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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:Carcinoid heart disease: a clinical pathologic, and therapeutic update. 704 96

While carcinoid tumors of the gastrointestinal tract continue to intrigue physicians and surgeons, the medical and surgical management of these tumors continues to evolve. Summarizing the current literature, patients should undergo local excision for incidental tumors of the stomach, rectum, and appendix, provided the tumor measures less than 2 cm in greatest dimension and does not demonstrate muscular invasion or lymph node metastases. Curative radical resection should be attempted, even in the presence of hepatic metastases, in patients with symptomatic or incidentally discovered carcinoid tumors of the small intestine, or in patients with large (> 2 cm) or invasive tumors. Palliative radical resection should be performed for patients with metastatic carcinoid tumors in light of the indolent tumor growth characteristics and the incapacitating symptoms related to tumor bulk and hormone production. Liver dearterialization should be considered only if resection cannot be safely accomplished. Carcinoid heart disease can be safely managed surgically and early intervention prior to the development of irreversible myocardial dysfunction is advocated. Medical therapy, primarily octreotide, should be offered to patients with disseminated tumors, and should be administered and available during the perioperative period to patients undergoing resection or liver dearterialization. With the use of new radioimmunoimaging techniques, earlier diagnosis, improved follow-up, and potentially targeted chemotherapy will be possible. As is evident, the treatment for gastrointestinal carcinoid tumors can be simple, as with small appendiceal tumors, or complex, as with disseminated small intestinal tumors. All surgeons should be familiar with the treatment options for these patients, as most can be treated by simple endoscopic excision or appendectomy. For the unusual patient with a disseminated carcinoid tumor, a multidisciplinary approach is required, combining the expertise of surgeons, oncologists, anesthesiologists, and radiologists for effective results.
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PMID:Surgical management of gastrointestinal carcinoid tumors. 787 84

Carcinoid heart disease occurs in about one third of patients with carcinoid syndrome, especially in those with ileal carcinoid and hepatic metastases. Patients with primary ovarian carcinoid tumor are extremely rare. In these circumstances, typical carcinoid cardiac lesions may develop unassociated with hepatic metastases, due to the venous drainage from the ovaries into the inferior vena cava of vasoactive released substances such as serotonin. The present report describes a woman with unrecognized primary ovarian carcinoid tumor, unexpectedly exhibiting heart failure. Diagnosis was performed on the basis of echocardiographic findings, occurrence of diarrhea and increased levels of 5-hydroxy-indoleacetic acid (5-HIAA). After complete surgical removal of the tumor, the patient was maintained under therapy with ACE-inhibitors and diuretics. Levels of 5-HIAA are still within normal range, there is regression of heart failure and echocardiographic findings are stabilized. These data confirm the importance of prompt diagnosis for a favorable prognosis of carcinoid heart disease.
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PMID:[Carcinoid heart disease and primary ovarian tumor]. 1202 91

A 68-year old woman was hospitalised because of isolated right heart failure. Doppler echocardiography revealed severe tricuspid regurgitation with thickened, shortened, hypomobile leaflets. Pulmonary valve was thickened with mild pulmonary regurgitation. Mitral and aortic valves were normal. The patient was finally diagnosed with carcinoid heart disease from an isolated ovarian carcinoid cancer without hepatic metastases. Ovarectomy was performed and the patient was considered cured of her cancer. Because of refractory right heart failure, she underwent tricuspid valve replacement with a bioprosthesis. Such cardiovascular manifestations are rarely the presenting symptoms of carcinoid disease. Carcinoid heart disease from ovarian primary cancer is exceptional. In this circumstance, carcinoid cardiac lesions may develop in the absence of hepatic metastases because the venous blood from the ovaries drains into the inferior vena cava without hepatic first past effect. Surgical resection of primary ovarian carcinoid tumor is often curative and the prognosis depends mainly on the cardiac condition. The diagnosis of carcinoid syndrome should be discussed in patients with organic tricuspid regurgitation without left valvular disease.
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PMID:[Isolated carcinoid tumor of the ovary disclosed by tricuspid insufficiency]. 1462 39

Progress in the medical and surgical management of patients with carcinoid disease has resulted in improved symptoms and survival. Carcinoid heart disease remains a major cause of morbidity and mortality among patients with malignant carcinoid syndrome. Limited medical treatment options are available for patients with symptomatic carcinoid heart disease. At the Mayo Clinic (Rochester, MN), we have taken an aggressive approach to severe valvular dysfunction from carcinoid heart disease. Patients with severe carcinoid heart disease currently are referred for cardiac operation when they develop cardiac symptoms, ventricular dysfunction, or (rarely) in anticipation of hepatic surgery. Surgical outcome depends on patient age and functional class at the time of cardiac surgery. Despite metastatic disease that limits longevity, cardiac surgical survivors usually demonstrate dramatic improvement in functional capacity. Cardiac surgery should be considered early for patients with symptomatic carcinoid heart disease and controlled carcinoid symptoms. An experienced medical, surgical, and anesthetic team approach to the patient with carcinoid heart disease is critical in order to provide state of the art management.
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PMID:Carcinoid heart disease. 1652 35

