Recognition and management of phaeochromocytoma

Phaeochromocytomas and paragangliomas (PPGL) are catecholamine-secreting neuroendocrine tumours. These tumours may be identified incidentally, as part of a work-up for multiple endocrine neoplasia or following haemodynamic surges during unrelated procedures. Advances in preoperative management and improved management of intraoperative haemodynamic instability have significantly reduced surgical mortality from around 40% to less than 3%. Surgery is the definitive treatment in most cases and laparoscopic resection where possible is associated with improved outcomes. Anaesthetic management of PPGL cases represents a unique haemodynamic challenge both before, during and after tumour resection. In this article we describe the physiology of these tumours, their diagnosis, preoperative optimization methods, intraoperative anaesthetic management and management of postoperative complications.     

Recognition and management of phaeochromocytoma

Phaeochromocytomas are catecholamine-secreting neuroendocrine tumours arising from the chromaffin cells in the adrenal medulla. These tumours may be identified incidentally, as part of a workup for multiple endocrine neoplasia or during unrelated surgery. Better understanding of catecholamine physiology and advances in preoperative preparation has significantly reduced surgical mortality from around 40% to less than 3%. Surgery is the definitive treatment in most cases and laparoscopic resection is associated with reduced hospital stay and earlier mobilisation. Phaeochromocytomas are of particular interest to anaesthetists as it presents a unique haemodynamic challenge both before and after adrenal resection. In this article we describe the physiology of these tumours, their diagnosis and perioperative management.     

Perioperative Risk Factors,Morbidity, and Outcome of 145 Patients during Phaeochromocytoma Resection

Background : Phaeochromocytoma and paraganglioma resection carries a high perioperative risk. The aim of this study was to determine the risk factors for and frequency of perioperative morbidity and mortality during resection of these tumours.

Methods : Computerized surgical, medical, and histopathology records, as well as anaesthesia cards for 145 patients undergoing surgery for phaeochromocytoma resection between 1995 and 2009 were analysed retrospectively. Preoperative notes, adverse intraoperative events, and postoperative complications occurring in the 30 days following surgery were recorded. Preoperative phenoxybensamine and propranolol dose, age, sex, ASA score, catecholamine urinary level and profile, tumour weight, duration of surgery, and malignancy and presence of bilateral tumours were investigated to determine their contribution to major intraoperative haemodynamic events. Univariate comparisons were carried out using the student t-test. The Mann-Whitney test was also used to allow for deviation from normality. Logistic regression with backward removal of insignificant variables was used for multivariate analysis. Spearman test was used for correlation analysis.

Results : The only statistically significant factor that increased the probability of intraoperative haemodynamic variability was the duration of surgery (p = 0.025). The most common intraoperative event was transient hypertension, occurring in 59 (40.7%) patients. Sustained hypertension was registered in only 10 (6.9%) patients. No mortality, myocardial infarction, or cerebrovascular incidents were recorded.

Conclusions : We found that the severity of perioperative haemodynamic changes significantly correlated with the duration of surgery. Our patients had low perioperative morbidity and no mortality.     

Hepatic resection for tumours in cirrhotic livers

A liver resection was performed in 25 out of 36 cirrhotic patients operated on for liver cell carcinomas. In the remaining 11 cases hepatectomy was not performed mainly because of the presence of other intrahepatic neoplastic nodules or thrombi in the portal branches revealed by intraoperative echography. The operative mortality in the 25 patients operated on was 16%; the actuarial survival at three years is 58%. Liver resection was carried out using a transparenchymal procedure; in 18 cases clamping of the hepatic pedicle was performed for an average period of 15 min. Twenty patients with small tumours had a segmentary or sub-segmentary resection; intraoperative echography proved indispensable in this situation, making it possible to recognize the lesion and outline the limits of the resection. The presence of a peritumoral capsule seems to have been an important prognostic factor.     

