Comparison of Octreotide and Vasopressors as First-Line Treatment for Intraoperative Carcinoid Crisis.

BACKGROUND: Intraoperative carcinoid crisis is typically sudden onset of profound hypotension during operations on patients with neuroendocrine tumors. The crisis was thought to be due to massive release of hormones, and perioperative octreotide was recommended as a prophylaxis against the crisis and as first-line treatment. Recent studies show that octreotide does not prevent the crisis and that no massive release of hormones occurs. Therefore, the authors hypothesized that octreotide is not effective for treating the crisis. METHODS: A prospective carcinoid anesthesia database was analyzed for occurrences of crisis. Outcomes were compared between protocols when first-line therapy was bolus octreotide and when it was vasopressors without octreotide. Significance was determined by Student's t test, the Mann-Whitney U test, and Fisher's exact test. RESULTS: Among operations performed with octreotide as first-line treatment (n = 150), crisis occurred for 45 (30 %) patients, the median crisis duration was 6 min, 12 (27 %) patients had crises longer than 10 min, 42 patients (93 %) required subsequent vasopressor administration to resolve the crisis, and 3 (2 %) operations were aborted. Among operations performed with vasopressors as the first-line treatment (n = 195), crisis occurred for 49 (25 %) patients (p = 0.31), the median crisis duration was 3 min (p < 0.001), and no crisis lasted longer than 10 min (p = 0.001). Patients treated with vasopressors were less likely to have multiple crises and had a shorter total time in crisis, a shorter anesthesia time, and no aborted operations (p < 0.05 for all). CONCLUSIONS: First-line octreotide was ineffective treatment for carcinoid crisis, with patients requiring vasopressors to resolve the crisis, and many crises lasting longer than 10 min. First-line vasopressor treatment resulted in significantly shorter crisis durations, fewer crises and aborted operations, and shorter anesthesia times. Vasopressors should be used as first-line treatment for intraoperative crisis, and treatment guidelines should be changed.

The Influence of Preoperative Symptoms on the Death of Patients with Small Intestinal Neuroendocrine Tumors.

BACKGROUND: Small intestinal neuroendocrine tumors (SI-NETs) are uncommon tumors with an annual incidence of about 1 per 100,000. Usually, SI-NETs have a slow progression, and patients often present with generalized disease. Many patients do well, and the disease has a relatively favorable 5-year survival rate. Some SI-NETs, however, have a more negative prognosis. This study aimed to establish prognostic factors for death identifiable at primary surgery. METHODS: A nested case-control study investigated 1150 patients from the cohort of all patients with a diagnosis of SI-NETs in Sweden between 1961 and 2001. The study cases consisted of all patients who died of SI-NETs during the study period. Each case was assigned a control subject matched by age at diagnosis and calendar period. Possible prognostic factors [gender, degree of symptoms, indication for surgery, World Health Organization (WHO) stage] were evaluated in uni- and multivariable analyses. RESULTS: The patients with symptomatic disease had an increased risk of dying. The indication for primary surgery influenced survival, showing a more negative prognosis for elective surgery. The WHO stage influenced survival, and stage 4 patients had an almost threefold risk of dying compared with stages 1 to 3b patients. CONCLUSIONS: This study showed that preoperative symptoms are important in prognostication for SI-NETs. Hormonal symptoms generally signify a patient with a more advanced disease stage and a worse prognosis. Including symptomatic disease together with the WHO stage and grade could possibly increase the accuracy of prognostication.

Anesthetic considerations and management of a patient with unsuspected carcinoid crisis during hepatic tumor resection.

Anesthetic management for massive blood loss in liver surgery concomitant with hemodynamic instability secondary to carcinoid crisis can be challenging in the perioperative setting. Hypotension, diarrhea, facial flushing, bronchospasm, and tricuspid and pulmonic valvular diseases are the common manifestations of carcinoid syndrome. This report illustrates the importance of early recognition and treatment for signs and symptoms of carcinoid syndrome not only in the preoperative setting but also in the intraoperative phase to prevent undue cardiovascular collapse.

Anesthetic Management of Gastrointestinal Carcinoid Tumors: A Case Report.

Carcinoid tumors are neuroendocrine tumors (NET) that secrete hormones and other substances into the circulation, causing shifts in hemodynamics and other unpleasant symptoms. This case report describes a case in which the patient had a midgut NET that metastasized to the liver, causing carcinoid syndrome. The patient underwent general anesthesia for an exploratory laparotomy, small bowel resection with anastomosis, mesenteric mass resection, and a right liver lobectomy. The patient had carcinoid syndrome and developed carcinoid crisis during the surgery. Octreotide and phenylephrine infusions and phenylephrine boluses were used during the procedure to stabilize hemodynamics. The pathophysiology of carcinoid tumors, carcinoid syndrome, carcinoid crisis, and carcinoid heart disease are reviewed and octreotide application in the setting of carcinoid syndrome and carcinoid crisis is also reviewed. Octreotide is a first-generation somatostatin analog that binds to somatostatin receptor 2 with high affinity, somatostatin receptor 3 and somatostatin receptor 5 with lower affinity, and suppresses the secretion of serotonin. Anesthesia recommendations are given for patients undergoing surgery with carcinoid syndrome.

