儿童嗜铬细胞瘤的诊断与治疗（附6例分析及文献复习）

目的：提高儿童嗜铬细胞瘤的诊断和治疗水平。方法：回顾性分析1998年至２０１１年来6例患儿的临床资料及复习文献。结果：24 h尿香草苦杏仁酸（vanillylmandelic acid,VMA）增高4例;血儿茶酚胺（catecholamine,CA）增高6例;超声、CT、MRI检查均有阳性发现。肾上腺嗜铬细胞瘤4例,肾上腺外嗜铬细胞瘤2例。全部患儿均在全麻下手术成功切除肿瘤,术后病理检查均证实为嗜铬细胞瘤。术后随访1～13年,5例无瘤存活至今,1例复诊发现肿瘤多发转移,家长放弃进一步治疗,2月后死亡。结论：虽然术前充分准备如扩容、控制血压,术中减少对肿瘤的挤压避免术中突然出现恶性高血压、控制术后低血压是至关重要的,但手术切除肿瘤是根治方法;组织病理学鉴别肿瘤良恶性很困难;若儿童伴恶性高血压合并心律失常、心悸、视力障碍或腹痛,需警惕此病。     

意外发现的嗜铬细胞瘤39例临床分析

目的分析意外发现的嗜铬细胞瘤的临床特点。方法回顾性分析2003年至2012年在华中科技大学附属同济医院经手术病理证实的160例嗜铬细胞瘤患者的临床资料,并根据临床症状将患者分为典型症状组、非典型症状组以及意外发现组。结果典型症状组23例,非典型症状组98例;因腰腹部不适或常规体检经影像学检查时意外发现的患者39例。在前两组患者中有不同程度的儿茶酚胺增多的症状,表现为头痛、心悸、出汗等。而在意外发现组中无明显儿茶酚胺增多的症状,24h尿甲氧基羟苦杏仁酸（VMA）及高血压检出率明显低于典型症状组,但高血压发生率仍然高达59％。嗜铬细胞瘤患者的临床症状表现得越典型,24h尿VMA水平及高血压检出率越高。结论意外发现的嗜铬细胞瘤临床症状不明显,高血压为其重要的诊断线索;影像学检查对无症状嗜铬细胞瘤的诊断有较大帮助。     

嗜铬细胞瘤诊治分析（附50例报告）

目的：提高对嗜铬细胞瘤的诊治水平。方法：对50例嗜铬细胞瘤的临床资料进行回顾性分析。结果：本组患肾上腺嗜铬细胞瘤直径在2-11.8cm，内部结构为不均质，有血儿茶酚胺、尿香草基扁桃酸（VMA）增高；术后，39例血压恢复正常。结论：术前明确诊断，严密监控血压及肿瘤切除后充分补液是手术治疗成功的关键。     

肾上腺外嗜铬细胞瘤的诊断及治疗

目的：了解肾上腺外嗜铬细胞瘤临床特点,提高临床诊疗水平。方法：回顾分析我院2001~2008年资料完整、经病理证实的肾上腺外嗜铬细胞瘤5例患者的资料。结果：本组5例患者中,2例为多发肾上腺外嗜铬细胞瘤。手术共切除7个肿瘤,瘤体直径1.5cm~10.0cm,平均7.1cm。术后随访15个月~8年,平均3.8年。本组无肿瘤复发及转移病例,无死亡病例。结论：肾上腺外嗜铬细胞瘤的临床症状复杂多变,定性诊断主要依靠血、尿儿茶酚胺及尿香草扁桃酸（VMA）检查。术前控制血压、补充血容量及纠正心律失常是减少术中、术后并发症,保证手术成功的关键。手术方式以开放手术为主,但在定位诊断明确的前提下可以尝试腹腔镜手术切除。术后需终生随访。     

嗜铬细胞瘤78例临床分析

目的总结嗜铬细胞瘤的临床特点，提高诊断水平。方法对78例嗜铬细胞瘤的文献复习，进行分析。结果肾上腺内肿瘤69例，肾上腺外肿瘤9例。有症状66例，无症状12例，尿VMA或儿茶酚胺升高73例，腹部B超异常47／52例，腹部CT异常61／64例。结论嗜铬细胞瘤非典型的特殊表现需引起重视。儿茶酚胺及其代谢产物检测和影像学检查目前仍是首选诊断方法。     

