Comparative cardiovascular effects of SNP,ATP and phentolamine during norepinephrine-induced hypertension in dogs

There has been no study comparing the advantage and disadvantage of various antihypertensive agents during surgery for pheochromocytomas because the study is difficult in clinical setting. In the present experiments using dogs, after increasing the arterial blood pressure with norepinephrine, we decreased it to the baseline with sodium nitroprusside (SNP), adenosine triphosphate (ATP), or phentolamine (PE) and compared the hemodynamic changes. A hyperdynamic state was found with ATP and with PE, but not with SNP. The norepinephrine-induced pulmonary hypertension could be successfully treated with SNP, but not with ATP or PE. The reason for these differences are thought to be the different vasodilative properties on peripheral arteries and veins. We conclude that agents that dilates the arteries and veins should be used to regulate the arterial pressure during surgical removal of a pheochromocytoma.(Murata K, Takahashi H, Ikeda K: Comparative cardiovascular effects of SNP, ATP and phentolamine during norepinephrine-induced hypertension in dogs. J Anesth 5: 396–403, 1991)     

特殊类型的嗜铬细胞瘤

１９８３年７月～１９９３年６月，收治经手术及病理证实的嗜铬细胞瘤７９例，其中２９例特殊类型或表现者４５例次，肾上腺外１１例，恶性或恶变者１２例，双侧或多发性６例，无功能性及隐匿型４例，复发性８例，家族性２个家庭４例。本组显示恶性或恶变发生呈上升趋势。充分的术前准备使手术更顺利平稳。     

Minimally invasive adrenalectomy for pheochromocytoma during pregnancy

Pheochromocytoma during pregnancy is a very rare condition; fewer than 200 cases have been reported in the literature. We present the case of a 24-year-old pregnant woman found to have a pheochromocytoma during investigation of abdominal pain. This is the second reported case of laparoscopic adrenalectomy for pheochromocytoma detected during pregnancy. After appropriate radiologic investigation and medical management, a laparoscopic left adrenalectomy was performed at the beginning of the second trimester. There were no complications, and she was delivered of a healthy baby at term. We review the management of pheochromocytoma in pregnant patients and discuss the role of laparoscopy.     

膀胱嗜铬细胞瘤的诊断和治疗

目的：提高膀胱嗜铬细胞瘤的诊断与治疗水平。方法：5例膀胱嗜铬细胞瘤患者均通过膀胱镜检查获得定位诊断并均经手术治疗，其中4例行膀胱部分切除，1例行全膀胱切除术。结果：5例获随访，时间2个月～9年。3例痊愈，2例分别于术后2个月、5年死于脑溢血。结论：膀胱镜是最主要的定位检查手段，术前准备与其他部位嗜铬细胞瘤同样重要，术式选择应依据病灶大小及部位。     

膀胱嗜铬细胞瘤的诊治（附13例报告）

目的：提高对膀胱嗜铬细胞瘤的诊断和治疗水平。方法：回顾性分析13例膀胱嗜铬细胞瘤的临床和病理资料,并结合文献复习进行讨论。结果：13例患者中,高血压患者6例,其中5例在排尿时典型发作;间歇性肉眼血尿4例。术前确诊5例。13例均行手术治疗,9例行膀胱部分切除术,2例行膀胱部分切除和输尿管再植术,1例行TURBT术,1例行TURBT后中转开放性膀胱部分切除术。13例均经术后病理检查及免疫组化证实为膀胱嗜铬细胞瘤,其中9例患者获得3～11年随访,1例患者术后6年死于肝转移。结论：与排尿有关的阵发性高血压发作,头痛头晕为膀胱嗜铬细胞瘤的典型表现,可应用B超、CT、MRI、123I-MIBG作定位诊断,尿VMA和儿茶酚胺作定性诊断。手术切除是首选治疗方法,术后应严格随访。     

肾上腺肿瘤影像学诊断与病理组织学类型的关系（附32例分析）

为探讨肾上腺肿瘤影像学诊断与病理组织学类型的关系，对１９８８～１９９５年收治的３２例肾上腺肿瘤病例进行分析。其中２８例手术治疗者经病理证实为嗜铬细胞瘤８例，髓样脂肪瘤５例，皮质腺癌５例，皮质腺瘤３例，节细胞神经瘤３例，副神经瘤２例，肾上腺囊肿１例，恶性淋巴瘤（Ｂ）弥漫型１例。２８例患者均行ＣＴ检查，８例作了ＭＲＩ检查，３例作了ＤＳＡ检查。结合病理，分析了ＣＴ、ＭＲＩ和ＤＳＡ的影像学表现。结果表明：嗜铬细胞瘤，髓样脂肪瘤，皮质腺癌，皮质腺瘤的影像学改变各有特点。本组资料对积累ＣＴ、ＭＲＩ和ＤＳＡ对肾上腺肿瘤的定位及定性诊断的经验有一定的参考价值。     

Usefulness of intraoperative 123I-MIBG during reoperation for malignant pheochromocytoma: a case report

Intraoperative MIBG radionuclear scanning has been used to improve pathological foci localization and surgical accuracy in patients with neural crest derived tumors. This intraoperative detection has been reported in less than 10 patients during reoperation for pheochromocytoma. We report a case of 123I-MIBG intraoperative detection allowing to improve surgical resection quality during reoperation for pheochromocytoma. The use of intraoperative MIBG radionuclear scanning is helpful when reoperation for pheochromocytoma is performed.     

