罕见表现的肾上腺嗜铬细胞瘤1例报告

2007年6月本院收治1例临床罕见特殊表现的静止型肾上腺嗜铬细胞瘤,现报告如下.     

肾上腺外嗜铬细胞瘤34例报告

目的 总结肾上腺外嗜铬细胞瘤的诊治经验.方法回顾性分析34例肾上腺外嗜铬细胞瘤患者的临床资料.就诊原因:高血压27例,血压170～230/110～155 mm Hg(1 mm Hg=0.133 kPa),病程3个月～15年;腹部疼痛10例,病程5个月～6年;间歇性肉眼血尿2例,病程1周和3个月.34例实验室检查,血、尿儿茶酚胺升高20例,尿3-甲氧-4-羟苦杏仁酸升高24例.B超检查34例,肿瘤准确定位31例,阳性率91.2%.CT扫描25例,24例准确定位,阳性率96.0%.MRI检查6例,均准确定位.15例经腹、3例经耻骨上、16例经腰手术,其中32例完整切除肿瘤,1例行包膜下摘除术,1例行术中活检.结果 肿瘤位于肾门旁12例、左肾下极2例、下腔静脉后方1例、腹主动脉和下腔静脉之间3例、腹主动脉前方肠系膜根部2例、右髂总动脉前方1例、肝门部1例、胰腺后方1例、膀胱内2例、降结肠旁1例、多发肿瘤8例(2～3枚).良性嗜铬细胞瘤22例,恶性12例.恶性嗜铬细胞瘤患者中肿瘤单发7例,多发5例.手术切除瘤体直径2～15 cm.术后30例随访6个月～13年.术前27例有高血压症状者,22例血压于术后3 d～6个月恢复正常,5例仍有高血压者中3例舒张压恢复正常,收缩压波动于150～170 mm Hg;2例血压较术前稍有下降.1例膀胱良性嗜铬细胞瘤患者术后6个月肿瘤复发,行膀胱全切.12例恶性嗜铬细胞瘤患者中9例获随访,术后1年内均复发、转移,其中2例为术中未能完整切除肿瘤者,6例术后6个月～3年内死亡,其中3例死于脑溢血,3例死于肿瘤广泛转移;3例经131Ⅰ-间位碘代苄胍(MIBG)治疗后病情稳定,荷瘤生存.结论 肾上腺外嗜铬细胞瘤定位较为困难,易漏诊,CT扫描定位准确可靠.手术切除肿瘤是最佳治疗方法.恶性肾上腺外嗜铬细胞瘤术后可给予131Ⅰ-MIBG治疗.     

肾上腺外嗜铬细胞瘤

目的 提高肾上腺外嗜铬细胞瘤的诊治水平。方法 总结１９５８￣１９９８年收治的肾上腺外嗜铬细胞瘤６６例,占同期嗜铬细胞瘤总数的２３．３％。结果 肾上腺外嗜铬细胞瘤恶性比例高,本组中３３％为恶性嗜铬细胞瘤。结论 １３１Ｉ－ＭＩＢＧ在肾上腺外嗜铬细胞瘤的诊治与随访中有重要意义,肾上腺外嗜铬细胞瘤术后需长期、严密随访。     

胆囊结石并肾上腺嗜铬细胞瘤死亡1例

患者女,31岁.反复右上腹疼痛 1年,加重1d入院.体查:体温38.5℃,血压24/82mmHg.急性痛苦病容,巩膜及皮肤无黄染,右上腹压痛,无反跳痛,莫菲氏征阴性.B超示:胆囊炎、胆囊结石.诊断:胆囊结石,胆囊炎.经术前准备后施行胆囊切除术.术中经过顺利.病员被送回病房后突然出现血压增高至170/130mmHg,给以酚妥拉明降压后血压降低.以后又反复出现高血压及低血压,最后血压降至0,抢救无效,死亡.死后经华西医科大学尸体解剖,病理诊断:右肾上腺嗜细胞瘤.     