嗜铬细胞瘤28例临床分析

目的：总结嗜铬细胞瘤的诊断经验,提高其术前治疗水平。方法:回顾性分析嗜铬细胞瘤28例l临床资料和病理检查。结果：临床主症为阵发性高血压、头痛、心悸、多汗124h尿VMA阳性率53．6％；B超定位诊断率85．7％，CT诊断率为92.9％,全部病例均经病理证实。结论：嗜铬细胞瘤术前CT检测率最高，尿VMA检测有一定价值。充分的术前准备是手术成功的关键之一。     

嗜铬细胞瘤28例临床分析

目的：总结嗜铬细胞瘤的诊断经验,提高其术前治疗水平。方法:回顾性分析嗜铬细胞瘤28例l临床资料和病理检查。结果：临床主症为阵发性高血压、头痛、心悸、多汗124h尿VMA阳性率53．6％；B超定位诊断率85．7％，CT诊断率为92.9％,全部病例均经病理证实。结论：嗜铬细胞瘤术前CT检测率最高，尿VMA检测有一定价值。充分的术前准备是手术成功的关键之一。     

膀胱嗜铬细胞瘤的诊断与治疗(附4例报告)

目的：提高对膀胱嗜铬细胞瘤的认识，探讨该病的诊断和治疗。方法：总结4例膀胱嗜铬细胞瘤的临床表现、诊断和治疗经验，并复习相关文献。结果：所有患者均表现为与排尿有关的心悸、出冷汗、头痛等高儿茶酚胺大量释放症状；4例均有血浆和/或24h尿中儿茶酚胺及VMA增高，结合B超、CT、MRI或膀胱镜可发现膀胱壁占位性病变。4例均行膀胱部分切除或肿瘤刻除术，经病理检查确诊，随访未见肿瘤复发。结论：对于排尿后出现的心悸、出冷汗、头痛等症状的患者，应高度怀疑膀胱嗜铬细胞瘤，检测血浆和尿中儿茶酚胺及其代谢产物结合。影响学和膀胱镜检查可明确该病的定性和定位诊断，膀胱部分切除或肿瘤刻除术是有效的治疗方法。     

儿茶酚胺症诊断及治疗体会

目的 总结儿茶酚胺症的诊断治疗经验。方法 报告218例儿茶酚胺症的临床资料，其中嗜铬细胞瘤208例，髓质增生10例。结果 218例均行手术治疗并经病理证实，良性196例（90％），恶性22例（10％）；肿瘤位于肾上腺181例（83％），异位嗜铬细胞瘤26例（12％），肾上腺髓质增生11例（5％）。结论 尿VMA检查是儿茶酚胺症定性诊断的主要依据，B超、CT、MRI检查为定位诊断的主要依据。根本治疗方法为手术切除，充分的术前准备和正确的围手术期处理十分重要。     

55例嗜铬细胞瘤临床分析

目的对嗜铬细胞瘤的诊治进行临床评价和探讨.方法对55例住院嗜铬细胞瘤患者的临床资料和诊治情况进行回顾性总结分析.结果 55例患者经病理证实为嗜铬细胞瘤,良性54例,恶性1例,嗜铬细胞瘤体位于肾上腺者47例(髓质增生2例),异位嗜铬细胞瘤8例.临床均有高血压,其中阵发性高血压35例(63.6%),持续性高血压20例(36.4%),多数病人诉头痛、头晕.51例行尿VMA检查,异常47例(92.1%).B超检查55例,阳性48例(87.3%);CT检查42例,阳性37例(88.1%).3例术后复发,1例经剖腹探查证实原手术区为术后炎症粘连,未见原位生长的嗜铬细胞瘤.结论头昏、头痛是最常见症状;高血压是最常见体征;24h尿VMA检查是儿茶酚胺症定性诊断的最常用指标,B超、CT检查为定位诊断的主要依据.手术切除为根本治疗方法.     

