was performed and the pathological examination confirmed the diagnosis of a cystic pheochromocytoma with a 2cm solid tumor. On reevaluation three months later using (131)I-MIBG, no evidence of remaining or recurrent disease was found. The patient, off any antihypertensive medication, reported mild recurrent hypertension and panic attacks that were adequately controlled with antidepressants. This is a rare case of a symptomatic pheochromocytoma without elevated urine catecholamines and metanephrines. According to the literature, plasma free metanephrines would be the ideal test for biochemical detection of the tumor. However, in the event that they are not available and there is a high clinical suspicion for the presence of pheochromocytoma, as in our patient, we suggest performance of a functional nuclear medicine study, such as (131)I-MIBG, to confirm the clinical diagnosis.     

中医结合不同手术方法治疗肾上腺嗜铬细胞瘤的临床疗效比较

目的：比较在中医治疗的基础上,采用腹腔镜手术与开放手术在治疗肾上腺嗜铬细胞瘤的有效性和安全性。方法选择于2012年2月至2015年2月期间就诊于本院内分泌科的良性肾上腺嗜铬细胞瘤患者64例,按照入组顺序分配至腹腔镜手术组和开放手术组,每组32例。腹腔镜手术组采用腹膜后腹腔镜肾上腺嗜铬细胞瘤切除术,开放手术组采用传统的术式。两组患者在手术治疗后根据患者不同中医症候给予中药方剂巩固治疗。通过比较两组患者手术时间、术中出血量、术中输血例数、术中血压波动情况,术后测量患者肿瘤体积大小,术后引流液体积等指标。术后随访观察患者术后1、3、6、12个月的血压情况及并发症状况等。结果腹腔镜手术组患者手术时间明显长于开放手术组,差别具有统计学意义（P＜0．05）。平均出血量远远少于开放手术组。腹腔镜手术中患者血压升高病例,即收缩压≥200 mmHg（1 mmHg＝0.133 kPa）者仅为2例,约占6．3％,开放手术组患者高血压病例明显较多,占12.5％。而两组患者术中低血压患者数相同（收缩压≤80 mmHg）,均为2例,占6．3％。术后腹腔镜手术组患者腹腔引流液体积明显低于开放手术组,差异具有统计学意义。疼痛缓解较快,且术后血压恢复较快,随访1个月时大多数患者血压降至理想水平。两组患者随访12个月时,均未有患者出现肿瘤复发情况。结论在给予中药治疗的基础上,采用腹腔镜切除肾上腺嗜铬细胞瘤在临床疗效和安全性上较开放手术具有明显的优势。     

后腹腔镜肾上腺肿瘤切除术35例体会

目的:探讨后腹腔镜肾上腺肿瘤切除术的临床治疗效果。方法:回顾性分析我院2007年1月至2011年6月开展的35例后腹腔镜肾上腺肿瘤切除术患者的临床资料,其中皮质腺瘤25例,嗜铬细胞瘤5例,肾上腺髓样脂肪瘤3例,肾上腺囊肿2例。结果:34例手术成功,1例中转开放手术。手术时间50-130min,平均85min;失血量20-250mL,平均50ml;术后住院时间3-8天,平均4.5天。所有患者术中、术后均未输血,无严重并发症发生。结论:后腹腔镜下手术切除肾上腺肿瘤是一种微创、安全、有效的手术方式,具有出血少、恢复快、住院时间短等优点。     

Incidentally discovered adrenal masses

This report describes a retrospective study of 23 patients with incidentally discovered adrenal masses. Two patients with subclinical cortisol secretion developed adrenal insufficiency after removal of benign adenomas. Another patient, who probably harbors an asymptomatic pheochromocytoma, developed a hypertensive crisis when a mass was palpated during aortic vascular surgery. Twelve patients underwent surgery. Seven benign adenomas, an angiomyolipoma, and a cyst were removed. Three patients had malignant masses that include an angiosarcoma, an adrenal adenocarcinoma, and a congenital neuroblastoma. We conclude that hormones, especially cortisol, may be secreted in subclinical amounts by incidentally discovered masses. We recommend a concise laboratory evaluation that includes an overnight dexamethasone suppression test. Based on our interpretation of the literature, we believe masses greater than 3 cm in size should be removed. Patients who do not undergo surgery should have computed tomographic scans repeated for one year and should be reassessed periodically for the development of hormone secretion.     

