Search

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., (© 2024. Society of Surgical Oncology.)

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., Competing Interests: Name: Bryan Wiederholt, DNAP, CRNA Contribution: This author made significant contributions to the conception, synthesis, writing, and final editing and approval of the manuscript to justify inclusion as an author; this author is the corresponding author. Disclosures: None. Name: Timothy Gengler, DNAP, CRNA Contribution: This author made significant contributions to the conception, synthesis, writing, and final editing and approval of the manuscript to justify inclusion as an author. Disclosures: None. Name: Jen Bendure, DNP, CRNA Contribution: This author made significant contributions to the conception, synthesis, writing, and final editing and approval of the manuscript to justify inclusion as an author. Disclosures: None., (Copyright © by the American Association of Nurse Anesthetists.)

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 χ 2 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., (Copyright © 2021 Elsevier Inc. All rights reserved.)

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., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2019 Elsevier Inc. All rights reserved.)

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 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.

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., (© Georg Thieme Verlag KG Stuttgart ˙ New York.)

Neuroendocrine tumors of the small intestine have been diagnosed with increasing frequency over the past 35 years and presently account for approximately 2% of all gastrointestinal neoplasms. While most of these tumors are discovered incidentally during emergency laparotomy or in the clinical setting of unknown primary cancer with hepatic metastases, the growing awareness of this rare entity and improved diagnostic methods promote earlier diagnosis. The classical carcinoid syndrome with flush, diarrhea and cardiac strain is observed only in 20-30% of patients. The clinical symptoms necessitate a special preoperative preparation of the patient including evaluation of cardiac function.Prospective studies assessing the efficacy of surgical treatment strategies for neuroendocrine neoplasms of the small intestine do not exist. However, retrospective studies have demonstrated that curative as well as palliative resection of the primary tumor improves the prognosis and the quality of life of patients. Besides limited resection of the small bowel in order to avoid postoperative short bowel syndrome an effective clearance of the regional lymph nodes is essential. A primary tumor site in the terminal ileum requires dissection of the lymph nodes on the right side of the ileocolic artery which usually implies an additional resection of the right colon. In cases of a primary tumor site located in the lower ileum up to the distal jejunum, a cone-shaped resection of the mesenterium of the small bowel with extension of lymphadenectomy into adjacent segments with preservation of vascularization is performed.

The incidence of colorectal neuroendocrine tumors (NETs) is rising in developed countries primarily as a result of increased incidental detection by endoscopy and probably also due to a more adequate diagnosis according to the WHO classification. Less than 1% of colorectal NETs produce serotonin so that such tumors are practically never associated with a hormonal carcinoid syndrome. An exact clinico-pathological staging is of paramount importance for the therapeutic strategy and comprises the classification of the tumor type (well or poorly differentiated) and the assessment of established prognostic risk factors (depth of infiltration, vascular invasion, lymph node and distant metastases). Poorly differentiated colorectal NETs often present in an advanced, metastatic state, where surgical therapy is basically palliative. Well-differentiated tumors larger than 2 cm have a high risk of metastatic spread and should be treated as adenocarcinomas by radical oncological surgical resection. This applies to the majority of colon NETs. Tumors smaller than 1 cm, mainly locacted in the rectum, only rarely metastasize and are usually accessible for endoscopic treatment or transanal local surgery. Tumors between 1 and 2 cm in size have an uncertain prognosis and additional risk factors and co-morbidities of the patient have to be considered for a suitable, multidisciplinary therapeutic decision.

Carcinoid tumors are uncommon, slow-growing neoplasms. These tumors are capable of secreting numerous bioactive substances, which results in significant potential challenges in the management of patients afflicted with carcinoid syndrome. Over the past two decades, both surgical and medical therapeutic options have broadened, resulting in improved outcomes. The pathophysiology, clinical signs and symptoms, diagnosis, treatment options, and perioperative management, including anesthetic considerations, of carcinoid syndrome are presented., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Pulmonary carcinoid tumors are rarely associated with symptoms of the carcinoid syndrome, such as flushing and diarrhea. When present, these symptoms virtually always represent extensive hepatic metastases. In this article we describe the presentation, perioperative management, and presumed mechanisms of a patient with a localized pulmonary carcinoid with associated chronic diarrhea that resolved after operation., (Copyright (c) 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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.

