Carcinoid heart disease and carcinoid syndrome: successful surgical treatment

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.     

Carcinoid syndrome from gastrointestinal carcinoids without liver metastasis.

Although patients with bronchial and ovarian carcinoid tumors can develop the carcinoid syndrome (diarrhea and/or flushing) in the absence of hepatic metastasis, it is believed that development of the carcinoid syndrome in patients with carcinoid tumors of gastrointestinal origin occurs only after the patient has hepatic metastasis. This is explained by hepatic inactivation of most of the serotonin in the portal circulation or by the fact that hepatic metastases are larger than the primary tumor in the gastrointestinal tract. Three patients with ileal and jejunal carcinoid tumors who developed the carcinoid syndrome without obvious hepatic metastasis are described. Two of the patients had intra-abdominal, but extrahepatic, metastasis that probably drained directly into the systemic circulation. The third patient had an ileal carcinoid with clinical involvement limited to adjacent mesenteric lymph nodes. Following resection of her tumor, her urinary 5-HIAA excretion and platelet serotonin level returned to normal, and her attacks of carcinoid flushing virtually ceased. She has occasional spells of "blushing" that are thought to be benign; however, further close follow-up study will be needed to be certain that she is free of disease. It is suggested that each patient with the carcinoid syndrome be evaluated with CT and technetium-99 pertechnetate liver scans. If there is no liver involvement detected with these studies, one should consider hepatic arteriogram or laparotomy to determine if the patient's tumor might be totally resectable.     

Transatrial transcatheter tricuspid valve-in-valve implantation of balloon expandable bioprosthesis

Transcatheter valve-in-valve implantation into failing mitral and aortic bioprosthetic valves have been reported. This strategy avoids performing high-risk repeat cardiac surgery in elderly patients with multiple comorbidities. Tricuspid valve-in-valve implantation has not been described. We report a case of failing bioprosthetic tricuspid valve in a 48-year-old woman with carcinoid syndrome. We attempted a transatrial transcatheter approach and we successfully deployed a 26-mm Edwards Sapien balloon expandable bioprosthesis (Edwards Lifesciences, Irvine, CA) into a severely stenotic tricuspid bioprosthesis. This case demonstrates the technical feasibility and safety of this approach. Therefore, tricuspid valve-in-valve implantation may be a viable treatment alternative in carefully selected patients.     

Orthotopic liver transplantation for carcinoid tumour metastatic to the liver: anesthetic management

PURPOSE: To report the anesthetic management of a patient with carcinoid tumour metastatic to the liver who presented for orthotopic liver transplantation. Anesthetic implications of metastatic carcinoid tumour on liver transplantation and the use of octreotide are discussed. CLINICAL FEATURES: A 51-yr-old woman with intestinal carcinoid tumour metastatic to the liver presented for orthotopic liver transplantation, a recent treatment option for patients with extensive hepatic carcinoid metastases and disabling symptoms unresponsive to conventional therapy. Despite continuous administration of the somatostatin analogue octreotide via a hepatic artery infusate pump, the patient suffered from daily break through symptoms, which included flushing, palpitations, paroxysmal hypertension, and dyspnea. The patient presented to the operating room with sinus tachycardia and severe arterial hypertension. Octreotide and phentolamine were used to prevent further mediator release and to control the paroxysmal hypertension. Midazolam, fentanyl, thiopental, succinylcholine, vecuronium, and isoflurane were used to induce and maintain anesthesia safely. An intravenous octreotide infusion was initiated after induction and continued throughout the case. Infrequent and non-threatening peaks in arterial blood pressure were readily treated with small intravenous doses of vasoactive drugs and octreotide. No other manifestations of the carcinoid syndrome occurred. The patient had an uneventful recovery and was discharged on postoperative day #6. CONCLUSION: The patient safely underwent orthotopic liver transplantation for treatment of symptomatic carcinoid tumour metastatic to the liver. The anesthetic management followed recent recommendations favouring the use of octreotide to prevent patients from becoming symptomatic. Outlined dosing regimen for octreotide provided satisfactory hemodynamic stability.     

