后腹腔镜肾上腺嗜铬细胞瘤切除术的疗效评估

目的:探讨后腹腔镜肾上腺嗜铬细胞瘤切除术的有效性及安全性。方法:回顾分析2009年1月至2013年12月68例肾上腺嗜铬细胞瘤患者的临床资料,其中33例行开放肾上腺嗜铬细胞瘤切除术(开放组),35例行后腹腔镜肾上腺嗜铬细胞瘤切除术(后腹腔镜组),观察手术相关指标,包括术中是否出现血压剧烈波动、手术时间、术中出血量、引流量、拔除引流管时间、术后住院时间等。结果:68例手术均获成功,后腹腔镜组手术时间、出血量、引流管拔除时间及术中血压剧烈波动例数等明显优于开放组(P<0.05)。结论:后腹腔镜手术具有创伤小、患者痛苦少、康复快等优势,是治疗肾上腺嗜铬细胞瘤安全、有效的术式。     

妊娠合并嗜铬细胞瘤的临床诊治探讨(附4例报告)

目的:探讨妊娠合并嗜铬细胞瘤的临床诊断与治疗。方法:回顾性分析在我院诊治的4例妊娠合并嗜铬细胞瘤患者的临床资料。患者年龄分别为41、28、32和30岁,就诊时间为孕32、12、14和13周,所有患者均表现为妊娠期高血压,伴头痛、心悸、多汗。内分泌检查提示:24h尿儿茶酚胺明显升高。超声和MRI提示肾上腺区肿瘤,其中右侧肾上腺2例,左侧肾上腺1例,双侧肾上腺l例。肿瘤直径4.4~9.1cm,平均6.03cm。结果:1例患者于孕32周在全麻下行剖宫产术,顺利产一健康女婴,同时行腹腔镜右侧肾上腺嗜铬细胞切除术。2例患者选择流产,经充分药物准备后行腹腔镜肾上腺嗜铬细胞瘤切除术。1例选择流产,并拒绝进一步治疗。3例手术患者的术后病理均证实为嗜铬细胞瘤。结论:妊娠期具有典型的高血压,伴头痛、心悸、大汗三联征,应考虑到嗜铬细胞瘤的可能。早期诊断可显著降低母亲及胎儿病死率。治疗应根据妊娠所处的时期及嗜铬细胞瘤的功能状态选择恰当的方式。妊娠中期是较理想的手术治疗时机,腹腔镜手术治疗妊娠期嗜铬细胞瘤安全有效。     

原发性肾上腺节细胞神经瘤的诊治分析

目的：总结肾上腺节细胞瘤的诊治方法。方法：回顾性分析1992年10月～2011年1月诊治19例肾上腺节细胞瘤患者的临床资料,并复习相关文献资料。结果：19例患者中12例经开放手术切除肾上腺肿瘤,7例行腹腔镜肾上腺肿瘤切除术,术后病理均证实为肾上腺节细胞神经瘤,术后随访2个月～14年,因心脏疾病死亡4例,脑出血死亡1例,车祸伤死亡1例,无一例患者因原发性肾上腺节细胞神经瘤死亡,随访期间肿瘤无复发和转移,预后良好。结论：肾上腺节细胞神经瘤为神经源性良性肿瘤,临床主要与静息型嗜铬细胞瘤相鉴别,对伴有疑似嗜铬细胞瘤临床表现的患者,且实验室检查与嗜铬细胞瘤相关的指标有异常时,术前准备需按嗜铬细胞瘤的要求。腹腔镜肿瘤切除可作为一线治疗方法,对于伴有周围血管脏器界限不清的肿瘤,建议行开放手术治疗。     

