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)

Peritoneal carcinomatosis is a well-known factor of poor prognosis in patients with digestive adenocarcinomas. Peritoneal dissemination may also occur in midgut well-differentiated neuroendocrine tumors, but its influence on survival is ill-defined. We report here the history of a 64-year-old woman who had a neuroendocrine tumor of the cecum with multiple synchronous metastases in the liver and diffuse peritoneal carcinomatosis. She underwent surgical resection of the primary tumor and cytoreduction of liver metastases, and received subsequently chemotherapy and somatostatin analogs. In spite of the widespread extension of the disease, she survived for 13 years and died from a carcinoid heart disease. We discuss the natural history and prognostic factors in patients with midgut well-differentiated neuroendocrine tumors, with a focus on the impact of the peritoneal carcinomatosis.
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PMID:Prolonged survival in a patient with neuroendocrine tumor of the cecum and diffuse peritoneal carcinomatosis. 2264 33

Midgut neuroendocrine tumors (MNETs) are rare, and the primary tumor is usually small and difficult to visualize at imaging. Patients often present late with extensive liver and nodal metastases and may experience symptoms secondary to the release of active substances by the primary tumor, such as serotonin and its metabolites, which have local and systemic effects. Locally, this causes desmoplasia and vascular encasement and may lead to small bowel obstruction and ischemia, with significant morbidity and mortality. Systemically, the release of active substances into the circulation can cause flushing, diarrhea, and abdominal pain (carcinoid syndrome); these substances can be detected in urine and blood serum and used as markers for diagnosis and treatment follow-up. MNETs retain expression of specific peptide receptors such as somatostatin receptors, which will bind to synthetic somatostatin analogs such as octreotide. This feature is useful for functional imaging of patients with MNETs and for peptide receptor radionuclide therapy using somatostatin analogs. Resection of the primary tumor is advocated, even in patients with extensive metastases, because it may prevent development of local complications, can help control systemic symptoms, and has been shown to confer some survival advantage. Computed tomography and functional imaging are used to identify the primary tumor and assess its resectability. The main factors governing resectability are patient comorbidities (eg, carcinoid heart disease), vascular involvement, and desmoplasia.
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PMID:Midgut neuroendocrine tumors: imaging assessment for surgical resection. 2461 88

The management of patients with midgut neuroendocrine tumors (MNET) is rapidly evolving. Current preoperative detection rates of primary tumor sites are higher than ever and progression-free survival in patients with already advanced disease is expanding due to the implementation of novel efficacious treatment strategies. This survival benefit may potentially translate into a need for a multidisciplinary approach to an even more heterogenous variety of clinical conditions, among these, carcinoid syndrome (CS) and carcinoid heart disease (CHD). The latter often triggers substantial morbidity and mortality, hence a systematic screening, an accurate diagnosis, as well as effective interventions are critically important. The rarity of the disease has result in a relative lack of statistically powerful evidence, which in turn may have rendered significant variability between practices. In this regard, despite recent guidelines, the optimal follow-up of patients with CHD remain debatable to some authors, perhaps due to the preponderance of certain schools throughout the manuscript. Herein, we present a concise and practical guidance document on clinical screening and echocardiographic surveillance of patients with CHD based on a comprehensive review of the literature, and complemented by our experience at the Center for Carcinoid and Neuroendocrine Tumors at The Mount Sinai Hospital.
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PMID:Echocardiography in functional midgut neuroendocrine tumors: When and how often. 2908 Sep 35

The background to this study was that factors associated with carcinoid heart disease (CHD) and its impacts on overall survival (OS) are scantly investigated in patients (pts) with neuroendocrine tumors (NETs). In terms of materials and methods, a retrospective multicenter cohort study was conducted of factors associated with CHD in advanced NET pts with carcinoid syndrome (CS) and/or elevated urinary 5-hidroxyindole acetic acid (u5HIAA). CHD was defined as at least moderate right valve alterations. The results were the following: Among the 139 subjects included, the majority had a midgut NET (54.2%), 81.3% had CS, and 93% received somatostatin analogues. In a median follow-up of 39 months, 48 (34.5%) pts developed CHD, with a higher frequency in pts treated in public (77.2%) versus private settings (22.9%). In a multivariate logistic regression, unknown primary or colorectal NETs (Odds Ratio (OR) 4.35; p = 0.002), at least 50% liver involvement (OR 3.45; p = 0.005), and being treated in public settings (OR 4.76; p = 0.001) were associated with CHD. In a Cox multivariate regression, bone metastases (Hazard Ratio {HR} 2.8; p = 0.031), CHD (HR 2.63; p = 0.038), and a resection of the primary tumor (HR 0.33; p = 0.026) influenced the risk of death. The conclusions were the following: The incidence of CHD was higher in pts with a high hepatic tumor burden and in those treated in a public system. Delayed diagnosis and limited access to effective therapies negatively affected the lives of NET patients.
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PMID:Carcinoid Heart Disease and Decreased Overall Survival among Patients with Neuroendocrine Tumors: A Retrospective Multicenter Latin American Cohort Study. 3090 90

A 55-year-old female patient was admitted for flushing and abdominal pain in the right upper quadrant. Her past medical history revealed high blood pressure and a recent echocardiography showed thickened appearance of tricuspid valve with coaptation defect and grade II tricuspid regurgitation. Contrast enhanced abdominal CT scan and MRI were subsequently performed and revealed a large macronodular liver mass, as well as other micronodular lesions disseminated in the liver parenchyma. CT guided biopsy from the main liver mass revealed neuroendocrine tumor of unknown origin (probably GI) with Ki-67 of 8%. Surgical exploration was decided. During laparotomy, the primary tumor was found in the proximal ileum and the patient underwent segmental enterectomy. Non-anatomical hepatectomy was also performed to remove the bulk of the tumor burden (more than 90%). Postoperative course was uneventful and the carcinoid syndrome relieved. At present, 15 months postoperatively, the patient is under treatment with somatostatin analogue for its antiproliferative effect, with good clinical, biochemical and tumoral control and stable heart disease. In patients with neuroendocrine liver metastases from unknown primary, surgical exploration could allow detection (and resection) of the primary tumor and surgical debulking of liver metastases to control carcinoid syndrome and carcinoid heart disease.
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PMID:THE ROLE OF SURGERY IN A PATIENT WITH CARCINOID SYNDROME, COMPLICATED BY CARCINOID HEART DISEASE. 3114 45

Cardiac hemangioma is a benign and rare primary tumor of the heart. Though it has benign histopathological features, its complications can be life-threatening. We report a young adult male without any prior structural heart disease or medical history who presented with ventricular tachyarrhythmia. Echocardiography revealed an echogenic mass located intramurally in the left lateral ventricle and its distinctive characteristics were revealed with further imaging modalities. Though simple complete removal of the mass is the preferred treatment, its firm texture and thinned encircling myocardium prevented the total excision. In this case report, we discussed cardiac hemangioma, its potential complications and treatment options.
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PMID:Cardiac hemangioma in a difficult anatomical location presented with ventricular tachycardia. A rare case report. 3327 Sep 34


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