Radiofrequency ablation treatment of refractory carcinoid hepatic metastases

BACKGROUND: Our institution has experienced excellent success using hepatic artery embolization for treating symptoms and slowing tumor progression for patients with unresectable hepatic metastases for carcinoid tumors. Our previous treatment strategies used hepatic artery embolization alone, examining control of symptoms and dependence on octreotide therapy. However, some patients exhibit hepatic metastases that are unresponsive to embolization. This report describes the use of radiofrequency ablation (RFA) as salvage treatment for these refractory metastases. METHODS: Thirteen patients with unresectable bilobar hepatic metastases from biochemically confirmed carcinoid tumors were treated with selective hepatic artery embolization using Lipiodol/Gelfoam between 1994 and 2000. Three patients developed symptoms resistant to embolization treatment resulting from progression of existing metastases or development of new metastases. These patients underwent surgical exploration and intraoperative ultrasound of their refractory lesions, followed by treatment with RFA. Tumor size, symptoms of carcinoid syndrome, and octreotide requirements were monitored postoperatively. RESULTS: Median follow-up for the three patients treated with RFA was 6 months. During the first 3-month interval following RFA, all three patients demonstrated decrease in the size of treated lesions. Using our previously developed symptom scoring system, all three patients demonstrated decreased symptoms following treatment. One patient was able to discontinue octreotide treatment, and the other two patients required decrease octreotide dosages. CONCLUSIONS: This study demonstrates that utilization of RFA treatment for carcinoid metastases refractory to hepatic artery embolization may represent a useful adjunct for symptomatic control, decreased octreotide dependence, and slowing of disease progression.     

Treatment of the carcinoid syndrome by hemihepatectomy and radical excision of the primary lesion

Two patients with the carcinoid syndrome whose symptoms were difficult to control with drugs and who had early signs of right heart valvular changes are reported on. Left hemihepatectomy was believed to be justified; resection of the left half of the liver, a tumor of the small bowel, and lymph node secondaries in one patient and resection of a tumor of the small bowel and the quadrate hepatic lobe in another patient resulted in symptom-free periods of twelve and four months, respectively. These cases support the adoption of more aggressive surgical treatment of this manifestation of carcinoid tumors.     

Quadruple valve surgery in carcinoid heart disease

Abstract   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.     

Primary hepatic carcinoid tumor in children

Primary carcinoid tumors of the liver are rare, with fewer than 60 cases currently reported in the English literature. We present the evaluation and management of a solid hepatic tumor in a 14-year-old boy. Intraoperative biopsy was indeterminant for malignant potential, and the patient underwent complete resection by left hepatic lobectomy. Final histopathologic evaluation of the mass revealed a carcinoid tumor.Extensive endoscopic and radiologic workup revealed no other primary source. The patient recovered well from surgery and is currently free of disease 32 months after initial resection. Review of the literature suggests that primary hepatic carcinoid tumors are particularly rare in children. As the liver is frequently a site for carcinoid metastasis from the gastrointestinal tract, any patient with a suspected primary hepatic carcinoid tumor must undergo an extensive search for an extrahepatic primary site. These tumors are typically indolent but may metastasize. In addition, medical therapy is of limited benefit in reducing tumor bulk. The mainstay for treatment of primary hepatic carcinoid tumors is surgical resection, and these tumors carry a more favorable prognosis than other primary hepatic malignancies and metastatic carcinoid. Follow-up is long-term, as these tumors can recur many years after initial resection.     