肾上腺嗜铬细胞瘤自发破裂出血1例

患者,男,68岁.主因右侧腰腹部疼痛0.5 h急诊入院.发病前0.5 h饮酒后突发右侧腰腹部疼痛,呈持续性,伴大汗,无明显恶心、呕吐、头痛、头晕,无血尿.体检:血压120/100 mmHg(1 mmHg=0.133 kPa).右肾区叩痛明显.拟诊为泌尿系结石.     

不典型肾上腺嗜铬细胞瘤

目的 总结对不典型嗜铬细胞瘤的诊断和治疗效果。方法 无症状肾上腺嗜铬细胞瘤28例，均无高血压病史，内分泌功能检查仅3例尿儿茶酚胺（CA）和尿3-甲基4-羟基苦杏仁酸（VMA）水平轻度增高。21例肿瘤〉3C／T／者增强CT均可见肿瘤周边强化明显，内部有不均匀强化，个别可见偏心液化；7例肿瘤≤3cm者周边与内部强化较均匀一致。根据影像学检查拟诊为不典型嗜铬细胞瘤，2001年以前收治的12例术前未给予降压药和扩容准备；其后收治的16例术前3d每晚给予多沙唑嗪缓释片4mg，术晨加服4mg进行药物准备，不扩容。结果28例均行开放性肾上腺肿瘤切除术，术中血压波动14例，其中术前未服用多沙唑嗪缓释片组3例出现高血压危象，服用多沙唑嗪缓释片组无高血压危象。术后诊断隐匿型嗜铬细胞瘤14例，无功能嗜铬细胞瘤14例。结论 不典型嗜铬细胞瘤特别是隐匿型嗜铬细胞瘤应引起泌尿外科医生足够重视。术前应用缓释型“受体阻滞剂对于保证术中血压的平稳具有很好的应用价值。     

肾上腺嗜铬细胞瘤表现发热或腹泻二例报告

以发热或腹泻为主要临床表现的肾上腺嗜铬细胞瘤少见 ,我们收治 2例 ,现报告如下。例 1,女 ,5 2岁。因发热伴间歇性头痛 1年 ,于 2 0 0 2年 7月 2 3日入院。体温 37.6～ 38.2℃ ,无心悸 ,未测血压 ,曾口服抗生素治疗体温不降。B超示右肾上腺区 6cm× 6cm× 4cm实性不均质肿物。既往健康 ,家族中无类似病史。入院查体温 37.8℃ ,血压 16 0 /10 0mmHg(1mmHg =0 .133kPa) ,查体未见阳性体征。CT示右肾上腺 6cm× 6cm× 4cm肿物 ,增强扫描后呈不均匀强化。血白细胞 10 .0× 10 9/L ,中性粒细胞 0 .6 8,血沉 6 2mm/1h。尿常规及肝肾功能检查无…     

