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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.)

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.

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.

A 34-year-old man with carcinoid heart disease underwent tricuspid valve replacement and pulmonary valvotomy with enlargement of the right ventricular outflow tract in October, 1972. He has no cardiac symptoms eight years after operation. Only four previous successful valve replacements in patients with this condition have been reported.

Two female patients with carcinoid heart disease, ages 56 and 32 years, underwent pulmonic valve resection surgery and tricuspid valve replacement with a porcine bioprosthesis. Preoperatively, both patients were in function class 4 with severe right-side congestive failure and signs of tricuspid regurgitation and pulmonic stenosis. Both underwent surgery for porcine tricuspid valve replacement (33 and 31 mm valves) and pulmonic valve resection. Postoperatively, both patients had only minimal symptoms, including trace ankle edema and soft pulmonic murmurs, despite persistence of the systemic symptoms of carcinoid syndrome. Pre- and postoperative catheterization data documented hemodynamic improvements. One patient eventually died of hepatic failure due to metastatic disease. At autopsy, her bioprosthesis was free of carcinoid valvular changes.

The carcinoid syndrome is a rare clinical entity, the unique manifestations of which continue to excite the interest of physicians. Despite a common origin from neural crest tissue, the tumors are partially differentiated, as evidence by the different secretory products of foregut, midgut, and hindgut carcinoids. They also differ in their ability to metastasize, thus presenting an even more varied clinical picture. The prognosis of patients with carcinoid syndrome varies with the origin of the tumor and extent of metastases. The management of patients with carcinoid syndrome is difficult. Despite an understanding of the neurohormones that carcinoid tumors secrete, their various antagonists and inhibitors have been only partially successful in providing symptomatic relief. Carcinoid heart disease represents the most intriguing aspect of this syndrome. Although valvular dysfunction most often coexists with flushing and diarrhea, the findings of tricuspid regurgitation or stenosis occasionally provide the first clue to the presence of the disease. Despite intensive research, the definite etiology of these valvular lesions has not been established. A small group of patients has been managed by valve replacement. While surgical treatment has been successful in improving hemodynamics in most of these patients, it is expected to prolong life only in those without extensive liver metastases. In patients with extensive metastatic disease, one must carefully consider whether the risks and trauma of cardiac surgery for palliation are justified.