A prospective study of carcinoid crisis with no perioperative octreotide.

BACKGROUND: Carcinoid crises, defined as the sudden onset of hemodynamic instability in patients with neuroendocrine tumors undergoing operation, are associated with significantly increased risk of postoperative complications. Octreotide has been used prophylactically to reduce crisis rates as well as therapeutically to treat crises that still occur. However, studies using octreotide still report crisis rates of 3.4% to 35%, leading to the questioning of its efficacy. METHODS: Patients with neuroendocrine tumors undergoing operation between 2017 to 2020 with no perioperative octreotide were prospectively studied. Clinicopathologic data were compared by χ2 test for discrete variables and by Mann-Whitney U test for continuous variables. RESULTS: One hundred and seventy-one patients underwent 195 operations. Crisis was documented in 49 operations (25%), with a mean duration of 3 minutes. Crisis was more likely to occur in patients with small bowel primary tumors (P = .012), older age (P = .015), and carcinoid syndrome (P < .001). Those with crises were more likely to have major postoperative complications (P = .003). CONCLUSION: Completely eliminating perioperative octreotide resulted in neither increased rate nor duration compared with previous studies using octreotide. We conclude perioperative octreotide use may be safely stopped, owing to inefficacy, though the need for an effective medication is clear given continued higher rates of complications.

[Surgical treatment of pulmonary carcinoids - ten-year results]. / Chirurgische Behandlung von Lungenkarzinoiden - Zehnjahresergebnisse.

INTRODUCTION: Carcinoids are malignant neuro-endocrine tumours occurring in the bronchopulmonary location in about 25 %, and accounting for approximately 2 % of all pulmonary tumours. MATERIAL AND METHODS: Our retrospective analysis included 27  patients, 14  men and 13  women, mean age 58.4  years, treated from 2000 to 2009 for carcinoids in bronchopulmonary locations. The tumour manifested clinically in 52 % of the cases, the most common symptom being cough; one tumour manifested as carcinoid syndrome. All patients underwent fibrobronchoscopy that was positive in 20  cases (74.1 %). Pre-surgery histological diagnoses were made in 13  patients (48.1 %). Chest CT scans were carried out in 26  patients, and the investigation failed to detect the expected pathological process in 2  of the patients. Octreoscans were carried out in 12  patients, and were successful in identifying a primary neuroendocrine tumour in 75 %. RESULTS: All patients in the sample underwent rad-ical surgical therapy; the most common surgical procedure was lobectomy (70.4 %). Perioperative morbidity and mortality were zero. Typical carcinoids were found in 20  cases while 7  cases were atypical carcinoids, 20  tumours were located centrally. 74 % of the tumours were consistent with stage  I A disease. Mean follow-up period was 47 (range: 6-134)  months. Local recurrences were observed in 2  patients (7.4 %), but the tumour disseminated in 4  patients (14.8 %). Two patients (7.4 %) died during the follow-up period. Overall five-year survival in the sample was 92.3 %, 90.9 % in the typical carcinoid group and 100 % for atypical carcinoids. We found a statistically significant association between disease-free interval and histological type of the tumour; the risk of progression was 8  times higher in -patients with atypical carcinoids compared to patients with typical carcinoids (Log-Rank-Test: p-value = 0.0049). CONCLUSION: Radical surgical treatment of bronchopulmonary carcinoids is the optimum therapeutic approach that results in the best results both regarding perioperative morbidity and mortality and regarding long-term survival of the patients.

Anesthesia for Endocrine Emergencies.

Although endocrine emergencies are not common occurrences, their identification and careful perioperative management are of paramount importance for reduction of patient morbidity and mortality. The most common critical endocrine abnormalities are associated with functional tumors, such as pheochromocytomas, insulinomas, and carcinoid tumors, leading to carcinoid syndrome, abnormal thyroid function, or disturbances in the hypothalamus-pituitary-adrenal axis, causing adrenal insufficiency. This article aims to discuss the pathophysiology, diagnosis, and perioperative management of pheochromocytomas, hyperthyroidism, hypothyroidism, adrenal insufficiency, carcinoid disease, and insulinomas.

Long-term survival in a patient with carcinoid syndrome receiving treatment for Zollinger-Ellison syndrome.

A case of carcinoid syndrome in a patient receiving treatment for a malignant non-B-cell tumor of the pancreas is presented, and a survival of >14 years is noted. The probable cause is discussed. The literature is reviewed. It was found that such a presentation is rare, and long-term survival in these cases exceedingly rare.

Quadruple valve surgery in carcinoid heart disease.

