Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 64-year-old man with a six-month history of visual loss was found to have multiple darkly pigmented lesions scattered throughout the choroid of both eyes. Some lesions, up to several disc diameters in size, were flat, but others were elevated one to two millimeters. The right eye also contained a darkly pigmented ciliary body mass. Impairment of light transmission was striking. Ultrasonography of the elevated lesions demonstrated solid masses with high internal reflectivity. The initial diagnostic differential included the possibility of pigmented metastases from a primary tumor elsewhere, as well as multiple primary nevi. A general medical examination demonstrated arteriosclerotic heart disease, hypertension, diabetes mellitus, areas of patchy pigmentation of the oral mucosa, and a hilar prominence. A mediastinal lymph node biopsy specimen showed a grade 3 undifferentiated carcinoma compatible with a lung primary carcinoma. Enlargement of the choroidal lesions was observed until the patient's death 15 months later. Autopsy showed an undifferentiated carcinoma of the lung with widespread metastases. Each eye showed multiple discrete benign melanocytomas within a diffuse nevus involving the entire uveal tract. The nature of the relationship between multiple uveal melanocytomas and the systemic carcinoma is uncertain, but recognition of multiple uveal melanocytomas warrants a general medical examination to exclude primary malignancy elsewhere.
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PMID:7. Pseudometastatic lesions of the choroid. 724 11

Carcinoid is a slowly growing type of tumor; its pathological effects are primarily due to its endocrine symptomatology. Among the main causes of death are heart failure, liver disease and complications due to the tumor size. The purpose of this study was to investigate carcinoid heart disease in a wide necropsy sample. We analyzed 26,921 necropsies performed at the Institute of Pathology of the University of Trieste from January 1, 1976 to December 31, 1985. Out of the 26,921 necropsies we found 59 cases with carcinoid tumor. It is interesting to underline the presence of a second primary tumor in 28.8% of cases and of multiple tumors in 2 cases. In the heart we observed valvular abnormalities with no peculiar features in 16.1% of cases, ischemic heart disease in 52.2% and endocardial thickening in 15.9%. Our series, although rather large, did not show the high frequency of carcinoid heart disease that has been reported by other authors; instead, we observed a rather high prevalence of lesions typical of ischemic heart disease. The endocardial thickening seems to be the most interesting and specific finding.
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PMID:[Carcinoid cardiopathy. A study of 40 cases]. 802 41

A case of successful tricuspid valve replacement with a mechanical prosthesis and pulmonary valvulotomy for carcinoid heart disease is reported. The patient was a 61 years old women. The primary tumor was in the terminal ileum. Liver metastasis and carcinoid syndrome were present since 8 years. After cardiac surgery, the patient survived 38 months and late death was related to disseminated metastasis. Even in case of metastasis, carcinoid tumor is slow growing. Without cardiac operation for correction of valvular lesions, terminal symptoms and death may often be related to cardiac operation for correction of valvular lesions, terminal symptoms and death may often be related to cardiac failure rather than to tumoral growth. Thus, even in presence of metastasis, cardiac surgery may be mandatory to improve both quality of life and survival.
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PMID:Successful tricuspid valve replacement and pulmonary valvulotomy for carcinoid heart disease. 883 Aug 75

Carcinoid tumors are endocrine malignancies that are often associated with a characteristic syndrome, the malignant carcinoid syndrome, which is most common in patients with small bowel tumors and liver metastases. In the rare instances when the syndrome is present without liver metastases the primary tumor is usually localized to the bronchus or ovary and secretes hormones directly into the systemic circulation. About two thirds of patients with carcinoid syndrome have evidence of carcinoid heart disease. We report on a case of a primary ovarian carcinoid tumor with an unusual clinical presentation.
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PMID:Primary carcinoid tumor of the ovary: report of an unusual case. 1077 76

Primary cardiac tumors are uncommon with an estimated incidence of between 0.0017 % and 0.19 %. Most are benign. Whereas myxomas are the most common primary tumor in adults, in children they are exceptionally rare. Cardiac myxomas usually develop in the left atrium, 20 % occur in the right atrium and the remainder develops in the ventricles and rarely in the heart valves. Cerebrovascular strokes secondary to myxoma are rare in childhood. The diagnostic test of choice is transesophageal echocardiogram and early excision is the most effective treatment in preventing serious complications. We report a case of cerebral stroke as the only manifestation of an atrial myxoma in an 11-year-old-girl. The patient presented vertigo, right hemiparesis of the body and dysarthria without loss of consciousness. After diagnostic tests (computerized tomography, magnetic resonance imaging and cerebral angioresonance) she was diagnosed with an ischemic lesion in the left middle cerebral artery. Various investigations were performed to find the cause of the stroke, among them cardiologic study, and a mass in the left atrium suggestive of myxoma was found. The tumor was removed and the diagnosis of myxoma was confirmed by histopathological examination. Outcome was satisfactory. The presence of a cerebral ischemic episode, with or without concomitant heart disease, suggests the need to look for cardiac etiology.
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PMID:[Cerebrovascular stroke as a sign of atrial myxoma in childhood]. 1262 1