Clinical features and anesthetic management of multiple endocrine neoplasia associated with pheochromocytoma

Objective To investigate clinical features and anesthetic management of multiple endocrine neoplasia(MEN) associated with pheochromocytoma.Methods Medical records of patients who were diagnosed as multiple endocrine neoplasia associated with pheochromocytoma in our hospital from April 177 to April 201 were reviewed retrospectively.The demographic data,clinical presentations,family history,biochemical examination,stype of MEN,sequence of different surgical procedures,anesthetic methods and hemodynamics during surgery were analyzed.Results Thirteen cases of MEN associated with pheochromocytoma were investigated,accounging for 6%(13/213)of the pheochromocytoma patients admitted into our hospital.Nine of the 13 patients presented as type Ⅱa MEN(Sipple syndrome),one as type Ⅱ b MEN,and three as mixed MEN,Four patients with type Ⅱ a MEN had a family history of similar disease.Five patients with other coexisting endocrine disorders first underwent excision of the pheochromocytomas,although only two had hypertensive symptoms at the time of admittance.Seven patients without histories of hypertension received surgical treatment for pheochromocytoma secondly.The excision of pheochromocytoma was performed under general anesthesia in 8 patients and epidural block in 4 patients.Marked hemodynamic fluctuation was recorded in 8 patients.No perioperative death was recorded.Conclusion Pheochromocytoma may be linked to other endocrine disorders during MEN,either as the main clinical presentation or most frequently as an occult tumor.Recognition of this feature of pheochromocytoma is of importance to the improvement of diagnosis and treatment both for pheochromocytoma and MEN.     

嗜铬细胞瘤的外科治疗

目的：探讨嗜铬细胞瘤的外科治疗方法及临床效果。方法：通过外科手术的方法治疗52例嗜铬细胞瘤患者．观察其疗效。结果：术后7例（13．5％）患者发生低血压。病理提示良性嗜铬细胞瘤50例（96．2％），恶性嗜铬细胞瘤2例（3．8％）。出院时血压恢复正常42例（80．8％）。2例恶性嗜铬细胞瘤患者中．5年内均死于肿瘤转移。良性嗜铬细胞瘤者复发3例（5．8％），均复发于肾上腺区。结论：嗜铬细胞瘤的手术效果好。但手术风险大；良好的术前准备、术中及术后的监护可以降低手术死亡率。     

异位嗜铬细胞瘤的诊断与治疗

目的总结异位嗜铬细胞瘤的临床特点,增进对本病的认识,提高诊断和治疗水平。方法回顾性分析天津医科大学总医院1990年至2010年36例经病理证实为异位嗜铬细胞瘤的临床资料。就诊原因：高血压28例,排尿终末血压升高5例,查体发现1例,腹痛就诊2例。36例均检测24h尿香草基扁桃酸（VMA）,VMA异常升高阳性率为88．9％（32／36）。经腹部B超、CT、MRI及^131碘-间位碘代苄胍（^131Ⅰ-MIBG）等检查进行定位。36例均行手术治疗,4例行腹腔镜手术。结果本组异位嗜铬细胞瘤单发34例,多发2例。手术切除肿瘤直径为3．4～18．2cm。术后病理证实均为嗜铬细胞瘤,其中恶性嗜铬细胞瘤9例。结论VMA是异位嗜铬细胞瘤定性诊断的主要依据,^131Ⅰ-MIBG定位、定性准确可靠。充分的术前准备是手术成败的关键。手术切除肿瘤是最佳治疗方法。腹腔镜手术治疗异位嗜铬细胞瘤也成为一种术式选择。恶性异位嗜铬细胞瘤术后可给予^131Ⅰ-MIBG辅助治疗。     

肾上腺肿瘤的影像学表现与病理对照分析

目的：探讨肾上腺肿瘤影像学表现的病理基础及在诊断中的价值。方法：总结71例肾上腺肿瘤患者的临床资料，对照分析肾上腺肿瘤的影像学表现与病理类型间的关系。结果：手术治疗63例，术后病理：嗜铬细胞瘤20例，皮质腺瘤27例，皮质腺癌5例，髓性脂肪瘤4例，囊肿5例，节细胞神经纤维瘤2例。转移癌3例经临床随访证实。嗜铬细胞瘤、皮质腺瘤、髓性脂肪瘤和囊肿的B超、CT及MRI的影像学改变各有其特点。结论：综合分析多种检查的影像学特点对判定肾上腺肿瘤的病理类型有一定的帮助。     