We describe a 42-year-old male with primary carcinoid tumor of the ileum, secondary liver metastases, and subsequent severe carcinoid heart disease with quadruple valve involvement. The patient underwent tricuspid and pulmonic bioprosthetic valve replacement, mitral and aortic valve reconstruction. Transthoracic echocardiography at 25 months showed competent mitral and aortic valves with only mild regurgitation. Valve reconstruction is rarely performed in patients with carcinoid heart disease. However, in selected cases it is a valuable alternative technique with good mid-term outcome.

Successful double bioprosthetic valve replacement in a 64-year-old man with carcinoid syndrome and bipolar disorder: case report.

The case is described of a 64-year-old man with bipolar disorder and severe carcinoid heart disease who required a double valve replacement. Multidisciplinary team involvement and extensive preoperative investigations resulted in a successful regimen which prevented reactivation of carcinoid syndrome and avoided the serious side effects of lantreotide and lithium therapy. In addition, two bioprosthetic valves were used, thereby avoiding the potential complications of anticoagulation in a patient with known hepatic metastases.

[Radical surgical treatment of a new neuroendocrine tumor of the ileocecal valve with liver metastasis and carcinoid syndrome]. / Tratamiento quirúirgico radical de un tumor neuroendocrino de la válvula ileocecal con metástasis hepáiticas y síndrome carcinoide.

BACKGROUND: Neuroendocrine tumors are rare neoplasms which have a slow growth pattern. When liver metastases are diagnosed, treatment is controversial and it is focused in symptomatic control. AIM: To present a patient with a neuroendocrine tumor that arised from the ileocecal valve and it was diagnosed with carcinoid syndrome and treated with radical liver and colonic resection. A discussion of the different forms of treatment is presented. CASE REPORT: A 41 year-old woman was sent to our hospital with liver metastases and carcinoid syndrome from a neuroendocrine tumor of the ileocecal valve for treatment. Right colectomy, right hepatic trisectionectomy and radiofrequency ablation of two left sided lesions was performed as a first procedure. A second procedure was performed two months afterwards when resection of residual left sided lesions was done. After a two year follow up a 2 cm residual liver lesion was diagnosed and percutaneous radiofre-quency ablation was done. After four years of follow up the patient has been asymptomatic without tumor recurrence CONCLUSIONS: Radical surgical treatment of neu-roendocrine tumors controls symptomatology, improving quality of life and survival. However, this treatment should only be performed in a well selected group of patients.

[Surgical treatment of gastric, enteric, and pancreatic endocrine tumors Part 1. Treatment of primary endocrine tumors]. / Traitement chirurgical des tumeurs endocrines gastro-entéro-pancréatiques.

Endocrine tumors (ET) of the digestive tract (formerly called neuroendocrine tumors) are rare. They are classified into two principal types: gastrointestinal ET's (formerly called carcinoid tumors) which are the most common, and pancreaticoduodenal ET's. Functioning ET's secrete polypeptide hormones which cause characteristic hormonal syndromes. The management of ET is multidisciplinary. Poorly-differentiated ET's have a poor prognosis and are treated by chemotherapy. Surgical excision is the only curative treatment of well-differentiated ET's. The surgical goals are to: 1. prolong survival by resecting the primary tumor and any nodal or hepatic metastases, 2. control the symptoms related to hormonal secretion, 3. prevent or treat local complications. The most common sites of gastrointestinal ET's ( carcinoids) are the appendix and the rectum; these are often small (<1 cm), benign, and discovered fortuitously at the time of appendectomy or colonoscopic removal. Ileal ET's, even if small, are malignant, frequently multiple, and complicated in 30-50% of cases by bowel obstruction, mesenteric invasion, or bleeding. The carcinoid syndrome (consisting of abdominal pain, flushing, diarrhea, hypertension, bronchospasm, and right sided cardiac vegetations) is caused by the hypersecretion of serotonin into the systemic circulation; it occurs in 10% of cases and is usually associated with hepatic metastases. More than half of the cases of pancreatic ET are non-functional. They are usually malignant and of advanced stage at diagnosis presenting as a palpable or obstructing mass or as liver metastases. Insulinoma and gastrinoma (cause of the Zollinger-Ellison syndrome) are the most common functional ET's. 80% are sporadic; in these cases, tumor size, location, and malignant potential determine the type of resection which may vary from a simple enucleation to a formal pancreatectomy. In 10-20% of cases, pancreaticoduodenal ET presents in the setting of multiple endocrine neoplasia (NEM type I), an autosomal-dominant genetic disease with multifocal endocrine involvement of the pituitary, parathyroid, pancreas, and adrenal glands. For insulinoma with NEM-I, enucleation of lesions in the pancreatic head plus a caudal pancreatectomy is the most appropriate procedure. For gastrinoma with NEM-I, the benefit of surgical resection for tumors less than 2-3 cm in size is not clear. The lesions are frequently small, multiple, and widespread and recurrence is frequent after excision. The long-term prognosis is nevertheless fairly good. But the eventual development of liver metastases which are the most common cause of mortality still argues for an aggressive surgical approach in the early stages of the disease.