Treatment of ruptured hepatocellular adenoma

BACKGROUND: As the morbidity and mortality rates associated with emergency resection in patients with a ruptured hepatocellular adenoma are high, the authors have favoured initial non-operative management in haemodynamically stable patients. METHODS: A retrospective study was performed to evaluate the treatment of ruptured hepatocellular adenoma. RESULTS: Over a 21-year interval, 12 patients presented with a ruptured hepatocellular adenoma. Haemodynamic observation and support was the initial management in all 12 patients. Three underwent urgent laparotomy and gauze packing because of haemodynamic instability; no emergency liver resection was necessary. Eight patients had definitive surgery; three developed postoperative complications but none died. Regression of the tumour was observed in three of four patients treated conservatively. CONCLUSION: The initial management of a ruptured hepatocellular adenoma should be haemodynamic stabilization. Definitive resection is required for rebleeding or for tumours exceeding 5 cm in diameter. A conservative approach may well be justified in case of regression of an asymptomatic adenoma.     

The value of a second transurethral resection in evaluating patients with bladder tumours

OBJECTIVES: To evaluate the usefulness of a second transurethral resection for superficial and muscle-invasive bladder tumours. METHODS: A review of the literature relevant to repeat resection for bladder tumours was conducted using Medline Services. RESULTS: Transurethral resection of the bladder has two shortcomings: underestimating clinical stage, and overlooking other lesions. A second transurethral resection, when performed 2-6 weeks after the initial resection, corrects clinical staging errors in 9-49% of cases and detects residual tumour in 26-83% of cases. A second resection is particularly warranted for T1 tumours since 2-28% of them prove to be muscle-invasive, thus requiring a change in management. For muscle-invasive tumours, a second resection may be performed only if bladder sparing is being considered, as it helps to exclude the presence of tumour sites contra-indicating conservative treatment. CONCLUSIONS: A second transurethral bladder resection may be warranted for T1 tumours, and for invasive tumours when a bladder preservation is planned.     

Coincident vesical transitional cell carcinoma and prostatic carcinoma. Clinical features and treatment

Twenty-two patients with vesical urothelial carcinoma associated with prostatic carcinoma were reviewed. They represented 1.5% of the bladder and prostatic tumours treated in our department within a 12-year period from 1968 to 1979. Their management included several treatment policies, based on the separate assessment of each tumour variant. For non-infiltrating bladder tumours, transurethral tumour resection was combined with hormonal treatment, external radiotherapy or resection of the prostate depending on the stage of the prostatic tumour. Radical cystoprostatectomy was performed for two cases of infiltrating bladder tumour with well localised prostatic tumours. A conservative primary approach seems justifiable in the management of double carcinoma of the bladder and prostate. The coincidence of bladder urothelial carcinoma and prostatic carcinoma per se is not an adverse prognostic factor; prognosis is more closely related to the pathological stage and grade of the bladder tumour. Cystoprostatectomy for patients with infiltrating bladder tumours could be curative, in selected cases, for the prostatic cancer as well.     

Carotid body tumours: report of six cases and a review of management

We have treated six patients with carotid body tumours in the period from 1972 to 1988. All patients had a neck mass on presentation. In addition one patient complained of tinnitus and another was noted to have Horner's syndrome. The diagnosis was confirmed by ultrasound and angiography in all cases. Five patients subsequently underwent successful surgical resection. At the time of surgery one of the tumours was found to be locally invasive. One elderly patient was deemed unfit for surgery and was managed non-surgically with a satisfactory outcome. A review of the literature reveals that surgery is still the preferred mode of treatment although preoperative embolization may be a useful adjunct. Although the incidence of peroperative stroke has gradually been reduced from that found in earlier series, injury to the cranial nerves remains high and is the main hazard of surgical management. The improved results of surgical resection in more recent reports support the view that these tumours should be treated in units with expertise in vascular surgery of the neck.     

Solitary fibrous tumour of the pleura: surgical treatment.