成人肾上腺肿瘤的CT诊断与鉴别诊断

目的:探讨成人肾上腺肿瘤性病变的CT特点,以提高诊断与鉴别诊断的能力。方法:回顾性分析经手术病理或临床证实的74例成人肾上腺肿瘤性病变的CT表现。结果:肾上腺良性肿瘤39例,其中肾上腺腺瘤18例,良性嗜铬细胞瘤3例,肾上腺囊肿7例,肾上腺节细胞神经瘤3例,肾上腺髓脂瘤6例,肾上腺神经鞘瘤2例;恶性肿瘤35例,其中肾上腺皮质癌2例,肾上腺转移瘤31例,恶性嗜铬细胞瘤2例。结论:CT平扫及增强能较好地显示肾上腺肿瘤性病变的内部结构和影像学特点,反映病变的血供情况和病理特征,有助于肾上腺肿瘤的诊断与鉴别诊断。     

多发性内分泌瘤病2A型13例临床分析

目的探讨多发性内分泌瘤病2A型（MEN-2A型）的临床特点和治疗方法。方法回顾性分析1988年至2011年我院收治的13例MEN－2A型患者的临床资料。13例均为嗜铬细胞瘤伴甲状腺髓样癌,无甲状旁腺功能亢进。其中8例甲状腺髓样癌发病早于嗜铬细胞瘤,5例同时伴发嗜铬细胞瘤。均行嗜铬细胞瘤切除,其中9例行双侧肾上腺肿物切除,4例行腹腔镜肾上腺肿物切除术;10例行甲状腺癌根治术,颈部淋巴结清扫,3例行甲状腺单侧叶切除术。结果术后病理检查,肾上腺均为嗜铬细胞瘤,其中恶性倾向3例;甲状腺均为甲状腺髓样癌。13例术后均随访至2012年6月,存活10例,死亡3例,死因均为甲状腺髓样癌远处转移。结论此病罕见,手术是治疗该病的惟一方法,当嗜铬细胞瘤与甲状腺髓样癌同时存在时,首先应切除嗜铬细胞瘤。     

Diagnosis and surgical treatment of multiple endocrine neoplasia

Background Multiple endocrine neoplasia （MEN） is relatively rare. But more patients could be found by detailed examination. We discuss the diagnosis and surgical treatment of MEN. Methods The clinical data of 95 MEN cases were retrospectively analyzed. There were 30 cases of MEN1 including 19 cases from 6 families. The MEN1 gene mutation was detected in 81.48% of cases admitted after 1997. There were 22 cases of primary hyperparathyroidism （PHPT）, 10 cases of enteropanceatic tumor including 9 cases of insulinoma, 15 cases of pituitary adenoma, 9 cases of adrenal adenoma, 2 cases of thymic carcinoid. Two patients had 4 glands involved, 3 patients had 3 glands involved, 16 patients had 2 glands involved, and 6 patients had only one gland involved. Three patients had neither clinical symptoms nor biochemical changes, and was diagnosed by MEN1 gene mutation. Six patients presented with nephrolithasis and 6 patients had impaired pancreatic endocrine function. There were 60 cases of MEN2a and 5 cases of MEN2b. 58 cases of MEN2a belongs to 19 kindreds. All MEN2a patients but one presented RET gene mutation in codon 634, and all MEN2b cases had mutation in codon 918.48 cases of MEN2a had thyroid masses with elevated calcitonin levels. 27 patients had pheochromocytoma including 12 cases of multiple foci and 5 malignancy. 13 patients presented with hyperparathyroidism. 5 MEN2b patients had medullary thyroid carcinoma and mucosal ganglioneuromatosis with Marfanoid. Among them, 3 patients had bilateral pheochromocytoma. Results In MEN1, subtotal parathyroidectomy was performed in 12 patients with PHPT and one patient received parathyroid adenoma enucleation. Insulinomas were enucleated in 4 patients. Two patients underwent thymus tumor extirpation. Total thyroidectomy with bilateral dissection of regional lymph nodes was performed in 16 patients with MEN2a and nodule enucleation was performed in 9 patients. Twenty two MEN2a patients underwent pheochromocytoma enucleation including bilateral adrenal resection in 10 cases. 5 MEN2b patients underwent total thyroidectomy with bilateral lymph node dissection. Among them, 3 cases underwent bilateral adrenal operations. Conclusions MEN varies in symptoms. Germline mutation test is helpful in establishing a diagnosis. Surgical management should be aimed at the improvement of life quality in MEN1 and prevention of the fetal tumors in MEN2.     