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。     

肾上腺嗜铬细胞瘤引起库欣综合征一例报告并文献复习

目的探讨肾上腺嗜铬细胞瘤引起库欣综合征的临床诊断和治疗。 方法结合文献复习,回顾性分析2015年12月我院收治的1例右侧肾上腺嗜铬细胞瘤引起库欣综合征患者的资料。患者女性,45岁,因反复头痛半年,加重伴头晕、多尿、消瘦2个月收入我院内分泌科。术前诊断考虑为肾上腺嗜铬细胞瘤引起库欣综合征,同时按库欣综合征和嗜铬细胞瘤做术前准备,行腹膜后入路腹腔镜右侧肾上腺肿瘤切除术。 结果内分泌检查示促肾上腺皮质激素（ACTH）、皮质醇高于检测值上限,且大、小地塞米松抑制试验不受抑制。血儿茶酚胺三项均升高,以肾上腺素为主,可乐定试验不受抑制。甲氧基肾上腺素及甲氧基去甲肾上腺素均升高。胸部CT平扫及PET-CT未发现可疑异位ACTH来源。肾上腺MR示双侧肾上腺内、外支弥漫性增厚,右侧肾上腺内、外侧支之间可见一类圆形肿块,边界清,考虑肾上腺嗜铬细胞瘤可能。予口服甲吡酮抑制皮质醇生成,常规剂量酚苄明作术前准备,行腹膜后入路腹腔镜右侧肾上腺肿瘤切除术。病理提示右侧肾上腺嗜铬细胞瘤,合并肾上腺皮质区带性增生。免疫组化CgA（+）,ACTH（+）。术后ACTH及皮质醇降至正常范围。术后6个月复查提示患者高血压、糖尿病等表现消失。 结论嗜铬细胞瘤引起库欣综合征是一种罕见疾病,手术切除是有效治疗手段,术前需同时按照嗜铬细胞瘤和库欣综合征完善术前准备。     

经腹腹腔镜肾上腺嗜铬细胞瘤切除术51例临床分析

目的观察经腹腹腔镜肾上腺嗜铬细胞瘤切除术的疗效。方法选取2016-06-2018-06间在郑州大学第一附属医院接受经腹腹腔镜肾上腺嗜铬细胞瘤切除术的51例肾上腺嗜铬细胞瘤患者,对其临床资料进行回顾分析。结果 50例成功完成经腹腹腔镜手术。1例患者术中出现血压波动,经用硝普钠注射液等药物处理,血压恢复平稳后,终止手术操作。手术时间63～235 min,出血量30～400 m L。术中未发生腹腔脏器损伤等严重并发症。随访12个月,临床症状消失,无患者出现肿瘤复发。结论经腹腹腔镜肾上腺嗜铬细胞瘤切除术具有出血量和并发症少等优点,可作为治疗肾上腺嗜铬细胞瘤的首选术式。     

腹膜后腹腔镜肾上腺嗜铬细胞瘤切除术与开放手术患者血压变化的对比研究

目的:对比分析腹膜后腹腔镜与开放肾上腺嗜铬细胞瘤切除术中患者血压的变化,评价腹膜后腹腔镜手术切除肾上腺嗜铬细胞瘤的安全价值.方法:回顾性分析2008年9月至2011年2月为26例患者行单侧腹膜后腹腔镜肾上腺嗜铬细胞瘤手术(腹腔镜组)和19例单侧开放性肾上腺嗜铬细胞瘤手术(开放组)的临床资料,对比两组患者术中血压的变化(...     

妊娠合并嗜铬细胞瘤的诊断与处理

目的 探讨妊娠合并嗜铬细胞瘤的诊断与处理方法.方法 妊娠合并嗜铬细胞瘤病例3例.年龄分别为41、28、32岁,均为妊娠期间出现高血压,并伴有头痛、心悸、多汗等症状,分别于妊娠27周+1,12周+4,14周+3就诊.24 h尿儿茶酚胺明显升高;肿瘤位于右肾上腺2例,双侧肾上腺1例,肿瘤直径4.5～10.0 cm,术前诊断"妊娠合并嗜铬细胞瘤".结果 例1于孕32周行全麻下剖宫产术,顺利分娩一健康女婴,同时行后腹腔镜下右肾上腺肿瘤切除术,切除7.0 cm肿瘤1枚.例2及例3均选择流产.例2行后腹腔镜下右肾上腺肿瘤切除术,切除右肾上腺4.5 cm肿瘤1枚.例3拒绝进一步治疗.例1及例2术后病理均证实为嗜铬细胞瘤.结论 对于妊娠早期出现高血压、典型发作性高血压或伴有头痛、心悸、大汗三联征,分娩后高血压仍未恢复正常的孕妇,应考虑嗜铬细胞瘤;及时诊断可显著降低母亲及胎儿死亡率;处理上应根据妊娠所处的时期及嗜铬细胞瘤的功能状态选择恰当的方式.妊娠中期是较理想的手术治疗时机.腹腔镜手术治疗妊娠嗜铬细胞瘤安全、有效.     