OBJECTIVE: Solitary fibrous tumours (SFT) of the pleura are rare tumours originated from the mesenchimal tissue underlying the mesothelial layer of the pleura. This tumours present unpredictable clinical course probably related to their histological and morphological characteristics. METHODS: Twenty-one patients affected by SFT of the pleura were referred to us for surgical resection from September 1984 to April 2000. They were 15 males and six females with median age of 51 (range 15--73) years. Nine patients (43%) were symptomatic and predominant clinical symptoms or signs were dyspnoea (19%), coughing (14.3%), chest pain (28.5%), finger clubbing (14.3%) and hypoglycaemia (14.3%). Hypoglycaemia was related to a pathological incretion of insulin-like growth factor 2 by the tumour. Chest radiograph and computed tomography of the chest revealed intra-thoracic homogeneous sharply delineated round or lobulated mass sometimes associated with ipsilateral pleural effusion (19%) or causing pulmonary atelectasis with opacification of the complete hemithorax (19%). Surgical excision required 14 posterolateral thoracotomies, six anterior thoracotomies and one video-assisted thoracoscopy. Thirteen tumours arose from visceral pleura and wedge resection was performed, seven tumours arose from parietal pleura and extrapleural resection was carried out without any chest-wall resection, one tumour growth within the upper left lobe and required lobectomy. Tumours weighted from 22 to 1942 g and measured from 22x12x8 to 330x280x190 mm. At cut section seven cases (34%) revealed focal necrosis and hemorrhagic zones and on light microscopy six cases (28.5%) were characterized by high mitotic count: characteristics related with uncertain clinical behaviour. Immuno-histochemical reactions were in all cases positive for CD34. RESULTS: In all our patients resections were complete. Paraneoplastic syndromes like hypoglycaemia and clubbing receded after surgery. No intraoperative or perioperative medical or surgical complications occurred. Median chest-drain duration timed 3 (range 2--5) days and median hospital stay was 5 (range 4--7) days. Perioperative mortality rate was 0%. Median follow-up was 68 (range 2--189) months: during this period patients were submitted to chest X-ray with 6-months interval to evaluate possible local recurrence. Only one patient experienced tumour recurrence after 124 months follow-up: the tumour was suspected after observation of finger clubbing. The tumour was detected and excised by redo-thoracotomy. CONCLUSIONS: Surgical resection of benign solitary fibrous tumours is usually curative, but local recurrences can occur years after seemingly adequate surgical treatment. Malignant solitary fibrous tumours generally have a poor prognosis. Clinical follow-up and radiological follow-up are indicated for both benign and malignant solitary fibrous tumours.     

活体供肝切除术的麻醉管理

目的 回顾性分析45例活体供肝切除术的麻醉管理和手术经过.方法 回顾45例活体肝移植供肝切除术的麻醉处理过程和手术经过,术中持续监测BP、HR、SpO2、CVP、PETCO2等重要的生理指标.在手术开始、供肝切除前、后30 min三个时点分别采血检测血常规、血生化、肝肾功能、凝血功能和动脉血气.结果 术中血流动力学稳定,所有供体均未发生术中并发症和死亡.肝功能的各项指标在供肝切除过程中发生了剧烈的变化,凝血功能随着手术的进行也有一定程度的恶化,肾功能则未受到明显影响.结论 尽管术中多项生理指标发生明显异常,但活体供肝切除术能够在保持血流动力学稳定、术后无手术并发症的情况下顺利完成.     

Value of transoesophageal echocardiography for diagnosis of intraoperative tumour embolization

Malignant neoplasms such as renal cell carcinoma may invade the inferior vena cava leading to a risk of pulmonary tumour embolization during surgical excision. Although massive pulmonary tumour embolism occurs relatively rarely, it can have catastrophic consequences. We report the case of an acute intraoperative pulmonary tumour embolism during resection of a renal cell carcinoma. The use of transoesophageal echocardiography allowed the immediate diagnosis and appropriate management of the underlying cause of acute haemodynamic instability. The role of transoesophageal echocardiography in the diagnosis of pulmonary embolism is discussed.     