Demonstrated are eight cases of carcinoid heart disease (7 females, 1 male; average age 63.4 y.). The primary tumor was localized in the ileum in 5, and in the cecal area in 3 patients, with liver metastases in each case. The characteristic heart lesion appears as an endocardial plaque composed of loose intercellular matrix with scattered smooth muscle cells, covered by an endothelial layer. The plaques are apposed on an otherwise normal valvular, or mural endocardium of the right heart. The resulting combined valvular lesions show a predominance o tricuspid incompetence, or of pulmonary stenosis. In one case with a widely patent foramen ovale there was involvement of left-heart valves in addition. Local effect of serotonin appears to play the main role in pathogenesis of the endocardial plaques.
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PMID:[Carcinoid heart disease]. 1287 1

Carcinoid tumors are slow-growing neuroendocrine neoplasms most commonly associated with the gut and broncho-pulmonary system. In many instances, they are identified at surgery for unexplained bowel obstruction or during exploration of the small bowel in search of a primary tumor once distant metastases have been detected. Carcinoid tumors of the small bowel often present with pronounced fibrosis in the peri-tumoral tissues, distant in the heart or lungs, and locally in the peritoneal cavity. Despite medical and therapeutic advances that have alleviated symptoms and prolonged life, a substantial subset of patients develops mesenteric and small bowel carcinoid fibrosis and/or carcinoid heart disease. Fibrosis, and increasingly cardiac heart disease, are important components of intestinal carcinoid disease and are of considerable clinical concern, as both of these conditions reflect a connective tissue disorder whose etiology, biology, and therapy are unknown. In the past, individuals with carcinoid disease died of metastasis and uncontrollable symptomatology. Currently, there exists no clinical method to determine the development of fibrosis and little is understood about the biological basis of fibrosis. The elucidation of the biology and management of fibrosis is thus an issue of paramount clinical and scientific importance in determining appropriate diagnostic and therapeutic strategy. Therefore, the unraveling of the molecular events indicative of fibrosis in these cells and the identification of appropriate therapeutic targets is of considerable patient-care relevance. We have surveyed the world literature over the past 40 yr to evaluate both the incidence of carcinoid processes and track the evolving understanding of this process. In addition, we have provided more current mechanistic information in regard to the biological basis of fibrosis associated with small bowel carcinoid tumors.
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PMID:Carcinoid tumors and fibrosis: an association with no explanation. 1557 97

Factors that determine the clinical course and outcome of patients with gastrointestinal (GI) carcinoid tumors are complex and multifaceted. These include the site of origin within the GI tract, the size of the primary tumor, and the anatomical extent of disease, whether localized, regional, or metastatic to distant sites. The new World Health Organization (WHO) histological classification of endocrine tumors, including carcinoids, represents a significant advance in terms of providing a consistent framework for histopathological interpretation that should facilitate multicenter research on treatment outcomes. Histochemical indicators of a poorer prognosis are the degree of expression of the proliferation protein Ki-67 and the p53 tumor suppressor protein. Adverse clinical indicators are the malignant carcinoid syndrome, carcinoid heart disease, and high concentrations of the tumor markers, urinary 5-HIAA and plasma chromogranin A.
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PMID:Prognostic indicators for carcinoid neuroendocrine tumors of the gastrointestinal tract. 1571 76

A 23-year-old female patient with malignant pheochromocytoma was admitted to the Tokyo Women's Medical University. The patient had been clinically diagnosed with Holt-Oram syndrome at birth. Since she had complex congenital heart disease, chronic heart failure, and severe hypoxia, the risk surrounding surgery to remove the primary tumor was predicted to be very high, and subsequently, chemotherapy was performed. The patient was not able to continue chemotherapy due to adverse effects. However, for one year, both her hypertension and catecholamine-dependent symptoms were well controlled by an alpha-adrenergic and beta-adrenergic receptor blockade, although the patient did experience high plasma norepinephrine levels. To our knowledge, this is the first report of a patient with the combination of malignant pheochromocytoma and Holt-Oram syndrome. A correlation between chronic hypoxia and pheochromocytoma has been reported. This instructive case reminds us to consider the possibility of pheochromocytoma with congenital heart disease when these types of unexpected or unusual symptoms are encountered.
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PMID:A case of malignant pheochromocytoma with Holt-Oram syndrome. 1825 May 43

Liver metastases from endocrine tumors can reduce 5-year survival from 90% to 40% and, in cases of functional gastrointestinal endocrine tumors, lead to a carcinoid syndrome. Complete resection of cancerous disease should be considered in all cases. Indeed, after hepatectomy, prolonged survival (41-86% at five years) can be achieved, with low rates of surgery-related mortality (0-6.7%). Extended liver resection is required in most cases. Percutaneous portal embolization increases the volumetric feasibility of resection, and sequential hepatectomy techniques enable a two-stage resection of both bilobar metastases and the primary tumor. For carcinoid syndrome that does not respond to medical therapy, incomplete resection of liver metastases, by reducing tumor volume, may be indicated to reduce symptoms and halt the progression of carcinoid heart disease. In cases of non-resectable liver metastases in selected patients, liver transplantation can lead to 5-year survival rates as high as 77%.
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PMID:Surgical management of liver metastases from gastrointestinal endocrine tumors. 2034 42


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