Most carcinoid tumors originate in the gut. Carcinoid heart disease typically occurs when tumor progression results in the formation of hepatic metastases, which allow vasoactive substances to reach the heart without being metabolized in the liver. Except for patients with primary ovarian carcinoid tumors, the occurrence of carcinoid heart disease without hepatic metastases has been reported only anecdotally. From a retrospective analysis of 265 patients, 4 patients were identified who developed carcinoid heart disease in the absence of liver metastases or primary tumors located in the ovaries. All 4 patients had metastases to the retroperitoneal lymph nodes and had carcinoid syndrome. The reasons for referral to cardiac evaluation by transthoracic echocardiography were findings on auscultation in 3 patients and exertional dyspnea in 1 patient. In conclusion, cardiac symptoms or findings on auscultation should prompt further evaluation by transthoracic echocardiography in these patients, although the classic prerequisites for development of carcinoid heart disease are lacking.
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PMID:Carcinoid heart disease in patients without hepatic metastases. 1722 38

Carcinoid heart disease (CHD) develops in serotonin-producing neuroendocrine tumours (NET) due to fibrotic endocardial plaques with associated valve dysfunction leading most often to right-sided heart failure. The classical carcinoid syndrome usually occurs when serotonin-producing NET metastasize to the liver. Up to 50% of those patients will exhibit carcinoid heart disease. The pathophysiological process is not yet completely understood: serotonin is considered to be a major initiator of the fibrotic process, but other tumour secreted factors may contribute to the pathogenesis. Histopathology reveals intact valvular cusps with superimposed fibrotic plaques, leading to thickening and retraction of the valves, causing valvular dysfunction. A high index of clinical suspicion to diagnose CHD is needed since symptoms can be rather non-specific. Transthoracic echocardiography is the gold standard for diagnosis and should probably be performed at the time of diagnosing serotonin-producing NET and then repeated annually. On the other hand, when diagnosing right-heart failure, the presence of CHD and underlying serotonin-producing NET should be taken into account. Therapeutic options include pharmacotherapy for heart failure, control of the systemic carcinoid disease and in selected individuals cardiac valve replacement. The elucidation of the pathologic process is necessary to develop targeted antifibrotic therapeutic agents since CHD seems to be irreversible and associated with poor prognosis.
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PMID:Carcinoid heart disease--a hidden complication of neuroendocrine tumours. 1940 69

We present the case of a 69-year-old woman with a metastatic neuroendocrine tumour of the ileum and severe tricuspid valve regurgitation due to carcinoid valvulopathy. Carcinoid heart disease arises in the context of metastatic serotonin-producing neuroendocrine tumours and typically presents as structural and functional abnormalities of tricuspid (TV) or pulmonary valve (PV). Carcinoid heart disease arises from a low-grade neuroendocrine tumour derived from serotonin-producing enterochromaffin cells that reduces the mobility of the leaflets. Following the development of this type of heart failure, the prognosis is unfavourable and patients usually die as a result of heart failure and not because of the metastatic disease. Our patient was not considered a candidate for valvular surgery because of the progressive nature of the malignancy. However, surgical valve replacement is a therapeutic option that, although it has a significant mortality, needs to be considered in selected patients and leads to improvement in functional capacity and survival.
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PMID:Carcinoid heart disease: case and literature review. 2045 39

Carcinoid heart disease (CHD) is a rare form of valvular heart disease characterized by right-sided heart valve dysfunction. Carcinoid heart disease occurs most frequently when carcinoid tumour cells metastasize from a primary site in the gut to the liver, so that vasoactive substances produced by the tumour are able to reach the systemic circulation. By contrast, in ovarian carcinoid tumours carcinoid heart disease develops in the absence of liver metastasis, because vasoactive substances can be released directly into the systemic circulation, bypassing the first-pass metabolism of the liver. There are only a few case reports in the world literature of carcinoid heart disease caused by ovarian carcinoid tumour. We report a case of an 85-year-old woman with carcinoid heart disease caused by a bilateral ovarian carcinoid tumour metastasized from a primary ileal site.
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PMID:An unusual cause of carcinoid heart disease. 2130 82

Carcinoid heart disease is a rare cause of heart failure with or without right valvular heart impairments. In this study, we showed a case of carcinoid tumour with hepatic metastases inducing carcinoid heart disease. Neuroendocrine heart involvement happens for severe tricuspid valve insufficiency and plaques on right ventricular (RV) walls produced by a release of serotonin (5-HT). A patient affected by primitive ileal tumour with 5-HT-secernent hepatic metastases inducing tricuspid insufficiency is showed. Transthoracic 2-D echocardiography showed tricuspid valve regurgitation and both right atrium, RV-walls plaques and RV dilation. Continue-wave Doppler showed a characteristic "dagger shaped" spectrum of tricuspid systolic flow. RV function was evaluated with 3-D transthoracic echocardiography. In particular, RV volumes, RV ejection fraction and stroke volume were defined by this technique. 2, 3-D echocardiography and Doppler method are useful techniques to show heart valves' derangements and RV function to non-invasively detect RV impairments in carcinoid heart disease.
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PMID:Carcinoid Heart Disease: A Rare Cause of Right Ventricular Dysfunction Evaluation by Transthoracic 2D, Doppler and 3-D Echocardiography. 2186 Jul 26


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