肾上腺嗜铬细胞瘤的腹腔镜手术治疗

手术切除肿瘤是治疗肾上腺嗜铬细胞瘤的唯一有效方法，但手术具有一定的危险性。采用腹腔镜技术切除尚有争议。     

肾上腺组合性嗜铬细胞瘤四例报告及文献复习

目的 探讨肾上腺组合性嗜铬细胞瘤(嗜铬细胞瘤-神经节瘤)的临床特点及治疗方法.方法病理确诊的肾上腺组合性嗜铬细胞瘤患者4例,均为男性,年龄37～62岁.临床表现:高血压3例,其中伴有阵发性心悸1例;血压正常1例.B超、CT检查提示肿瘤均为单发,直径分别2.5、3.8、6.3、15.0 cm.行131Ⅰ-间位碘代苄胍显像检查2例,均为阳性;99Tcm-奥曲肽显像检查2例,阳性1例.24 h尿儿茶酚胺检查,去甲肾上腺素(196.1±92.2)nmol/24 h,肾上腺素(26.6±8.9)nmol/24 h,多巴胺(1957.9±913.5)nmol/24 h,其中轻度升高2例,正常2例.术前α受体阻滞剂准备2～4周.结果 3例行后腹腔镜下肾上腺肿瘤切除手术,1例行开放手术切除.病理报告:肾上腺组合性嗜铬细胞瘤.4例随访15～38个月,平均28个月,肿瘤未见复发、转移;3例高血压患者中2例血压正常.结论 肾上腺组合性嗜铬细胞瘤临床罕见,临床表现与嗜铬细胞瘤相似,儿茶酚胺稍升高,无恶性倾向,手术效果良好.     

静止型嗜铬细胞瘤和非静止型嗜铬细胞瘤的比较

目的：提高对静止型嗜铬细胞瘤的诊断和治疗水平。方法：回顾性分析12例静止型嗜铬细胞瘤和同期105例非静止型嗜铬细胞瘤的临床资料。结果：12例静止型嗜铬细胞瘤患者的血压、尿儿茶酚胺(CA)、尿香草基苦杏仁酸(VMA)均正常或略高于正常值；静止型嗜铬细胞瘤常见于肾上腺偶发瘤及上腹部肿物诊断中，以女性及右侧偏多，肿瘤多呈球形，直径大多＜2cm或＞5cm，瘤体中常见液化坏死，并且面积较大；所有病例均进行手术治疗，其中6例术中血压无上升，3例有轻度上升，3例发生高血压危象，无一例死亡。结论：静止型嗜铬细胞瘤在临床上具有潜在的危险性，应充分认识，术前充分准备，术中及时妥善处理。     

腹腔镜肾上腺嗜铬细胞瘤切除术七例报告

目的探讨腹腔镜手术治疗肾上腺嗜铬细胞瘤的可行性及安全性。方法2003年10月至2007年4月，我院行腹腔镜肾上腺嗜铬细胞瘤切除术7例，肿瘤最大径为3．0～6．5cm，平均5．0cm。6例患者术前均有不同程度的高血压。术前常规行降压、扩容处理。采用后腹腔镜入路6例，经腹入路1例，术中术后严密监测血压变化并给予相应处理。结果5例腹腔镜手术成功，2例中转开放手术。手术时间为70-360min，平均163min，术中出血50-600ml，平均300ml。7例患者术中均出现血压波动，无围手术期患者死亡。病理报告证实为嗜铬细胞瘤。随访7～49个月，5例患者术后2个月内血压恢复正常，1例仍需服用降压药物。结论腹腔镜肾上腺嗜铬细胞瘤切除术可行，疗效确切，充分的术前准备和熟练的腹腔镜操作技术是手术安全的保证。     

肾上腺外嗜铬细胞瘤手术治疗的体会：附10例报告

报告肾上腺外嗜铬细胞瘤１０例，其中纵隔内肿瘤１例，左隔下肿瘤１例，腹主动脉旁肿瘤５例，膀胱肿瘤３例。１例纵隔肿瘤在全麻下开胸摘除肿瘤，其余９例在连续硬膜外麻醉下摘除。结果除１例左隔下肿瘤患者术后３年死于全身衰竭外，其余９例均存活。     

儿童嗜铬细胞瘤8例报告

目的探讨儿童嗜铬细胞瘤的临床特点及诊治方法。方法对1984—2004年诊治的8例儿童嗜铬细胞瘤进行回顾性分析。术前通过临床表现、24h尿儿茶酚胺、^131碘-间碘苄胍全身核素扫描（^131-MIBG扫描）、B超或CT检查明确定性及定位诊断。并用α-受体阻滞荆行术前药物准备。结果8例均行手术切除肿瘤；病理诊断：良性5例，恶性3例。结论儿童嗜铬细胞瘤少见，病情进展快；早期诊断、手术切除是目前惟一可治愈的方法。     