肾上腺外嗜铬细胞瘤的诊断及治疗（附48例报告）

目的提高肾上腺外嗜铬细胞瘤的诊断和治疗水平。方法分析1986年至2005年资料完整、经病理证实的肾上腺外嗜铬细胞瘤48例。其中高血压43例、血尿6例,体检发现肿瘤3例。48例患者中尿VMA及血、尿儿茶酚胺升高分别为44例（91%）和40例（83%）。所有患者经B超检查,阳性率为92%、经CT扫描阳性率为100%。48例患者均行手术治疗。结果切除肿瘤40例,包膜下剜除肿瘤8例。手术切除瘤体直径2-9cm。随访6个月-19年,43例高血压患者术后血压正常31例、仍有高血压2例。肿瘤复发、转移6例,恶性嗜铬细胞瘤12例,死亡7例。结论尿VMA及血、尿儿茶酚胺是定性诊断异位嗜铬细胞瘤的主要依据,CT诊断定位准确,131碘-间位碘代苄胍（131I-MIBG）定位准确、敏感性高,且可作为治疗措施。术前降压、扩容、纠正心律失常是手术成功的关键,经腹径路手术暴露良好,肿瘤外侵时可作囊内剜除,术后应密切随访。     

Current problems in adrenal surgery

Retrospective study on diagnosis and management during the perioperative period of patients with adrenal tumors who underwent adrenalectomy in the Second Department of Surgery Kyoto University, from 1960 to 1983, was carried out. Most of the patients presented with functioning tumors; primary aldosteronism (43 cases), Cushing's syndrome (53 cases), pheochromocytoma (39 cases), and DOC-producing tumor (1 case), whereas, only 3 cases were non-functioning adenoma and cyst. Although recent development of CT has permitted easy and non-invasive diagnosis of adrenal tumors, aldosterone-producing tumors may be overlooked due to their small sizes, because their CT value is identical to that of fatty tissue. Moreover, blood sampling from vena cava at various levels is also necessary in patients with extra-adrenal pheochromocytomas. The preoperative control of blood pressure by alpha- and beta-adrenergic blocking agents was a prerequisite, and the clinical course was uneventful following intraoperative additive use of sodium nitroprusside in patients with pheochromocytoma. Autotransplantation of the adrenal cortex into the rectus muscle was performed in three patients with bilateral pheochromocytoma and two of them could return to work. Non-functioning adrenal tumors have been found increasingly by routine CT study without previous suspicion and the surgical indication of these tumors must be discussed in more detail in the future.     

嗜铬细胞瘤的外科治疗(附151例报告)

目的 提高嗜铬细胞瘤的治疗水平。方法 总结151例嗜铬细胞瘤术前准备、麻醉管理、手术操作及术后处理经验。男82例，女69例，年龄15～73岁，平均39岁。肾上腺内120例，肾上腺外31例。结果 肿瘤切除术132例，肿瘤囊内剜除术12例，膀胱部分切除术7例。术中死亡l例。术后血压正常136例，仍需持续用药控制15例。良性嗜铬细胞瘤137例，9例复发；恶性嗜铬细胞瘤14例，其中10例5年内死于肿瘤转移。结论 手术切除肿瘤是嗜铬细胞瘤的根治方法，正确的术前诊断和围手术期处理十分重要。     

The association of neurofibromatosis, pheochromocytoma, and somatostatin-rich duodenal carcinoid tumor

The association of neurofibromatosis and pheochromocytoma is well recognized; more recently, attention has been drawn to links between neurofibromatosis, pheochromocytoma, and ampullary somatostatin-rich carcinoid. Because of this association, the duodenum was explored during a recent laparotomy for resection of bilateral pheochromocytoma in a patient with von Recklinghausen's disease. A clinically unsuspected ampullary tumor was discovered; this proved to be in part a ganglioneuroma and in part a somatostatin-rich carcinoid. This paper presents full details of this carefully investigated and documented case and reviews the recent advances in this field. These studies lead us to conclude that: the clinical association of neurofibromatosis, pheochromocytoma, and D cell carcinoids ("somatostatinomas") of the ampullary region is confirmed; this association may be more common than has been previously thought, and the duodenum should be carefully examined in any patient with neurofibromatosis who undergoes laparotomy for pheochromocytoma.     