Background: Metastatic neuroendocrine tumors (NETs) can present with complications of gastrointestinal tract obstruction or ischemia and carcinoid syndrome (CS). The purpose of this study was to assess whether aggressive surgical intervention of metastatic NETs provides effective palliation from these symptoms., Methods: Sixty-six patients with metastatic gastrointestinal tract NETs that presented with either CS and/or obstructive symptoms were retrospectively reviewed. All patients were managed according to a standardized protocol that involved initial surgical resection of regional and/or hepatic disease followed by appropriate medical therapy., Results: Symptoms of obstruction or ischemia were present in 24 patients (36%) and CS in 56 (85%). All patients with obstructive symptoms undergoing operative therapy had complete symptomatic relief. Hepatic cytoreduction was performed in 30 (45%). Overall symptoms of CS improved in 42 patients (75%); 86% of patients that underwent hepatic cytoreduction and 64% of those receiving medical therapy alone (P = .064). Postoperative morbidity was 22% with no mortality. Mean follow-up was 47 months (range, 6-156). Overall 5-year survival rate was 74%., Conclusions: Surgical resection is highly effective in relieving symptoms of intestinal obstruction and ischemia. Hepatic cytoreduction seems to enhance the ability to control the symptoms of carcinoid syndrome. A surgically aggressive approach in patients with metastatic NETs provides effective palliation in carefully selected patients.

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.

Carcinoid heart disease is a rare form of valvular heart disease. The management of these patients is complex, as the systemic malignant disease and the cardiac involvement have to be considered at the same time. Progress in the treatment of patients with carcinoid disease has resulted in improved symptom control and survival. Development and progression of carcinoid heart disease are associated with increased morbidity and mortality. In patients with severe cardiac involvement and well-controlled systemic disease, cardiac surgery has been recognized as the only effective treatment option. Valve replacement surgery may not only be beneficial in terms of symptom relief, but may also contribute to the improved survival observed over the past 2 decades in patients with carcinoid heart disease. Early diagnosis and early surgical treatment in appropriately selected patients may provide the best results. In this article, we review the current literature regarding the biology, diagnosis, treatment, and prognosis of carcinoid heart disease.

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.

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.

Background: Patients with metastatic neuroendocrine cancers to the liver often present with disabling endocrinopathies and pain associated with bulky disease. Quality of life for these patients is poor and can require long-term therapy with somatostatin analogs for control of their symptoms. Alternative therapies to decrease tumor burden and subsequent hormone release have been investigated. Of these, cytoreductive surgery was found to have the most consistent and profound impact on symptom regression and overall survival., Methods: Several cases are reported that illustrate an aggressive multimodality approach in the treatment of metastatic neuroendocrine cancers to the liver. The literature is reviewed and the role of cytoreductive surgery in the management of hepatic neuroendocrine metastases is discussed., Results: Cytoreductive surgery can be performed safely with minimal morbidity and mortality. Regression of symptoms occurs in the majority of patients and survival is prolonged., Conclusions: Surgical intervention as part of an aggressive multimodality treatment plan results in improved outcomes for patients with advanced hepatic metastases of neuroendocrine origin. Future directions may include earlier surgical intervention with adjuvant therapies reserved for aggressive recurrent disease.

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.

Carcinoid syndrome creates many challenges during anaesthesia, including hypertension, hypotension and bronchospasm. These challenges are less common and less severe after the routine use of octreotide. We describe the use of remifentanil as part of the anaesthetic management of a 67-yr-old man undergoing resection of a carcinoid tumour of the terminal ileum. The combination of perioperative octreotide administration, intraoperative remifentanil infusion and sevoflurane anaesthesia, with postoperative epidural analgesia proved satisfactory. We review the recent literature and suggest that remifentanil is a useful addition to the armamentarium of the anaesthetist in the management of a patient with carcinoid syndrome.

Objective: The purpose of this study was to evaluate the efficacy of percutaneous radiofrequency ablation of hepatic neuroendocrine metastases for symptomatic relief of neuroendocrine syndromes., Conclusion: Percutaneous radiofrequency ablation, a minimally invasive technique, is an effective and safe way to reduce systemic symptoms in patients with hepatic metastases from neuroendocrine neoplasms.