达芬奇机器人与不同入路传统腹腔镜在肾上腺嗜铬细胞瘤切除术中的围手术期比较

目的:探讨3种手术方式在肾上腺嗜铬细胞瘤切除术中的临床应用价值,提高肾上腺嗜铬细胞瘤的治疗水平。方法:回顾性分析2009年1月—2019年12月在我院手术治疗的186例嗜铬细胞瘤患者的临床资料,比较经腹腔、经后腹腔和机器人辅助腹腔镜手术在围术期的各项数据。结果:经后腹腔组在手术时间、术后恢复进食及术后引流液量方面均优于经腹腔组;而在术中血压波动、中转开放率、失血量及术后拔除引流管时间方面,二者无明显差异;经腹机器人手术组在手术时间和切除巨大肿瘤方面优于经腹腔组。结论:经后腹腔行腹腔镜肾上腺嗜铬细胞瘤切除术的手术时间短、患者恢复快,可作为肿瘤体积较小(5 cm)患者优先选择的手术方式;机器人手术因精细度高,操作灵活更适合肿瘤体积较大的复杂性嗜铬细胞瘤患者。     

妊娠合并嗜铬细胞瘤的诊断与处理

目的 探讨妊娠合并嗜铬细胞瘤的诊断与处理方法.方法 妊娠合并嗜铬细胞瘤病例3例.年龄分别为41、28、32岁,均为妊娠期间出现高血压,并伴有头痛、心悸、多汗等症状,分别于妊娠27周+1,12周+4,14周+3就诊.24 h尿儿茶酚胺明显升高;肿瘤位于右肾上腺2例,双侧肾上腺1例,肿瘤直径4.5～10.0 cm,术前诊断"妊娠合并嗜铬细胞瘤".结果 例1于孕32周行全麻下剖宫产术,顺利分娩一健康女婴,同时行后腹腔镜下右肾上腺肿瘤切除术,切除7.0 cm肿瘤1枚.例2及例3均选择流产.例2行后腹腔镜下右肾上腺肿瘤切除术,切除右肾上腺4.5 cm肿瘤1枚.例3拒绝进一步治疗.例1及例2术后病理均证实为嗜铬细胞瘤.结论 对于妊娠早期出现高血压、典型发作性高血压或伴有头痛、心悸、大汗三联征,分娩后高血压仍未恢复正常的孕妇,应考虑嗜铬细胞瘤;及时诊断可显著降低母亲及胎儿死亡率;处理上应根据妊娠所处的时期及嗜铬细胞瘤的功能状态选择恰当的方式.妊娠中期是较理想的手术治疗时机.腹腔镜手术治疗妊娠嗜铬细胞瘤安全、有效.     

2型多发内分泌腺瘤病的诊断及外科治疗

目的探讨2型多发内分泌腺瘤病的临床特点、治疗方法及疗效。方法回顾分析我院1980～2002年诊治的8例2型多发内分泌腺瘤病患者的临床特点、诊断及治疗方法与疗效。结果7例有阵发性高血压,5例最高血压超过200mmHg(1mmHg=0．133kPa),3例查体发现肿瘤。经B超、CT及24h尿儿茶酚胺检查确定诊断,2A型6例,2B型2例;表现为甲状腺髓样癌合并嗜铬细胞瘤伴或不伴甲状旁腺腺瘤或增生(6例),1例伴多发黏膜神经瘤,1例为嗜铬细胞瘤伴有马凡综合征;双侧肾上腺肿瘤7例;分期行肾上腺与甲状腺、甲状旁腺结节切除或次全切除术。术后平均随访9年,效果良好。结论2型多发内分泌腺瘤病的诊断主要依赖相应的内分泌检查及B超和CT检查,手术是主要的治疗手段,当嗜铬细胞瘤与其他肿瘤同时存在时,宜首先切除嗜铬细胞瘤。     