Intraoperative ultrasonography during planned liver resections: why are we still performing it?

Background Intraoperative ultrasonography (US) is used in many centers before oncologic liver resections to detect additional tumors and to evaluate the relationship of tumors to major vascular structures. As preoperative imaging improves, it is expected that the diagnostic yield from intraoperative US will diminish. In this study we attempt to determine if fewer unrecognized tumors were being detected and whether intraoperative US is having less impact on surgical decision making. Methods We compared 50 consecutive cases (mean age = 57.2 ± 10 years; 27 men) who underwent laparotomy for a planned resection of primary liver malignancies or metastases between September 2003 and July 2005 with 50 consecutive cases (mean age = 56.9 ± 14 years; 25 men) between January 1999 and September 2003. Dedicated intraoperative liver US was performed or supervised by a gastrointestinal radiologist using a 5.0-MHz linear- or curvilinear-array transducer during each procedure. Results The rate of detecting unrecognized tumors has not changed significantly (14% vs. 20%, p = 0.70). The use of US to establish the relationship between tumor and the vasculature has not changed (48% vs. 60%, p = 0.23). The percentage of cases where the US findings were responsible for altering surgical management was 20% for both groups. The resection rate was 72% for both groups. The negative resection margin rate has also not changed significantly (86% vs. 69%, p = 0.09). Conclusions Despite the advances in cross-sectional imaging, the frequency of unrecognized tumors found during intraoperative liver US and its use for surgical guidance has not changed significantly. Currently routine intraoperative US alters the management of approximately one fifth of our patients undergoing attempted liver resection for primary malignancies or metastases. Presented at the Meeting of the Society of American Gastrointestinal and Endoscopic Surgeons (SAGES), Dallas, TX, 26–29 April 2006     

Spotlight on a distant mirror – colorectal cancer surgery in a 20-year period at a general hospital

Background A published audit of the management of colorectal cancer at a general hospital in the 1970s was available for comparison with a later audit at the same hospital in the 1990s. Methods Case note analysis. Results In the later audit, more cases were treated annually by an unchanged surgical team. The incidence of synchronous combined excision of the rectum, for rectal cancers suitable for resection, was halved, and that of anterior resection of the rectum (sphincter sparing, without a permanent stoma) increased almost threefold. The incidence of local recurrence in cases suitable for rectal surgery dropped from 17% to 9%, in spite of the change in the principal operation undertaken for this population. Outcomes associated with critical care improved as resources in this discipline became available. Overall survival figures were only improved by 6% in the20‐year period, reflecting a diagnosis of Dukes C tumours or worse in at least 45% of the stable population studied in both audits. Conclusion More resources are necessary in Great Britain to increase survival figures in this common cancer. Earlier diagnosis and more specialist management of the disease may allow us to emulate American and Swedish survival figures.     

A review of Japanese literatures concerning surgery for lung cancer with idiopathic interstitial pneumonia

OBJECTIVE: The aim of the present study is to investigate the perioperative management of acute exacerbation of idiopathic interstitial pneumonia (IIP) after pulmonary resection for lung cancer. METHODS: We reviewed 5 Japanese literatures published from 1992 through 1998. The present study included 50 lung cancer patients with IIP, of which 3 were our cases and 47 were reported cases. RESULTS: Within 30 days after operation, acute exacerbation of IIP occurred in 12 cases (24.0%). Preoperative profiles (gender, age, smoking status, respiratory function, pathologic stage) of the exacerbated cases did not differ from those of non-exacerbated cases. The mean intraoperative PaO2 of the exacerbated cases was significantly higher than that of non-exacerbated cases (224 +/- 45.6 Torr vs 120 +/- 41.2 Torr, p = 0.005). The rate of acute exacerbation increased with the extent of resection, that is 42.8% in pneumonectomy, 24.3% in lobectomy, and 0% in wedge resection although the difference was not significant. After acute exacerbation of IIP, all 12 cases were given high doses of steroids. However, 11 cases died due to the disease progression (mortality rate = 91.7%). CONCLUSION: In order to establish the perioperative management for prevention of acute exacerbation of IIP, multi-institutional study is warranted on the basis of the present results (intraoperative FiO2, administration schedule of steroids etc).     