Hypertensive crisis inducing rhabdomyolysis during laparoscopic resection of a large pheochromocytoma: a case report

Intense vasospasm during hypertensive crisis as a cause for rhabdomyolysis has been described in ambulatory patients with pheochromocytoma. We report this phenomenon as an intraoperative complication in a patient during laparoscopic pheochromocytoma resection and discuss patient and procedure related risk factors. Unexplained intraoperative hyperkalemia following a severe hypertensive episode during surgery may be a first sign of rhabdomyolysis. Anesthesiologists should be aware of such a possibility. Intra- and postoperative serial electrolyte determinations and if suspected, timely screening for myoglobiuria, may aid in early detection and treatment of intraoperative rhabdomyolysis during pheochromocytoma resections.     

The usefulness of [131I]-metaiodobenzylguanidine ([131I]-MIBG) scintigraphy performed one week after administration in diagnosing pheochromocytoma

BACKGROUND: Imaging performed 36-48 h after metaiodobenzylguanidine (MIBG) injection is being widely used in the diagnosis of pheochromocytoma. However, there are some difficult cases to diagnose due to a high concentration of MIBG remaining in the background. We studied the significance of scans on the 7th day after MIBG injection when the concentration of MIBG in the background has declined. METHODS: Imaging was carried out on 11 cases before operation, five cases (eight times) after operation and 12 cases which had been strongly suspected of being pheochromocytoma, but later proved to be non-pheochromocytoma. RESULTS: In all the cases of pheochromocytoma, except one, the tumor imaging was clear 24-72 h after MIBG injection. As for the images after operation and those of the 12 non-pheochromocytoma cases, the scintigram made on the 7th day proved the negative pheochromocytoma. CONCLUSION: This approach was very effective not only for finding early small pheochromocytomas and the remnants of tumors after resection, but also in diagnosing non-pheochromocytoma.     

Etiology and clinical guidelines for the diagnosis and treatment of pheochromocytoma in Japan

Although pheochromocytoma is one curable cause of endocrine hypertension, approximately 10% of patients have malignant disease. Since the histopathologic diagnosis of malignancy at the time of first surgery is difficult and malignancy is confirmed only after the detection of metastasis, it is a representative intractable rare disease without effective treatment. A nationwide survey in Japan in 2009 found 2,600 cases of benign pheochromocytoma and 320 of malignant pheochromocytoma. There was no gender bias among patients, and the average age of onset was 40 to 45 years. Sixty-five percent were symptomatic including hypertension, but 35% were asymptomatic and found occasionally as incidentaloma Extraadrenal, bilateral, malignant, and familial cases each represented 10% of the total. A task force of the Ministry of Health, Labour and Welfare of Japan established the diagnostic criteria and clinical guidelines for pheochromocytoma/paraganglioma and malignant pheochromocytoma/paraganglioma. Typical cases of pheochromocytoma are readily diagnosed based on high catecholamine levels and imaging of tumor localization and can be cured by surgical resection of the tumor. In contrast, since no effective treatment for malignant pheochromocytoma has been established, a combination of various treatments including the administration of a-blockers, 131-MIBG irradiation, and cisplatin/vinblastine/dacarbazine chemotherapy, and radiation of bone metastases is recommended. Careful long-term follow-up is essential even in patients with benign pheochromocytoma.     

Influence of tumor size on anesthetic management for pheochromocytoma resection

The relationship between tumor size and the complexity of anesthetic management was studied using several values: plasma catecholamine concentrations, requirement of vasoactive agents, surgical time, blood loss, plasma glucose concentrations, and hemodynamic variables. Ten patients with clinical and laboratory diagnosis of pheochromocytoma were prospectively studied. Each anesthesia was maintained using inhalational anesthetic agents. Control of arterial blood pressure (ABP), heart rate (HR), and pulmonary artery blood pressure (PABP) was attempted with only titrating the inhalational anesthetics and adenosine triphosphate (ATP). If the titration of both the inhalational anesthetic and ATP failed to control ABP, HR, or PABP, then phentolamine, propranolol, trinitroglycerine, or norepinephrine was additionally used. Tumor weight was significantly correlated with amount of blood loss, surgical time, duration of ATP requirement, maximal dose of ATP infusion used, maximal plasma glucose concentration, and plasma total catecholamine concentration. However, the tumor weight was not correlated with hemodynamic variables. Patients who required propranolol generally had a significantly larger tumor than those who did not. In conclusion, surgical removal of large pheochromocytoma required more complicated anesthetic management than that of small pheochromocytoma.     