不同直径肾上腺肿瘤应用后腹腔镜技术治疗的安全性及可行性对比

目的评价后腹腔镜手术切除不同直径肾上腺肿瘤的安全性和可行性。方法回顾性分析2009年1月至2013年6月61例行后腹腔镜肾上腺肿瘤切除术患者的临床资料,根据肿瘤直径分为A组和B组,A组肿瘤直径〉6em（24例）,B组肿瘤直径在3—6em（37例）,比较两组临床疗效。结果所有手术均较顺利完成,A组和B组手术时间[（138±66）min比（106±45）min]、出血量[（324±168）ml比（165±50）m1]比较差异均有统计学意义（P〈0．05）。术中并发症发生率和输血率A组略高于B组,但差异无统计学意义（P〉0．05）。两组术后住院时间[（6．7±1．2）d比（5．4±0．6）d]比较差异无统计学意义（P〉O．05）。A组随访（22±10）个月,19例B超未见局部复发,失访3例,2例发现肾积水或胰腺囊肿;B组随访（24±11）个月,32例B超未见局部复发,失访4例,1例肾功能不全。A组术后病理以髓样脂肪瘤、嗜铬细胞瘤和神经节细胞瘤为主;B组病理以皮脂腺瘤、嗜铬细胞瘤和髓样脂肪瘤为主。结论后腹腔肾上腺肿瘤切除术治疗肾上腺肿瘤效果良好,对于有一定经验的术者,可以安全地经后腹腔途径切除肿瘤直径在6cm以上的复杂肾上腺肿瘤。     

膀胱嗜铬细胞瘤的诊断和治疗

目的了解膀胱嗜铬细胞瘤患者诊治情况,提高膀胱嗜铬细胞瘤的诊断和治疗水平.方法对5例膀胱嗜铬细胞癌的临床资料进行回顾性分析.4例行膀胱部分切除术,1例行膀胱全切除术.结果术前确诊4例,术后5例均经病理证实为膀胱嗜铬细胞瘤.随访10个月～12 a,1例术后16个月转移,死于脑出血,其他4例血压正常,无肿瘤复发.结论血尿、高血压、排尿时典型发作三联征为膀胱嗜铬细胞瘤的主要症状.B超、CT、MRI、131I-MIBG作定位诊断,尿4-羟-3甲氧扁桃酸(VMA)和尿儿茶酚胺作定性诊断.手术切除是最有效的治疗方法,术后密切监测尿儿茶酚胺含量,可以了解有无转移和复发.     

A newly detected mutation of the RET protooncogene in exon 8 as a cause of multiple endocrine neoplasia type 2A

Multiple endocrine neoplasia type 2A (MEN2A) is a syndrome of familial neoplasias characterized by medullary thyroid carcinoma (MTC), pheochromocytoma and hyperplasia of the parathyroid glands. RET protooncogene mutations are responsible for MEN 2A. Mutations in exons 10 or 11 have been identified in more than 96% of patients with MEN 2A. We herein report for the first time a patient with MEN 2A harboring a mutation (Gly(533)Cys) in exon 8. A 66-year old male patient was referred to our department for bilateral adrenal nodules. The patient's family history was remarkable in that his mother had pheochromocytoma. Biochemical evaluation and findings of the magnetic resonance imaging of the adrenals were compatible with the diagnosis of bilateral pheochromocytomas. The patient underwent laparoscopic bilateral adrenalectomy and histological examination confirmed the preoperative diagnosis of pheochromocytoma. Absence of phenotypic characteristics of VHL or NF1 and elevated calcitonin levels both basal and post pentagastrin stimulation, raised the possibility of MEN 2A syndrome. Total thyroidectomy was performed and histological examination showed the presence of MTC. Direct sequencing of exon 8 from the patient's genomic DNA revealed the mutation c.1,597G-->T (Gly533Cys). Although this missense point mutation has been associated with familial MTC (FMTC), to the best of our knowledge mutations in exon 8 have not previously been identified in patients with MEN 2A. In conclusion, in patients with clinical suspicion of MEN 2A syndrome, analysis of RET exon 8 should be considered when the routine evaluation of MEN 2A-associated mutations is negative. Furthermore, patients with FMTC and exon 8 mutations should also be screened for pheochromocytoma.     

Adrenal incidentaloma: a clinical problem related to imaging

Two female patients, 68 and 67 years of age, were referred for right abdominal pain and pyelonephritis, respectively. During the diagnostic work-up, an unsuspected adrenal mass was found in both patients. Hormonal evaluation and imaging showed a benign non-hyperactive functioning adenoma in one patient and a pheochromocytoma in the other. Both patients were successfully treated with endoscopic adrenalectomy. Wider application and improvement of abdominal imaging procedures have caused an increase of incidentally detected adrenal masses, posing a common clinical problem. Typically, a diagnosis can be made on the basis of the characteristic radiological image. The exact nature of the defect is often unclear and further evaluation is required to determine functionality and possible malignancy. An algorithm is presented for the management of adrenal incidentalomas.