以嗜铬细胞瘤为主要表现的多发性内分泌腺瘤

目的：探讨以嗜铬细胞瘤为主要表现的多发性内分泌腺瘤（MEN）的诊治。方法：3例以嗜铬细胞瘤为主要表现的MEN,均行单侧肾上腺切除术,其中例1 、例2为同胞组妹均诊断为嗜铬细胞瘤并发甲状腺髓样癌,属MRN－Ⅱa型,行甲状腺全切加同侧淋巴结清扫术;例3为嗜铬细胞瘤并发胰岛素瘤,属MEN混合型,同时行嗜铬细胞瘤、胰岛素瘤切除术。结果：3例手术切除效果良好。例1术后6年死于其他疾病,例2术后已生存23年无复发,例3术后血压、血糖、血胰岛素恢复正常,随访6个月无复发。结论：提高对本病的认识,选择适当的内分泌和影像学等检查手段,是早期诊断的关键。MEN中嗜铬细胞并发其他肿;且先行嗜铬细胞瘤切除术,是否全切双侧肾上腺应依具有体情况而定,然后再处理其他肿瘤。     

Adrenalectomy for familial pheochromocytoma in the laparoscopic era

OBJECTIVE: To report the results of treatment of patients with familial pheochromocytomas in the laparoscopic era. SUMMARY BACKGROUND DATA: The optimal surgical management of pheochromocytomas that arise in familial neoplasia syndromes may be complicated by bilateral involvement and associated endocrinopathies. METHODS: Twenty-one patients with familial pheochromocytomas (15 with multiple endocrine neoplasia [MEN] 2A, 4 with MEN 2B, 1 each with von Hippel-Lindau and neurofibromatosis type 1) underwent adrenalectomy between December 1993 and July 2001. Clinical, biochemical, and pathologic data were obtained by retrospective review of perioperative medical records, postoperative biochemical testing, and patient questionnaire. RESULTS: Mean age at diagnosis was 37 +/- 11 years. Twenty of the 21 patients had elevated urine catecholamines, and all had radiographic evidence of an adrenal tumor or tumors. Pheochromocytoma-related symptoms were present in 11 patients (52%). One patient with MEN 2B underwent open adrenalectomy due to previous adrenal surgery and megacolon. Laparoscopic adrenalectomy was attempted in the remaining 20 patients (9 right, 11 left, 2 bilateral). Two patients (9.1%) were converted to open adrenalectomy. Intraoperative hypertensive episodes occurred in 15 patients (71%) and were easily controlled medically. Mean operative time was 216 +/- 57 minutes, mean postoperative length of stay was 3.1 +/- 1.3 days, and mean tumor size was 3.1 +/- 1.0 cm. Minor complications occurred in three patients (14.3%) and major complications in two patients (9.5%). During a mean follow-up of 57 months, a contralateral pheochromocytoma developed in four patients with MEN 2 (33%); three of them underwent adrenalectomy. There have been no long-term complications related to hypertension or adrenalectomy. CONCLUSIONS: This study is the largest series of patients with familial pheochromocytoma undergoing adrenalectomy during the laparoscopic era. The results suggest that the laparoscopic approach is safe and effective for managing unilateral or bilateral adrenal medullary disease in this population.     

Unilateral laparoscopic adrenalectomy followed by contralateral retroperitoneoscopic partial adrenalectomy in a patient with multiple endocrine neoplasia type 2a syndrome

We report the first patient who had bilateral pheochromocytoma associated with multiple endocrine neoplasia type 2a syndrome (MEN 2a) and underwent unilateral laparoscopic adrenalectomy followed by contralateral retroperitoneoscopic partial adrenalectomy 2 years later. The postoperative course was uneventful both times, and the patient was cured of hypertension without any need for steroid replacement. Endoscopic partial adrenalectomy is a minimally invasive procedure for pheochromocytoma with mild symptoms. We believe that this procedure has considerable potential for treating bilateral pheochromocytoma, which is frequently observed in patients with MEN 2a.     