肾上腺组合性嗜铬细胞瘤四例报告及文献复习

Objective To discuss the clinical characteristics and management principles of com-posite pheochromoeytoma of adrenal gland (pheochromocytoma-ganglioneuroma). Methods Four cases of composite tumor of adrenal gland diagnosed pathologically were reviewed. All the cases were male, aged 37 to 62 years;three of them had the history of hypertension, one of them accompanied with paroxysmal palpitation, while one case was asymptomatic. Computerized tomography and ultra-sonography showed single tumor in adrenal, the diameter was about 2.5、3.8、6.3、15cm respectively. Two cases showed positive results in 131Ⅰ-metaiodobenzylguanidine (MIBG) scintigraphy. One case showed positive result in 99Tcm-hydrazinonicotinamide-3trysinoctreotide (HTOC) scintigraphy. Twenty-four hours urinary catecholamine examination revealed the average of norepinephrine, epi-nephrine and dopamine were (196.1±92.2)nmol/24 h, (26.6±8.9)nmol/24 h, (1957.9±913.5) nmol/24 h respectively. Two cases were at a little elevated level and the others with normal. All cases were managed with α-adrenergic receptor blocker preoperatively for 2-4 weeks. Results Tumor re-section was performed in all 4 cases, and 3 cases by retroperitoneal laparoscopy, one by open surgery. All 4 cases were diagnosed as composite pheochromocytoma of adrenal (Pheochromocytoma-gangli-oneuroma) by pathology. All patients had no evidence of recurrence or metastasis during follow-up from 15 to 38 months. Two of three cases with hypertension reverted to normotensive. Conclusions Composite pheochromocytoma of adrenal is rare, difficult to be diagnosed preoperatively. Except mild in eatecholamine secretion, which clinical manifestations are similar to pheochromocytoma. Pheochro-mocytoma-ganglioneuroma is no apparent tendency for aggressive behaviors, the results of surgical management are favorable.     

肾上腺组合性嗜铬细胞瘤四例报告及文献复习

Objective To discuss the clinical characteristics and management principles of com-posite pheochromoeytoma of adrenal gland (pheochromocytoma-ganglioneuroma). Methods Four cases of composite tumor of adrenal gland diagnosed pathologically were reviewed. All the cases were male, aged 37 to 62 years;three of them had the history of hypertension, one of them accompanied with paroxysmal palpitation, while one case was asymptomatic. Computerized tomography and ultra-sonography showed single tumor in adrenal, the diameter was about 2.5、3.8、6.3、15cm respectively. Two cases showed positive results in 131Ⅰ-metaiodobenzylguanidine (MIBG) scintigraphy. One case showed positive result in 99Tcm-hydrazinonicotinamide-3trysinoctreotide (HTOC) scintigraphy. Twenty-four hours urinary catecholamine examination revealed the average of norepinephrine, epi-nephrine and dopamine were (196.1±92.2)nmol/24 h, (26.6±8.9)nmol/24 h, (1957.9±913.5) nmol/24 h respectively. Two cases were at a little elevated level and the others with normal. All cases were managed with α-adrenergic receptor blocker preoperatively for 2-4 weeks. Results Tumor re-section was performed in all 4 cases, and 3 cases by retroperitoneal laparoscopy, one by open surgery. All 4 cases were diagnosed as composite pheochromocytoma of adrenal (Pheochromocytoma-gangli-oneuroma) by pathology. All patients had no evidence of recurrence or metastasis during follow-up from 15 to 38 months. Two of three cases with hypertension reverted to normotensive. Conclusions Composite pheochromocytoma of adrenal is rare, difficult to be diagnosed preoperatively. Except mild in eatecholamine secretion, which clinical manifestations are similar to pheochromocytoma. Pheochro-mocytoma-ganglioneuroma is no apparent tendency for aggressive behaviors, the results of surgical management are favorable.