Midazolam-sufentanil anaesthesia for phaeochromocytoma resection

This is a case report of a 66-year-old patient to whom a combined infusion of midazolam and sufentanil was administered for phaeochromocytoma resection. With the exception of a drop in blood pressure immediately after tumour removal, significant intraoperative haemodynamic stability was observed. There was no need for the intraoperative administration of hypotensive or anti-arrhythmic drugs, nor was any prolonged postoperative anaesthetic effect noted. In this case, the combination of midazolam and sufentanil with N2O:O2 was successful in maintaining cardiovascular stability until the tumour was removed. The consequent drop in blood pressure responded to fluid infusion, a not uncommon event in phaeochromocytoma surgery. Although a prospective randomized study for resection of phaeochromocytoma showed that the choice of the anaesthetic technique is not a crucial factor in determining the patient outcome, we feel this technique of midazolam sufentanil is a worthy alternative to the use of inhalational anaesthetics. The lack of significant myocardial depressive effect of the two drugs, coupled with its simple administration, makes it a useful technique in the anaesthetic management of phaeochromocytoma resection.     

Anaesthesia for elective liver resection: some points should be revisited

Improvement in surgical techniques, technology and perioperative assessment has dramatically simplified the anaesthetic care for elective liver resection. Patients with a non-tumorous healthy liver should only need the usual preoperative assessment. Patients with pre-existing parenchymal liver disease should be specifically assessed for gas exchange impairment, alcoholic or nutritional-associated cardiomyopathy, infection, cirrhosis decompensation, acute alcoholic hepatitis, and kidney impairment. The type of anaesthetic management does not influence the intra- and postoperative courses. Intermittent clamping of the portal vascular triad is better tolerated than prolonged continuous periods of ischaemia--especially in patients with abnormal liver parenchyma. Intraoperative antibiotic prophylaxis must be administered to prevent translocation of intestinal enterobacteria to the systemic circulation in patients with both healthy and diseased livers. Blood-salvage techniques have limited indications in liver resection. Systematic invasive haemodynamic monitoring is no longer warranted. An arterial cannula should only be considered in procedures of long duration and in selected situations likely to cause anticipated circulatory impairment: total liver vascular occlusion, repeat surgery, combined organ resection, and surgery conducted on tumours >10 cm in size or in connection with the vena cava. In a recent large series of liver resections, 60% of patients did not need a blood transfusion, only 2% of transfused patients received >10 units of blood and cirrhosis was not predictive of increased intraoperative bleeding. Postoperative ascites, which always develops at the expense of circulating fluid, is a frequent occurrence in patients with healthy or diseased livers. Intra- and postoperative fluid limitation does not prevent postoperative ascites. Volume expansion, diuretics and vasopressor therapy should be initiated early to prevent kidney failure.     

Surgery for periampullary and pancreatic carcinoma: a Liverpool experience.

The development of a single-surgeon specialist referral practice for pancreatic surgery which evolved over an 8 year period is described. Source of referral, protocol for patient management, and operative strategy are outlined. Preoperative endoscopic retrograde cholangiopancreatography (ERCP), endoscopic sphincterotomy, and stent placement where possible (85% of cases), high-resolution contrast-enhanced CT and standard pylorus-preserving pancreaticoduodenectomy with a unique reconstructive technique were employed. In 105 patients receiving curative resection for pancreatic or periampullary tumours, the overall operative mortality was 4.8% and overall morbidity 26%. Actuarial 5-year survival rates were 11% for pancreatic carcinoma and 34% for ampullary carcinoma. Resectability rate was 81% without the use of time-consuming and expensive imaging techniques for staging such as laparoscopy, intraoperative ultrasound or laparoscopic ultrasound. No specific regimen of perioperative chemoirradiation was utilised over the study period. To achieve comparable results it is recommended that patients should be referred to regional specialist surgeons in whose hands mortality and morbidity is low, costs reduced and training of pancreatic surgeons can be undertaken.     