Carcinoids are rare tumors of enterochromaffin cells. The carcinoid-syndrome most often occurs with hepatic metastases of carcinoids and is evoked by release of serotonin and other vasoactive substances, leading to typical symptoms such as hyper- or hypotension, bronchospasm, tachycardia, diarrhoe, and flushing. A lethal perioperative "carcinoid-crisis" may occur. We report on a patient with carcinoid-syndrome due to liver metastases undergoing hemihepatectomy. For prophylaxis, the patient preoperatively received H 1- and H 2-histamine-receptor antagonists, corticosteroids, and a continuous somatostatin infusion. Besides monitoring cardiovascular variables we intermittently measured serotonin- and catecholamine concentrations. Initially increased serotonin concentration decreased during the course of anaesthesia. However, it increased again during liver resection despite Pringle's manoeuvre and was associated with a decrease in arterial pressure, systemic vascular resistance, and central venous pressure. Hypotension was treated by volume and noradrenaline infusion. Thus, despite somatostatin infusion serotonin release is still possible, especially during surgical manipulation.

Duodenal carcinoid tumors are uncommon. It is not known whether they behave more like carcinoid tumors in the appendix (indolent course) or those in the ileum (often virulent)-crucial information for determining the need for radical resection. A retrospective review at our tertiary referral center (from 1976 to 1999) identified 27 patients with primary duodenal carcinoid lesions, excluding functional islet cell tumors. Endoscopic biopsy provided the diagnosis in 78% of patients. Treatment was by endoscopic excision (n = 11), transduodenal excision (n = 8), pancreaticoduodenectomy (n = 3), segmental distal duodenectomy (n = 2), or palliative operation (n = 2). One patient did not undergo operation because of comorbidity. Eighteen of 19 patients with tumors smaller than 2 cm remained disease free after local (endoscopic or transduodenal) excision. The exception was a patient with a small periampullary carcinoid lesion. In contrast, all four patients with carcinoid tumors 2 cm or larger who were resected for cure developed a recurrence (2 to 9 years postoperatively). We conclude that duodenal carcinoid tumors smaller than 2 cm may be excised locally; to ensure complete resection we recommend open transduodenal excision for tumors between 1 and 2 cm. Endoscopic follow-up is indicated. It is unclear whether patients with larger tumors benefit from more aggressive locoregional resection. Ampullary/periampullary carcinoid tumors should be considered separately, as their behavior is unpredictable.

Introduction: Carcinoid tumors are the most common neuroendocrine tumors of the gastrointestinal tract. Surgical treatment and prognosis depend on the location of the tumor., Method: Between 01.01.1985 and 31.12.1999 25 patients with neuroendocrine tumors of the gastrointestinal tract or their metastases were treated in our institution. The records of these patients were reviewed retrospectively. Patients still alive were reexamined clinically., Results and Conclusions: The most frequent primary sites were the ileum and jejunum (36%), appendix (36%), stomach (12%), pancreas (8%), colon (4%) and bronchus with hepatic metastasis (4%). A malignant carcinoid syndrome was present in 8 patients. In patients with neuroendocrine tumors, curative, radical tumor removal should be attempted. Some patients with advanced disease needed some surgery for tumor debulking and resection of metastases. In non-resectable liver metastases hepatic arterial chemotherapy and chemoembolization after implantation of port catheters seem to be very beneficial therapeutic options. A fixed part of the therapeutic regime in progressive disease is adjuvant chemotherapy with 5-fluorouracil and streptozotocin and symptomatic therapy with octreotide.

Background: The purpose of this study was to assess the behaviour of atypical carcinoids operated at our Department in the period 1977-1998 and to review the last 19 cases according to Capella's classification (1994), indicating the most adequate surgical approach., Methods: On the basis of anatomo-pathological characteristics, we have reviewed surgical treatment and outcome in 46 patients, submitted in the last 22 years to surgical resection for neuroendocrine neoplasms., Results: 5-year survival is 77.2%; 10-year survival is 53.2%. Lymph node metastases are also important for survival, but less than the histotype. The review of our last six years' series, according to Capella's classification, of 19 patients affected by so-called atypical carcinoids revealed that: 5 were well differentiated neuroendocrine tumors (WDNT), 12 were well differentiated neuroendocrine carcinomas (WDNC), 2 were small cell neuroendocrine carcinomas (SCLC). The 5-year overall survival of our cases is 78%, for the WDNT 100%, for WDNC 81.2%. Of the 2 patients with SCLC, one survived 2 months; the other is still alive 5 months after surgery., Conclusions: The authors conclude that 5-year and 10-year survival are strongly related to the histological type of neuroendocrine neoplasm and to the presence of lymph node metastases. Capella's anatomo-pathological classification helps to give a more accurate prognosis for survival in so-called "atypical carcinoids". If the neoplasm is malignant, the authors recommend radical resection, if possible.