Simultaneous bilateral laparoscopic adrenalectomy: a surgical option for multiple endocrine neoplasia (MEN 2) patients with bilateral pheochromocytomas

Multiple endocrine neoplasia (MEN 2) is a rare disorder. Of this group, 42% develop a pheochromocytoma of which 60% will have bilateral involvement. Although the benefits of unilateral laparoscopic adrenalectomy have been well documented, fewer cases of simultaneous bilateral laparoscopic adrenalectomy have been reported. We present the cases of three patients with MEN 2 who underwent successful simultaneous bilateral laparoscopic adrenalectomy after their initial presentation with bilateral pheochromocytoma. Although the management of bilateral pheochromocytomas has traditionally been approached via open laparotomy or bilateral posterior incisions, the bilateral laparoscopic approach should be considered a viable alternative for patients requiring surgical intervention. Clinical outcomes and complications are similar to open laparotomy. Simultaneous bilateral laparoscopic adrenalectomy is a safe and effective procedure that results in a more rapid recovery and a shorter hospital stay for patients with bilateral pheochromocytomas originating from MEN 2.     

Endoscopic treatment of bilateral pheochromocytoma in MEN 2A syndrome: case report and review of the literature

The benefits of laparoscopic adrenalectomy for single adrenal lesion have been well documented in literature; less experience though has been reported with simultaneous bilateral laparoscopic adrenalectomy. This operation is indicated in case of primary hypercortisolism caused by bilateral adrenocortical hyperfunction, Cushing's disease after failure of pituitary surgery, ectopic adrenocorticotropic hormone (ACTH) production by a tumour inaccessible for surgical intervention, and pheochromocytoma when it occurs bilaterally in case of multiple endocrine neoplasia type 2A and 2B. Different laparoscopic approaches have been described to perform this operation, such as the "anterior" approach (transperitoneal), the "lateral" approach (transperitoneal and retroperitoneal), and the "posterior" approach (retroperitoneal). We report a case of bilateral laparoscopic adrenalectomy in a 33 years old female affected with bilateral pheochromocytoma due to multiple endocrine neoplasia type 2A treated with a bilateral transperitoneal laparoscopic adrenalectomy and disease free after 18 months follow-up.     

Laparoscopic resection of periadrenal paraganglioma in a patient with multiple endocrine neoplasia type 2A

We report here a case of recurrent pheochromocytoma successfully managed by laparoscopic surgery in a patient with multiple endocrine neoplasia type 2A. A 25-year-old man presented with the contralateral adrenal mass after earlier right adrenalectomy. For the preoperative diagnosis of left adrenal pheochromocytoma, adrenal sparing surgery was considered. From the intraoperative laparoscopic view, we found that the tumor originated in periadrenal sympathetic ganglia, and laparoscopic excision of paraganglioma was successfully performed that preserved the integrity of normal adrenal gland. Extra-adrenal pheochromocytoma is rather rare in MEN 2A and it is important to identify precise intraoperative localization of pheochromocytoma with laparoscopic surgery.     

Pheochromocytoma in multiple endocrine neoplasia type II: an example of the two-hit theory of neoplasia

Six kindreds in which pheochromocytomas were present as manifestations of the autosomal dominantly inherited multiple endocrine neoplasia (MEN) type II were studied. The patients underwent bilateral total adrenalectomy with the finding that the pheochromocytomas were bilateral, multifocal, and associated with distinct medullary hyperplasia and reduction in the normal corticomedullary ratio-- features not usually seen in patients with sporadic pheochromocytoma. These findings were exemplified in recent cases of a 34-year-old woman and a 40-year-old man who both had undergone total thyroidectomy for medullary carcinoma of the thyroid. Diagnoses of pheochromocytoma were made by catecholamine studies, computerized tomography, and 131I meta-iodobenzylguanidine (MIBG) scan. Bilateral adrenalectomy was performed with the finding of multiple bilateral pheochromocytomas and adrenal medullary hyperplasia. As in hereditary medullary carcinoma of the thyroid, the histologic findings in pheochromocytomas of the MEN II syndrome are consistent with Knudson's two-mutational-event theory for the initiation of neoplasia, with adrenal medullary hyperplasia representing the manifestation of the first or genetic mutational event and being present invariably in the hereditary cases.     