Metastatic Carcinoid to the Liver Treated by Hepatic Dearterialization

A historical review of work bearing on the rationale for hepatic artery ligation in the treatment of malignant disease in the liver is presented, particularly the early work showing that malignant disease of the liver is supplied primarily by the arterial circulation and not by the portal. A review is presented of some clinical reports dealing with the effectiveness of this approach and our own limited experience with three cases presented briefly. The major emphasis is placed on the metabolic, arteriographic, microscopic and clinical studies on one patient with the carcinoid syndrome who was followed carefully over several years, treated initially by hepatic resection, later by chemotherapy and finally by hepatic dearterialization as the tumor re-grew and the carcinoid syndrome again became incapacitating. Clinical remissions was documented by changes in the excretion of 5-hydroxyindole- acetic acid and by comparison between pre- and post-op arteriography and by microscopic sections taken at re-exploration for drainage of a necrotic area in the liver.     

Facial flushing due to recurrent bronchial carcinoid

Carcinoid syndrome is quite a rare presentation in bronchial carcinoid. A review of the literature suggests a figure of 2% to 7% in various series. This is usually associated with recurrent carcinoid tumor in the presence of hepatic metastasis. We discuss a patient who presented with flushing attacks 13 years after a left pneumonectomy. Further investigation found that the patient had recurrence at the pneumonectomy stump and in subcarinal lymph nodes.     

Symptomatic treatment of the malignant carcinoid syndrome with octreotide

Intractable diarrhoea and flushing due to the malignant carcinoid syndrome is seldom relieved by conservative medical treatment. Octreotide (Sandostatin; Sandoz) is a long-acting analogue of somatostatin and a powerful inhibitor of endogenous peptide release. A patient with severe diarrhoea and flushing due to the malignant carcinoid syndrome, in whom symptomatic control with octreotide was achieved, is described, and the value of octreotide treatment in the malignant carcinoid syndrome is discussed.     

Laparoscopic cryoablation of a metastatic carcinoid tumor

Carcinoid tumors are rare, slow-growing neuroendocrine neoplasms that can cause a carcinoid syndrome. The majority of carcinoid syndromes are the result of multiple hepatic metastases and are usually unresectable. Medical therapy has not proven effective and often causes intolerable side effects. Cryoablation has emerged as a promising treatment for various hepatic lesions when resection is not an option and medical therapy has been exhausted. With the addition of laparoscopy, surgeons now possess a new technique to treat primary and various secondary liver lesions. We report for the first time laparoscopic cryoablation for the treatment of a carcinoid metastatic to the liver.     

A Carcinoid Saga

The case is reported of a patient who developed the carcinoid syndrome when metastases appeared in the liver after resection of the primary ileal tumour. A right hepatic lobectomy was followed by complete remission of symptoms for 15 months, even though local removal of further liver metastases became necessary because of biochemical evidence of recurrence. Finally, systemic spread of the tumour became apparent. Two years after the original resection the patient appeared to be in the terminal phase of the disease. The protean manifestations of the carcinoid syndrome are reviewed, and the conclusion is reached that surgical removal of liver metastases can produce worth-while palliation even in the presence of advanced disease. The importance of proper operative management, including anaesthesia, is stressed. More long-term study and the publication of complete case reports seem necessary to delineate the prognosis of the carcinoid syndrome when this is treated surgically.     

Resection of carcinoid tumors of the small intestine is still indicated in the presence of disseminated hepatic metastases]

The authors report their experience with resection of small bowel carcinoid tumors associated with diffuse hepatic metastases. Nine patients were operated during the last ten years. A carcinoid syndrome was present biologically in every case and clinically in 8 cases. The tumor was removed in every case, either by right hemicolectomy (n = 6), or by small bowel resection (n = 3). Postoperative mortality and morbidity were nil. Adjuvant therapy included hepatic chemo-embolization and long-term therapy by a long-acting somatostatin analog. Five patients died during the period of follow-up and 4 patients were alive at the end of the study. Resection of small bowel carcinoid tumor is indicated even when the liver is involved by diffuse metastases to prevent the risk of small bowel occlusion, and to promote hepatic chemoembolization as subsequent therapy against the carcinoid syndrome and metastatic spread. Moreover, the procedure can participate in the selection of cases for orthotopic liver transplantation, when conservative therapy fails to control the carcinoid syndrome.     