This study was undertaken to provide investigators working in this particular research field with extensive and useful basic information based on an analysis of a large reliable series of cases regarding tracheal and bronchopulmonary carcinoids and their atypical varieties. A statistical evaluation was carried out which included a total of 1,875 patients with tracheal and bronchopulmonary carcinoids; these were divided into two series, one of 1,595 patients with typical carcinoids and the other of 280 with atypical varieties. These two series were compared regarding various aspects, which included the male to female ratio, age distribution, clinical manifestations, successful preoperative diagnosis, diagnostic accuracy of representative procedures, sites of involvement, tumor size distribution, metastases, carcinoid syndrome, serotonin activity in patients with or without the syndrome, immunohistochemistry, electron microscopy, and postoperative prognosis of the patients. The comparative analyses between the two series disclosed statistically significant differences (p<0.01) regarding various viewpoints; among others, such a difference was proved in the average age, sites of involvement in the lung (central or peripheral), rates and sites of metastases, adrenocorticotrophic hormone (ACTH) production, the association rate of the carcinoid syndrome, and postoperative 5-year and 10-year survival rates (93.3% and 82.1% for the typical carcinoid series versus 68.8% and 58.6% for the atypical variety series: p<0.0001). The postoperative 5-year and 10-year survival rates in the other two groups of patients with or without metastases were likewise calculated and showed a statistically significant difference (72.8% and 52.8% for the former versus 98.3% and 95.4% for the latter: p<0.0001).

No statistical evaluation of patients with carcinoid syndrome in a reliable number of cases has been available in the past 35 years. To update our knowledge about the syndrome, we have evaluated from various clinicopathologic viewpoints a large series of patients with the syndrome reported up to date. The data of 748 patients with the syndrome were collected from 8876 carcinoid patients reported in the literature and analyzed by the Gut-Pancreatic Endocrinoma Analyzing System (the Niigata Registry). The results are summarized as follows. 1) The patients with the syndrome had a tendency to be older than those without it. 2) The incidence of the syndrome was 8.4% of 8876 carcinoid patients. 3) Serotonin activities were extremely high in patients with the syndrome as compared to those without it (91.7% versus 26.6%). 4) The rate of metastases was higher in patients with the syndrome than in those without it (84.8% versus 29.2%), and higher in the liver than in lymph nodes among patients with the syndrome (73.4% versus 37.4%). 5) Flushing and carcinoid heart as most specific clinical manifestations of the syndrome were recorded at 78.3% and 17.4%, respectively. 6) The 5-year survival rate after resection of primary lesions was 76.0% of 304 patients with the syndrome, lower in patients with digestive carcinoids than in those with extradigestive lesions (67.2% versus 88.7%). It is expected that the results obtained in the present evaluation on patients with carcinoid syndrome will provide investigators active in this specialized field with useful and extensive information for their future activities.

Tumor debulking can greatly improve quality of life for patients with malignant carcinoid syndrome, but hepatic cytoreduction is confounded by carcinoid heart disease, which can cause postsinusoidal portal hypertension, thereby increasing the risk of death from hemorrhage during hepatic resection. We describe a patient with metastatic carcinoid syndrome and carcinoid heart disease who had repair of his carcinoid heart disease and, after improvement of right-sided heart function, had successful hepatic debulking of carcinoid metastases.

This case report describes an unusually large islet cell tumour of the pancreas presenting clinically with extensive metastases in the liver. The patient involved was a 55-year-old woman. The leading symptom was severe, hardly tractable diarrhoea. Histological examination including immunohistochemistry and measurements of the proliferation index revealed a probable malignant Vipoma of low grade. It was treated by simultaneous R0 resection of the tumour masses; the postoperative course was unremarkable. This outcome is seen as a strong argument in favour of a radical surgical approach even if there is significant metastatic disease in the liver. The advantage of intraoperative ultrasound is discussed along with aspects of tumour classification and alternative therapeutic modalities.

A patient who had previously undergone ileal resection and liver transplantation for a gastroenteropancreatic (GEP) tumor was evaluated with somatostatin receptor scintigraphy (SRS) using 111In-DTPA-D-Phe1-pentetreotide. Eighteen months after surgery, during follow-up procedures, conventional imaging techniques (ultrasound, computed tomography, magnetic resonance imaging) only showed a relapse in the gastropancreatic lymph nodes, while SRS demonstrated skeletal spread. This case report emphasizes the clinical impact of SRS on the management of patients affected by neuroendocrine gastroenteropancreatic tumors.