Diagnosis and surgical treatment of multiple endocrine neoplasia

Background Multiple endocrine neoplasia (MEN) is relatively rare. But more patients could be found by detailed examination. We discuss the diagnosis and surgical treatment of MEN. Methods The clinical data of 95 MEN cases were retrospectively analyzed. There were 30 cases of MEN1 including 19 cases from 6 families. The MEN1 gene mutation was detected in 81.48% of cases admitted after 1997. There were 22 cases of primary hyperparathyroidism (PHPT), 10 cases of enteropanceatic tumor including 9 cases of insulinoma, 15 cases of pituitary adenoma, 9 cases of adrenal adenoma, 2 cases of thymic carcinoid. Two patients had 4 glands involved, 3 patients had 3 glands involved, 16 patients had 2 glands involved, and 6 patients had only one gland involved. Three patients had neither clinical symptoms nor biochemical changes, and was diagnosed by MEN1 gene mutation. Six patients presented with nephrolithasis and 6 patients had impaired pancreatic endocrine function. There were 60 cases of MEN2a and 5 cases of MEN2b. 58 cases of MEN2a belongs to 19 kindreds. All MEN2a patients but one presented RET gene mutation in codon 634, and all MEN2b cases had mutation in codon 918. 48 cases of MEN2a had thyroid masses with elevated calcitonin levels. 27 patients had pheochromocytoma including 12 cases of multiple loci and 5 malignancy. 13 patients presented with hyperparathyroidism. 5 MEN2b patients had medullary thyroid carcinoma and mucosal ganglioneuromatosis with Marfanoid. Among them, 3 patients had bilateral pheochromocytoma. Results In MEN1, subtotal parathyroidectomy was performed in 12 patients with PHPT and one patient received parathyroid adenoma enucleation. Insulinomas were enucleated in 4 patients. Two patients underwent thymus tumor extirpation. Total thyroidectomy with bilateral dissection of regional lymph nodes was performed in 16 patients with MEN2a and nodule enucleation was performed in 9 patients. Twenty two MEN2a patients underwent pheochromocytoma enucleation including bilateral adrenal resection in 10 cases. 5 MEN2b patients underwent total thyroidectomy with bilateral lymph node dissection. Among them, 3 cases underwent bilateral adrenal operations. Conclusions MEN varies in symptoms. Germline mutation test is helpful in establishing a diagnosis. Surgical management should be aimed at the improvement of life quality in MEN1 and prevention of the fetal tumors in MEN2.     