Somatostatin, anaesthesia, and the carcinoid syndrome

A patient with carcinoid syndrome on long-term antiserotonin therapy with parachlorophenylalanine, experienced a flushing attack with hypotension during the prophylactic administration of aprotonin prior to the induction of anaesthesia. When she was subsequently prepared with a long-acting somatostatin analogue, octreotide (Sandostatin, Sandoz SMS 201-995), plasma levels of tumour-released hormones were reduced and anaesthesia for resection of hepatic metastases was uneventful. The advantages of an anaesthetic approach based on inhibition of carcinoid tumour activity, rather than antagonism of released hormones, are discussed.     

Peri–operative management of carcinoid syndrome using ketanserin

This report describes the successful use of ketanserin, a 5-HT2 receptor antagonist, for the acute control of systemic blood pressure in a patient with the carcinoid syndrome, undergoing hepatic artery embolisation. Serial measurements of plasma 5-hydroxyindoles, platelet 5-hydroxytryptamine and plasma catecholamines are also given.     

Perianesthetic management of carcinoid syndrome: contribution of the bispectral EEG index

This case report described the perioperative management of a patient undergoing laparotomy for ileal carcinoid tumor with liver metastases and preoperative carcinoid syndrome. During surgery, bispectral index monitoring, combined with classical blood pressure and heart rate, allowed to discriminate hypertension related to the carcinoid syndrome, from hypertension reflecting an inadequate level of anaesthesia. Consequently, adequate treatment by a somastotatin analogue could be early administered.     

Status of ischemic therapy for hepatic tumors

The use of hepatic artery ligation or permanent dearterialization as the sole procedure for the palliation of patients with malignant hepatic tumors has no proved value. The combination with cytotoxic drug administration via the portal route may offer some advantage. The use of transient dearterialization with one longer ischemic period has been successful in the treatment of metastatic carcinoid disease with carcinoid syndrome but ineffective in the treatment of other hepatic tumors. New knowledge of the effects of transient ischemia on the formation of arterial collaterals and the pathophysiologic mechanisms in cellular injury has led us to further refinement of this therapeutic principle. The first results of repeated short periods of ischemia are promising and give some hope for the future palliation of this group of tumor patients.     

Carcinoid syndrome with myasthenia gravis

The anaesthetic management of a patient suffering from carcinoid syndrome and myasthenia gravis is described including the successful treatment of a carcinoid attack with intravenous aprotonin. The differences between myasthenia gravis and the myasthenic (Eaton-Lambert) syndrome are considered and the rationale for the choice of vecuronium as the muscle relaxant is discussed.     

Minute liver metastases from a rectal carcinoid: A case report and review

We here report a 43-year-old male patient with minute liver metastases from a rectal carcinoid. Hepatic nodules were diagnosed during surgery, although they were not diagnosed by preoperative computed tomography or ultrasound examination. The rectal carcinoid was resected together with liver metastases and the patient has had no disease recurrence for 5 years following postoperative treatment of hepatic arterial infusion chemotherapy (HAIC) using 5-fluorouracil (5-FU) and oral administration of 1-hexylcarbamoyl-5-fluorouracil (HCFU). In 2003, a health check examination indicated presence of occult blood in his stool. Barium enema study revealed a rectal tumor in the lower rectum and colonoscopy showed a yellowish lesion with a size of 30 mm in diameter. Pathological examination of the biopsy specimen indicated that the rectal tumor was carcinoid. Although preoperative imaging examinations failed to detect liver metastases, 2 min nodules were found on the surface of liver during surgery. A rapid pathological examination revealed that they were metastatic tumors from the rectal carcinoid. Low anterior resection was performed for the rectal tumor and the pathological report indicated that there were 4 metastatic lymph nodes in the rectal mesentery. The patient received treatment by HAIC using 5-FU plus oral administration of HCFU and survived for 5 years.We also review world-wide current treatments and their efficacy for hepatic metastases of carcinoid tumors.