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。     

膀胱嗜铬细胞瘤7例诊治分析

目的探讨膀胱嗜铬细胞瘤的诊断和治疗经验。方法回顾性分析本院2003年4月至2010年4月诊治的7例膀胱嗜铬细胞瘤患者的临床资料。男性3例,女性4例,平均年龄45.3（31~57）岁。高血压者6例,5例在排尿时血压明显增高;间歇性肉眼血尿者3例。血浆游离MNs和24h尿CA定性诊断的阳性率分别为100.0%（7/7）和85.7%（6/7）。定位检查包括B超、CT、MRI、131I-MIBG和膀胱镜,其各自的定位准确率分别为85.7%（6/7）、100.0%（6/6）、66.7%（2/3）、100.0%（5/5）和75.0%（3/4）。1例进行放射治疗,2例实施膀胱部分切除,4例进行肿瘤剜除。结果 7例膀胱嗜铬细胞瘤在膀胱内的位置为：前壁2例、三角区2例、后壁1例、左侧壁1例、右侧壁1例。平均手术时间131.2（110~190）min,平均出血量283（100~1 200）mL。良性者6例,恶性者1例。随访5个月~7年,1例于术后3年复发,2次手术行全膀胱切除术。结论膀胱嗜铬细胞瘤罕见且容易误诊,高血压、血尿、排尿时典型发作为其主要症状;血浆游离MNs和24h尿CA是重要的定性检查方法,B超、CT、MRI、131I-MIBG及膀胱镜是重要的定位诊断方法;膀胱部分切除或肿瘤剜除是首选的治疗方法,术后应长期随访。     

Incidentally discovered pheochromocytoma in long-term hemodialysis patients.

Two cases of pheochromocytoma incidentally discovered in long-term hemodialysis patients are reported. Case 1 was a 47-year-old-man who had been receiving hemodialysis for 18 years. Case 2 was a 33-year-old woman who had been receiving hemodialysis for 12 years. Both cases were normotensive, and no specific symptoms suggesting pheochromocytoma were seen. Plasma norepinephrine (NE) levels were not elevated in both cases; however, the level of epinephrine (E) was double the normal range in Case 2. After surgery, plasma E level returned to the normal range in Case 2; however, the level of NE remained almost the same as the preoperative value in both cases. Plasma catecolamine levels in long-term hemodialysis patients with pheochromocytoma are reviewed in the present report, and the efficacy of imaging methods in the diagnosis of pheochromocytoma are discussed.     

Laparoscopic partial adrenalectomy in patients with hereditary forms of pheochromocytoma

PURPOSE: Patients with von Hippel-Lindau disease are predisposed to multiple bilateral adrenal pheochromocytoma. In these patients partial adrenalectomy may preserve adrenocortical function and avoid the morbidity associated with medical adrenal replacement. We report our experience with such cases. MATERIALS AND METHODS: Laparoscopic partial adrenalectomy was performed in patients with von Hippel-Lindau disease and pheochromocytoma when there was evidence of normal adrenocortical tissue on preoperative imaging or intraoperative examination. Suture ligature or a harmonic scalpel was used to excise the tumors, leaving a 2 to 3 mm. margin of normal tissue. RESULTS: Two patients underwent laparoscopic partial adrenalectomy and 1 laparoscopic bilateral partial adrenalectomy with preservation of normal adrenocortical tissue. Seven pheochromocytomas were removed. Laparoscopic ultrasound was essential for localizing 2 pheochromocytomas that were not visualized by the camera. Median operative time was 324 minutes, blood loss 100 cc and parenteral narcotic requirement 22 mg. morphine equivalents. No patient required hydrocortisone replacement. There has been no pheochromocytoma recurrence during short-term followup. CONCLUSIONS: Laparoscopic partial adrenalectomy is technically feasible in patients with a hereditary form of pheochromocytoma, and may preserve adrenocortical function. Laparoscopic ultrasound was necessary to identify 2 of the 7 pheochromocytomas removed.     

Reversible catecholamine-induced cardiomyopathy in a heart transplant candidate without persistent or paroxysmal hypertension.

BACKGROUND: Both dilated and hypertrophic cardiomyopathy have been reported in patients with pheochromocytoma, who were almost always hypertensive. The outcome frequently has been fatal, yet cardiac dysfunction can be reversible after medical or surgical therapy for the pheochromocytoma. METHODS: We report the case of a patient with dilated cardiomyopathy without persistent or paroxysmal hypertension, who was found to have a pheochromocytoma during initial medical evaluation. RESULTS: The identification and treatment of the pheochromocytoma led to significant improvement in cardiac function and cardiac transplantation was avoided. CONCLUSIONS: This case illustrates some unusual features in pheochromocytoma-induced cardiomyopathy: (1) absence of persistent or paroxysmal hypertension, (2) initial presentation with acute myocardial infarction and normal coronary arteries, and (3) recurrent episodes of nonsustained ventricular tachycardia.