Diagnosis and surgical treatment of multiple endocrine neoplasia

Background Multiple endocrine neoplasia (MEN) is relatively rare. But more patients could be found by detailed examination. We discuss the diagnosis and surgical treatment of MEN. Methods The clinical data of 95 MEN cases were retrospectively analyzed. There were 30 cases of MEN1 including 19 cases from 6 families. The MEN1 gene mutation was detected in 81.48% of cases admitted after 1997. There were 22 cases of primary hyperparathyroidism (PHPT), 10 cases of enteropanceatic tumor including 9 cases of insulinoma, 15 cases of pituitary adenoma, 9 cases of adrenal adenoma, 2 cases of thymic carcinoid. Two patients had 4 glands involved, 3 patients had 3 glands involved, 16 patients had 2 glands involved, and 6 patients had only one gland involved. Three patients had neither clinical symptoms nor biochemical changes, and was diagnosed by MEN1 gene mutation. Six patients presented with nephrolithasis and 6 patients had impaired pancreatic endocrine function. There were 60 cases of MEN2a and 5 cases of MEN2b. 58 cases of MEN2a belongs to 19 kindreds. All MEN2a patients but one presented RET gene mutation in codon 634, and all MEN2b cases had mutation in codon 918. 48 cases of MEN2a had thyroid masses with elevated calcitonin levels. 27 patients had pheochromocytoma including 12 cases of multiple loci and 5 malignancy. 13 patients presented with hyperparathyroidism. 5 MEN2b patients had medullary thyroid carcinoma and mucosal ganglioneuromatosis with Marfanoid. Among them, 3 patients had bilateral pheochromocytoma. Results In MEN1, subtotal parathyroidectomy was performed in 12 patients with PHPT and one patient received parathyroid adenoma enucleation. Insulinomas were enucleated in 4 patients. Two patients underwent thymus tumor extirpation. Total thyroidectomy with bilateral dissection of regional lymph nodes was performed in 16 patients with MEN2a and nodule enucleation was performed in 9 patients. Twenty two MEN2a patients underwent pheochromocytoma enucleation including bilateral adrenal resection in 10 cases. 5 MEN2b patients underwent total thyroidectomy with bilateral lymph node dissection. Among them, 3 cases underwent bilateral adrenal operations. Conclusions MEN varies in symptoms. Germline mutation test is helpful in establishing a diagnosis. Surgical management should be aimed at the improvement of life quality in MEN1 and prevention of the fetal tumors in MEN2.     

Diagnosis and surgical treatment of multiple endocrine neoplasia

Background Multiple endocrine neoplasia (MEN) is relatively rare. But more patients could be found by detailed examination. We discuss the diagnosis and surgical treatment of MEN. Methods The clinical data of 95 MEN cases were retrospectively analyzed. There were 30 cases of MEN1 including 19 cases from 6 families. The MEN1 gene mutation was detected in 81.48% of cases admitted after 1997. There were 22 cases of primary hyperparathyroidism (PHPT), 10 cases of enteropanceatic tumor including 9 cases of insulinoma, 15 cases of pituitary adenoma, 9 cases of adrenal adenoma, 2 cases of thymic carcinoid. Two patients had 4 glands involved, 3 patients had 3 glands involved, 16 patients had 2 glands involved, and 6 patients had only one gland involved. Three patients had neither clinical symptoms nor biochemical changes, and was diagnosed by MEN1 gene mutation. Six patients presented with nephrolithasis and 6 patients had impaired pancreatic endocrine function. There were 60 cases of MEN2a and 5 cases of MEN2b. 58 cases of MEN2a belongs to 19 kindreds. All MEN2a patients but one presented RET gene mutation in codon 634, and all MEN2b cases had mutation in codon 918. 48 cases of MEN2a had thyroid masses with elevated calcitonin levels. 27 patients had pheochromocytoma including 12 cases of multiple loci and 5 malignancy. 13 patients presented with hyperparathyroidism. 5 MEN2b patients had medullary thyroid carcinoma and mucosal ganglioneuromatosis with Marfanoid. Among them, 3 patients had bilateral pheochromocytoma. Results In MEN1, subtotal parathyroidectomy was performed in 12 patients with PHPT and one patient received parathyroid adenoma enucleation. Insulinomas were enucleated in 4 patients. Two patients underwent thymus tumor extirpation. Total thyroidectomy with bilateral dissection of regional lymph nodes was performed in 16 patients with MEN2a and nodule enucleation was performed in 9 patients. Twenty two MEN2a patients underwent pheochromocytoma enucleation including bilateral adrenal resection in 10 cases. 5 MEN2b patients underwent total thyroidectomy with bilateral lymph node dissection. Among them, 3 cases underwent bilateral adrenal operations. Conclusions MEN varies in symptoms. Germline mutation test is helpful in establishing a diagnosis. Surgical management should be aimed at the improvement of life quality in MEN1 and prevention of the fetal tumors in MEN2.