Management of pheochromocytomas in patients with multiple endocrine neoplasia type 2 syndromes.

OBJECTIVE: The authors sought to determine the optimal surgical management of pheochromocytomas that develop in patients with multiple endocrine neoplasia (MEN) type 2 syndromes. SUMMARY BACKGROUND DATA: The performance of empirical bilateral adrenalectomy in patients with MEN 2A or MEN 2B, whether or not they have bilateral pheochromocytomas, is controversial. METHODS: The results of unilateral or bilateral adrenalectomy were studied in 58 patients (49 with MEN 2A and 9 with MEN 2B). Recurrence of disease was evaluated by measuring 24-hour urinary excretion rates of catecholamines and metabolites and by computed tomography (CT) scanning. RESULTS: The mean postoperative follow-up was 9.40 years. There was no operative mortality and malignant or extra-adrenal pheochromocytomas were not present. Twenty-three patients with a unilateral pheochromocytoma and a macroscopically normal contralateral gland underwent unilateral adrenalectomy. A pheochromocytoma developed in the remaining gland a mean of 11.87 years after the primary adrenalectomy in 12 (52%) patients. Conversely, 11 (48%) patients did not develop pheochromocytoma during a mean interval of 5.18 years. In the interval after unilateral adrenalectomy, no patient experienced hypertensive crises or other complications related to an undiagnosed pheochromocytoma. Ten (23%) of 43 patients having both adrenal glands removed (either at a single operation or sequentially) experienced at least one episode of acute adrenal insufficiency or Addisonian crisis, including one patient who died during a bout of influenza. CONCLUSIONS: Based on these data, the treatment of choice for patients with MEN 2A or MEN 2B and a unilateral pheochromocytoma is resection of only the involved gland. Substantial morbidity and significant mortality are associated with the Addisonian state after bilateral adrenalectomy.     

Estimated risk of pheochromocytoma recurrence after adrenal-sparing surgery in patients with multiple endocrine neoplasia type 2A

HYPOTHESIS: Adrenal-sparing adrenalectomy is considered the treatment of choice for hereditary bilateral pheochromocytoma in patients with multiple endocrine neoplasia type 2A (MEN 2A). DESIGN: Retrospective analysis of prospectively documented data with a mean +/- SD follow-up of 81.5 +/- 85.3 months. The PubMed database was searched for articles published between 1975 and 2004 to identify published series and/or case reports. SETTING: University hospital referral center. PATIENTS: In 17 (22%) of 77 patients with various mutations of the RET proto-oncogene, unilateral (n = 12) or bilateral (n = 5) pheochromocytomas were documented at the time of diagnosis or during the course of MEN 2A. Adrenal-sparing surgery was performed in 13 patients (group 1), synchronous bilateral total adrenalectomy in 4 patients (group 2A), and metachronous bilateral total adrenalectomy in 5 patients after adrenal-sparing adrenalectomy (group 2B). MAIN OUTCOME MEASURES: Measurement of 24-hour urinary catecholamine levels (noradrenaline, adrenaline, and dopamine) and, in case of high catecholamine levels, imaging studies to localize the tumors in 1 or both adrenal glands to determine the size and exclude extra-adrenal tumors and distant metastasis. RESULTS: The mean+/-SD estimated 5- and 10-year cumulative risk of developing recurrence in both groups was 38.5% +/- 15.7%. Five (38%) of 13 patients in group 1 developed recurrence in the contralateral gland. Two (22%) of 9 patients in groups 2A and 2B developed several episodes of an addisonian crisis, 1 of whom died. CONCLUSIONS: Substantial morbidity and mortality are associated with addisonian crisis after bilateral adrenalectomy. Adrenal-sparing adrenalectomy and close monitoring of the remnant may be the treatment of choice for hereditary bilateral pheochromocytoma in MEN 2A, since overall recurrence is low.