Diagnosis and surgical therapy of organic hyperinsulinism

The diagnosis of an insulin producing tumour can be confirmed by a minimum of biochemical investigations. Its preoperative localisation is more difficult. Sonogram, Computertomogram, selective angiography and percutaneous transhepatic collecting of blood samples for insulin analysis from the portal system were preoperative measured to localize the tumours in 32 of 37 patients of our series. In 2 patients intraoperative tumour localisation by measurement of incorporated p32 proved to be effective. In B-cell-carinomas pancreas resection is the adequate therapy. With regard to the therapeutic effects a high risk is involved in the 'blind' left or right sited resection of non-localized tumours.     

The role of intraoperative parathyroid hormone testing in patients with tertiary hyperparathyroidism after renal transplantation

BACKGROUND: Intraoperative parathyroid hormone (PTH) testing has been shown to accurately define adequacy of parathyroid resection in patients with primary hyperparathyroidism (HPT) and alters the operative management in 10% to 15% of cases. However, the benefit of this technique in patients with tertiary HPT after renal transplantation undergoing parathyroidectomy is unclear. METHODS: Intraoperative PTH was measured in 32 consecutive patients undergoing parathyroidectomy for tertiary HPT after renal transplantation between March 2001 and November 2004 by using the Elecsys assay at baseline and, subsequently, 5, 10, and 15 minutes after curative resection. The outcomes of these patients were evaluated. RESULTS: All patients were cured after surgery. Of the 32 patients, 29 were found to have parathyroid hyperplasia, while 1 had a single adenoma and 2 had double adenomas. The average drop in intraoperative PTH levels after curative resection was 69 +/- 3.5% at 5 min., 77 +/- 2.3% at 10 minutes, and 83 +/- 3.4% at 15 minutes. PTH testing changed the intraoperative management in 5 (16%) patients. One patient with a single adenoma and 2 patients with double adenomas had a >50% drop at 10 minutes. after excision; therefore, the operation was terminated without further resection. Two patients did not have a >50% drop at 10 minutes after 3.5 gland resection. These patients were explored further, and additional supernumerary parathyroid glands were identified and resected. After resection of these additional glands, the PTH fell by >50%, indicating cure. CONCLUSIONS: In patients undergoing parathyroidectomy for tertiary HPT after renal transplantation, a decrease in intraoperative PTH levels >50% at 10 minutes after completion of the operation indicated adequate resection. Furthermore, intraoperative PTH testing altered the operative management in 16% of patients. Therefore, similar to its role in patients with primary HPT, intraoperative PTH testing appears to play an equally important role in the management of patients with tertiary HPT undergoing parathyroidectomy.     

Cancer of the oral cavity: management of the clinically negative neck

The management of the clinically uninvolved (No) neck in patients with cancers of the oral tongue, or floor of the mouth, including the gum, and treated by intra-oral resection remains contentious. The high incidence of metastasis to the neck found in patients selected for composite resection of tumours of these sites (30-59%) might suggest that elective treatment of the neck, either by neck dissection or irradiation, should be performed in all patients. To the contrary, it is shown that the incidence of late metastasis from T1 tumours, treated by intra-oral resection alone, is low (less than 20%), and although the incidence of late neck metastasis from T2 tumours which have been similarly treated, is 42.7%, death from uncontrolled disease in the neck, with the primary controlled, occurs in only 4%. Intra-oral resection of superficial T1 cancers without elective treatment of the neck appears justified, but the management of the No neck in patients with T2 tumours remains controversial.